MRCP PART 2 FORUM JULY ATTEMPT ONWARDS.

DR ALRAZI · Posted: Wed Feb 25, 2009 10:31 am Post subject:

Hi Dr

waiting for ur responses and suggestions.

Best regards.

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DR ALRAZI

DR ALRAZI · Posted: Wed Feb 25, 2009 2:20 pm Post subject:

Hi Dr,

alhamdulillah i pass Jan 2009 exam.Thanks God

hope this forum will be continue for mrcp-2 exam.

Cool

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DR ALRAZI

mrsidhwa · Posted: Wed Feb 25, 2009 5:35 pm Post subject:

DR ALRAZI IT APPEARS YOU HAVE WRITTEN THE WHOLE ENDOCRINOLOGY GREAT IT WILL TAKE TIME TO READ IT SO WHEN ARE YOUR PLANS FOR PART 2

Guest · Posted: Wed Feb 25, 2009 5:51 pm Post subject:

there is a section on how to analyse medical investigations on medicalrevision dot org
It is useful basic guidance for part 2

DR ALRAZI · Posted: Wed Feb 25, 2009 8:19 pm Post subject:

Hi Dr ,

i am thinking seriously to register in the next diet ( july 2009 ).

what about u ?

with my best wishes.

_________________
DR ALRAZI

DR ALRAZI · Posted: Thu Feb 26, 2009 1:19 am Post subject: Very important topic

Salam

read up on tirofiban

_________________
DR ALRAZI

dr_osler · Posted: Sat Feb 28, 2009 1:55 am Post subject: An aid to mrcp

An aid to mrcp audio tool

http://rapidshare.com/files/171706157/Aid_to_MRCP_Audio_Tool.part2.rar

DR ALRAZI · Posted: Sat Feb 28, 2009 2:24 am Post subject:

Salam

DERMATOLOGY

Rash Diagnosis Algorithm v1.1

Marc Roy, MD

Adapted from: PJ Lynch

Basic Terminology

Macule – < 2cm diameter area of color change, with no palpable substance
Patch – > 2cm diameter macule
Papule – Palpable mass < 1.5cm diameter
Nodule – Spherical enlargement of a papule > 1.5cm diameter
Plaque – Flat-topped palpable lesion > 1.5cm diameter, papule that is enlarged in 2 dimensions.
Wheal – Edematous papule on plaque. Nonloculated fluid.
Vesicle – Fluid-filled papule < 1-1.5cm diameter
Bulla – > 1-1.5cm vesicle
Pustule – Vesicle packed full of polys (may or may not be sterile).
Excoriation– Scratchmarks
Lichonification – Thickening secondary to chronic rubbing or scratching (seen only in eczematous diseases).

CLEAR FLUID-FILLED

Vesiculo-Bullous Diseases

A. Vesicular diseases
1. Herpes simplex
2. Varicella-zoster
3. Vesicular tinea pedis
4. Dyshidrosis (pompholyx)
5. Scabies
6. Dermatitis herpetiformis

B. Bullous diseases
1. Posion-ivy-type contact dermatitis
2. Bullous impetigo
3. Erythema multiforme bullosum (Steven Johnson syndrome)
4. Pemphigoid
5. Pemphigus

Others
• congenital: epidermolysis bullosa
• insect bite
• trauma/friction
• drugs: barbiturates, frusemide

PUSTULAR FLUID-FILLED

Pustular Diseases

A. True (soft pustules)

1. Acne vulgaris
2. Rosacea (acne rosacea)
3. Bacterial folliculitis
4. Fungal folliculitis
5. Candidiasis
6. Systemic bacterial infection (eg, gonorrhea)

B. Pseudo-pustules

1. (See white papules)

Solid, NON-RED

Skin-Colored Lesions

A. Keratotic (rough-surfaced lesions)

1. Warts: verruca vulgaris, paranychial and plantar warts
2. Actinic keratoses
3. Seborrheic keratoses
4. Corns and calluses

B. Nonkeratotic (smooth lesions)

1. Warts: genital warts, flat warts
2. Basal and squamous cell carcinoma (with or without ulceration)
3. Epidermoid (‘sebaceous’) cysts
4. Lipomas
5. Molluscum contagiosum
6. Nevi: intradermal

White Lesions

A. White patches and plaques

1. Pityriasis alba
2. Pityriasis (tinea) versicolor
3. Vitiligo
4. Postinflammatory hypopigmentation

B.White papules

1. Milia
2. Keratosis pilaris
3. Molluscum contagiosum
4. Sebaceous gland hyperplasia

Brown Lesions

A. Brown macules

1. Freckles
2. Lentigenes
3. Nevi: junctional

B. Brown papules and nodules

1. Nevi: compound and intradermal
2. Seborrheic keratoses
3. Melanoma

C. Brown patches and plaques

1. Café-au-lait patches
2. Postinflammatory hyperpigmentation
3. Giant congenital nevi

D. Generalized hyperpigmentation

1. Secondary to systemic disease
2. Secondary to medication
3. Postinflammatory hyperpigmentation

Yellow Lesions

A. Smooth yellow lesions

1. Xanthelasma
2. Necrobiosis lipoidica diabeticorum
3. Sebaceous gland hyperplasia

B. Rough yellow lesions

1. Actinic keratoses
2. Any crusted lesion (see vesiculo-bullous diseases, eczematousand insect bites)

Solid, Red, NONSCALING
Solid, Red, SCALING

Solid, Non-Red and is…

SKIN-COLORED
WHITE
BROWN
YELLOW

Solid, Red, Nonscaling and is…

DOME-SHAPED
FLAT-TOPPED

Solid, Red, Scaling and has…

EPITHELIAL DISRUPTION
NO EPITHELIAL DISRUPTION

[The rash is…]
[The rash is…]

Inflammatory Papules and Nodules

A. Nonscaling red papules
1. Insect bites
2. Cherry angiomas
3. Spider angiomas
4. Granuloma annulare
5. See nonconfluent papules
B. Nonscaling red nodules
1. Furuncles
2. Inflamed epidermoid cysts
3. Hidradenitis supportive
4. Erythema nodosum

[The rash is…]
Vascular Reactions

A. Nonpurpuric (blanchable) lesions
1. Toxic erythema: exanthems, medications, photosensitivity
2. Urticaria: infection, medications
3. Erythema multiforme
4. Cellulitis (erysipelas)
B. Purpuric lesions
1. Vasculitis (PMN type, palpable purpura)
2. Actinic (‘senile’) purpura
3. Petechia and ecchymoses secondary to medications

[The rash is…]
Eczematous Diseases

A. Excorations prominent
1. Atopic dermatitis (neurodermatitis, lichen simplex chronicus, infantile eczema)
2. Dyshidrotic eczema
3. Stasis dermatitis
4. Tinea: cruris, capitis, pedis
5. Psoriasis in atopic individuals
6. Candidiasis
B. Little or no excoriations
1. Seborrheic dermatitis
2. Contact dermatitis
3. Xerotic (asteatotic) eczema
4. Impetigo
C. Eczematous reaction patterns (seen with more than one of the above eczematous diseases)
1. Hand and foot eczema
2. Diaper dermatitis
3. Nummular eczema
4. Exfoliative erythrodermatitis
5. Autoeczematization (autosensitization, ‘Id’ reaction)

[The rash is…]
Papulosquamous Diseases

A. Prominent plaque formation
1. Psoriasis vulgaris
2. Tinea: corporis, capitis, pedis and cruris
3. Lupus erythematosis: discoid type
4. Parapsoriasis-mycosis fungoides
B.Nonconfluent papules
1. Pityriasis rosea
2. Lichen planus
3. Syphilis: secondary
4. Psoriasis: guttate type

[The rash is…]

Shin lesions

The differential diagnosis of shin lesions includes the following conditions:

• erythema nodosum
• pretibial myxoedema
• pyoderma gangrenosum
• necrobiosis lipoidica diabeticorum

Below are the characteristic features:

Erythema nodosum

• symmetrical, erythematous, tender, nodules which heal without scarring
• most common causes are streptococcal infections, sarcoidosis, inflammatory bowel disease and drugs (penicillins, sulphonamides, oral contraceptive pill)

Pretibial myxoedema

• symmetrical, erythematous lesions seen in thyrotoxicosis
• anywhere but typically on shins dorsum of feet
• shiny, orange peel, raised, indurated pinkish patches
• acropachy, ophthalmopathy and a high titre of TSH recpetor antibodies

Pyoderma gangrenosum

• initially small red papule
• later deep, red, necrotic ulcers with a violaceous border
• idiopathic in 50%, may also be seen in inflammatory bowel disease, connective tissue disorders and myeloproliferative disorders

Necrobiosis lipoidica diabeticorum

• shiny, painless areas of brown-red or slightly yellow skin
• often associated with surrounding telangiectasia
• commoner in females than males
• Fewer than 1% of diabetic patients have this but most with it have diabetes mellitus
• May prescede symptoms and signs of diabetes by several months
• topical and intralesional steroids have been used
• Inhibition of platelet aggregation with aspirin may be helpful

Skin disorders associated with pregnancy
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Polymorphic eruption of pregnancy

• pruritic condition associated with last trimester
• lesions often first appear in abdominal striae
• not associated with blistering

Herpes gestationis

• pruritic blistering lesions
• often develop in peri-umbilical region; also palms and around mouth
usually presents 2nd or 3rd trimester and is rarely seen in the first pregnancy

Oral hairy leucoplakia (OHL)

• potentially malignant condition

Associations

• immunocompromised eg AIDS (most common)
• Epstein–Barr virus
• inflammatory bowel disease
• Behcet’s

Predisposing factors

• Smoking
• Alcohol consumption
• Ill-fitting dentures
• Malocclusion of teeth

Features

• non-painful
• irregular white patches on the side of the tongue (or elsewhere on the tongue or in the mouth)
• cannot be moved or dislodged

Risk in HIV

• Smoking more than a pack of cigarettes a day
• Risk doubles with each 300-unit decrease in CD4 count

DD
• Aphthous ulcers: painful
• Oral candidiasis:white patches on gums, tongue & inside the mouth that can be peeled off leaving a raw area.
• Oral lichen planus: patches of fine white lines and dots
• white sponge naevus of Canon is a white folded dysplasia of the mucous membranes. It is a familial condition with an autosomal dominant pattern of inheritance

Diagnosis

• Clinical
• Biopsy to exclude dysplasia or malignancy.

Treatment

• gentian violet
• valaciclovir in few cases
• R/ of the underlying disorder, most often with anti-retroviral therapy.
• antiviral medication with aciclovir or ganciclovir if not taking anti-HIV therapy

Seborrhoeic keratoses

• also called basal cell papillomas, senile warts or brown warts
• commonest benign tumour in older individuals

Features

• both covered and uncovered parts of the body
• begin as slightly raised, skin coloured or light brown spots
• then they thicken and take on a rough, warty surface.
• They slowly darken and may turn black
• They can itch, grow, and become cosmetically unattractive
• Scratching or trying to pick them off the skin can result in a secondary infection

Variants of seborrhoeic keratoses include:

• Some solar lentigines: flat brown marks in sun exposed areas
• Stucco keratoses: numerous small dry grey stuck-on lesions usually found on lower legs and feet
• Dermatosis papulosa nigra: numerous brown warty papules on face, neck and chest of dark-skinned individuals
• Irritated seborrhoeic keratosis: inflamed lesion, often red and crusted; may resemble a skin cancer
• Lichenoid keratosis: resolving keratosis or lentigo, often pink or grey-coloured

Stucco keratoses
Dermatosis papulosa nigra
Irritated seborrhoeic keratosis

Treatment

• Cryotherapy (liquid nitrogen) for thinner lesions
• Curettage & cautery
• Laser surgery
• Shave biopsy (shaving off with a scalpel)

Herpes simplex virus
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There are two strains of the herpes simplex virus (HSV) in humans: HSV-1 and HSV-2. Whilst it was previously thought HSV-1 accounted for oral lesions (cold sores) and HSV-2 for genital herpes it is now known there is considerable overlap

Features

• recurrent episodes* (always recurs at the site at which the primary infection was)
• prodromal pain, itching, burning or tingling
• small grouped vesicles (cold sores) precipitated by fever. They burst, crust and then heal in 7–10 days. Initial vesicles contain clear fluid
• primary infection: may present with a severe gingivostomatitis. The gums are swollen and red and bleed easily. Vesicles (little blisters) occur in white patches on the tongue, throat, palate and insides of the cheeks. The white patches are followed by ulcers with a yellowish coating. The local lymph glands are enlarged and tender
• On the eyelids, it typically presents as a rash consisting of between three and five vesicles. The eyelid may be oedematous, erythematous and the patient may complain of localised pain and tenderness. These vesicles become pustular or ulcerative with formation of crusts within 72–96. comlications: corneal ulcer, acute retinal necrosis
• painful genital ulceration
• in adults certainly it is more likely than impetigo
• viral polymearse chain reaction (PCR) confirm the diagnosis

* The source of the virus may be from elsewhere on the body especially in nail biters or thumb suckers. Herpes simplex can also be inoculated from external sources. Examples include:

• Nailfold infection in a health-care worker (‘herpetic whitlow’)
• Facial blisters in a rugby player (‘scrum pox’)
• Suckling infant with mouth sores

Treatment

• No treatment: in Mild uncomplicated eruptions
• hydrocolloid patch if desired
• high protection factor sunscreens to prevent facial herpes
• Oral antiviral eg Acyclovir in severe infection

Recurrent cellulitis secondary to herpetic whitlow

• consider in cases of unexplained recurrent cellulitis.
• self-limiting disease
• incidence of 3 per 100 000 / year!
• Young adults
• Cause: HSV-2
• prodromal phase up to 72 h
• recurrence of 7–10 days’ duration.
• vesiculo-pustular plaque
• Lymphangitis and lymphadenitis
• Lymphoedema of the hand and forearm (rare)
• Acute infection should be treated with Aciclovir 800 mg 5 times/day followed by lower doses (400 mg bd) as prophylaxis

Herpes zoster infection

Cause

• The virus Herpes varicella-zoster lies dormant in the dorsal root ganglion following chicken pox, and later travels down the cutaneous nerves to infect the epidermal cells

Features

• prodromal period
• followed by a rash consisting of a group of vesicles on an erythematous background
• The rash is nearly always unilateral and confined to one or two dermatomes
• followed by weeping and crusting
• healing takes 3–4 weeks

Treatment

• Oral antiviral agents eg Aciclovir 800 mg 5 times/day
• Amitriptyline: prophylactic at night starting at 25 mg and increasing to 75 mg can help prevent post-herpetic neuralgia
• Carbamazepine: block the pain associated with post-herpetic neuralgia
• Axsain cream, (from an extract of capsicum): it depletes substance P in affected neurones and is particularly effective for postherpetic neuralgia

Herpes zoster ophthalmicus

• caused by the varicella zoster virus
• Vesicles present on the nasolabial fold or on the tip of the nose suggests involvement of the cornea
• urgent ophthalmological assessment to reduce the risk of loss of vision

Chickenpox
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Chickenpox is caused by primary infection with varicella zoster virus. Shingles is reactivation of dormant virus in dorsal root ganglion

Chickenpox is highly infectious

• spread via the respiratory route
• can be caught from someone with shingles
• infectivity = 4 days before rash, until all lesions scabbed over*
• incubation period = 11-21 days

Clinical features (tend to be more severe in older children/adults)

• fever initially
• itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular
• systemic upset is usually mild

Management is supportive

• keep cool, trim nails
• calamine lotion
• school exclusion: current HPA advice is 5 days from start of skin eruption. They also state "Traditionally children have been excluded until all lesions are crusted. However, transmission has never been reported beyond the fifth day of the rash."
• immunocompromised children and infants with peripartum exposure should receive varicella zoster immunoglobulin (VZIG). If chickenpox develops then IV aciclovir should be considered

A common complication is secondary bacterial infection of the lesions. Rare complications include

• pneumonia: Varicella pneumonia occurs in up to 20% of adults with chickenpox
• encephalitis (cerebellar involvement may be seen)
• disseminated haemorrhagic chickenpox
• arthritis, nephritis and pancreatitis may very rarely be seen

*it is now thought that patients are no longer infectious 5 days after the rash has developed - see management regarding school exclusion

Viral warts

Cause

• human papilloma virus infections

Types and Features

• epidermodysplasia verruciformis
• anogenital and/or mucosal
o Oral warts can affect the lips and even inside the cheeks. They include squamous cell papillomas.
o Genital warts are often transmitted sexually and predispose to cervical, penile and vulval cancer
• nongenital cutaneous
• spread by direct or indirect contact
• benign, self-limiting warts
• skin-coloured exophytic nodules
• Warts have a hard ‘warty’ or ‘verrucous’ surface
• tiny black dot in the middle of each scaly spot
• ‘kissing warts’ due to the autoinoculation on an adjacent finger
• There are various types
o Common warts arise most often on the backs of fingers or toes, and on the knees.
o Plantar warts
o Mosaic Plantar warts
o Plane, or flat, warts: very numerous and may be inoculated by shaving.
o Periungual warts at the sides or under the nails
o Filiform warts are on a long stalk.

Common warts
Cauliflower wart
Plantar warts

Treatment

• plaster or duct tape: Just keeping the wart covered 24 hours of the day
• Chemical treatment.
o Wart paints eg containing salicylic acid or Podophyllin *
o Upton's paste: for thick verrucas,
o 3% formalin solution: in multiple mosaic plantar warts
• Cryotherapy : The wart is frozen with liquid nitrogen
• Electrosurgery: curettage and cautery is used for large and annoying warts
• Other treatments
o bleomycin injections
o laser vaporization
o oral acitretin
o immune modulators such as imiquimod cream.

* cytotoxic agent, and must not be used in pregnancy or in women considering pregnancy.

Genital warts
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Genital warts (also known as condylomata accuminata) are a common cause of attendance at genitourinary clinics. They are caused by the many varieties of the human papilloma virus (especially types 6 & 11). It is now well established that human papilloma virus (especially types 16,18 & 33) predisposes to cervical cancer

Features

• small (2 - 5 mm) fleshy protuberances which are slightly pigmented
• may bleed or itch

Management

• topical podophyllum or cryotherapy are commonly used as first-line treatments depending on the location and type of lesion
• genital warts are often resistant to treatment are recurrence is common

AIDS skin lesions

1. Molluscum contagiosum

• Caused by pox virus of Molluscipox virus genus
• With advanced HIV/AIDS (CD4 count <200)
• Transmission requires direct contact with infected hosts or contaminated fomites
• Higher incidence in children, sexually active adults and immunodeficent
• acquired from bath towels, tattoo instruments, in beauty parlors and Turkish baths
• incubation time 2-7 weeks (up to 6 months)
• benign self-limited, Umbilicated, pearly papules 2-5mm in diameter
• confined to skin and mucous membranes, Commonly occur on face, especially near eyelids; also on genitals and trunk
• Treated by cryotherapy, liquid nitrogen or curettage

2. cryptococcosis: Caused by Cryptococcus neoformans

Erythema ab igne
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Cause

• over exposure to infrared radiation

Sources of heat

• A typical history would be an elderly women who always sits next to an open fire or electric heaters.
• as a response to chronic hot water bottle use

Features

• Limited exposure causes mild and transient red rash resembling lacework or a fishing net
• Prolonged and repeated exposure causes erythematous patches with hyperpigmentation and telangiectasia
• If the cause is not treated then patients may go on to develop squamous cell carcinomas

Treatment

• The source of chronic heat exposure must be avoided
• Topical tretinoin or laser if abnormally pigmented skin.

Seborrhoeic dermatitis in adults
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Seborrhoeic dermatitis in adults is a chronic dermatitis thought to be caused by an inflammatory reaction related to a proliferation of a normal skin inhabitant, a fungus called Malassezia (formerly known as Pityrosporum ovale). It is common, affecting around 2% of the general population

Features

• eczematous lesions on the sebum-rich areas: scalp (may cause dandruff), periorbital, auricular and nasolabial folds
• otitis externa and blepharitis may develop

Associated conditions include

• HIV
• Parkinson's disease

Scalp disease management

• over the counter preparations containing zinc pyrithione ('Head & Shoulders') and tar ('Neutrogena T/Gel') are first-line
• the preferred second-line agent is ketaconazole
• selenium sulphide and topical corticosteroid may also be useful

Face and body management

• topical antifungals: e.g. ketoconazole
• topical steroids: best used for short periods
• difficult to treat - recurrences are common

Dermatitis herpetiformis

• In young adults
• associated with a gluten-enteropathy (coeliac disease)
• ↑risk of small bowel lymphoma and non-Hodgkin’s lymphoma

Features

• severe itching especially at night (which can drive patients to suicide)
• The itch responds to dapsone (may be used as a diagnostic test)
• Blisters, in groups over extensor aspects of limbs, buttocks, nasal cleft and scalp (very similar to distribution of psoriasis).
• Often the blisters are not obvious and the disease may present as excoriated papules
• < 10% exhibit symptoms of an underlying gluten-sensitive enteropathy
• DD: eczema

Diagnosis

• IgA deposits in a granular pattern on the upper surface of the dermis on IF of normal skin (characteristic)
• anti-gliadin antibodies

Treatment

• Dapsone
o 25-200mg/24h PO which will stop the itching within 48h
o in 30% dapsone will need to be continued
o The maintenance dose may be as little as 50mg/wk
o SE (dose related): haemolysis, hepatitis, agranulocytosis (monitor FBC and LFT).
o CI: anaemia; G6PD-deficiency
• gluten-free diet will eradicate the IgA from the dermal papillae in 9–12 months and the itching will stop

Pyoderma gangrenosum
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Causes*

• idiopathic in 50%
• Trauma eg over the operation scar
• IBD: (2%) ulcerative colitis, Crohn's
• rheumatoid arthritis, SLE, Wegener’s granulomatosis
• myeloproliferative disorders
• lymphoma
• myeloid leukaemias (bullous form of pyoderma)
• monoclonal gammopathy (IgA)
• primary biliary cirrhosis
• hyperthyroidism

Features

• typically on the lower limbs
• initially small red papule
• later painful, rapidly spreading, deep, red, necrotic ulcers with a violaceous border
• systemic symptoms e.g. fever, myalgia
• diagnosis should always be considered in non-healing sterile ulcers

Management

• Oral steroids: first-line treatment eg Prednisolone 60 mg od, because of the potential for rapid progression is high in most patients
• topical and intralesional steroids have a role
• immunosuppressive therapy, eg ciclosporin and infliximab, in difficult cases

*note whilst pyoderma gangrenosum can occur in diabetes mellitus it is rare and is generally not included in a differential of potential causes

Onycholysis
sqweqwesf erwrewfsdfs adasd dhe
Onycholysis describes the separation of the nail plate from the nail bed

Causes

• idiopathic
• trauma e.g. excessive manicuring
• infection: especially fungal
• skin disease: psoriasis, dermatitis
• impaired peripheral circulation e.g. Raynaud's
• systemic disease: hyper- and hypothyroidism

Alopecia
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Alopecia may be divided into scarring (destruction of hair follicle) and non-scarring (preservation of hair follicle)

Scarring alopecia

• trauma, burns
• radiotherapy
• lichen planus
• discoid lupus
• tinea capitis

Non-scarring alopecia

• male-pattern baldness
• drugs: cytotoxic drugs, carbimazole, heparin, oral contraceptive pill, colchicine
• nutritional: iron and zinc deficiency
• autoimmune: alopecia areata
• telogen effluvium (hair loss following stressful period e.g. surgery)

Alopecia areata

• autoimmune condition
• at any age and affects both sexes equally

Association

• Hashimoto’s thyroiditis (hence the importance of checking the TFTs)
• Vitiligo
• Myasthenia gravis

Features

• localised, well demarcated patches of hair loss
• skin is normal in appearance but may be pruritus or a burning sensation
• At the edge of the hair loss, short broken hairs that taper at the proximal end (exclamation point hairs) —pathognomonic but not always present
• positive pull test at the periphery indicates that the disease is active
• Precipitating factors
o major life event
o febrile illness
o drugs
o pregnancy
• may be associated with atopic dermatitis

Treatment

• Hair will regrow in 50% of patients by 1 year, and in 80-90% eventually. Careful explanation is therefore sufficient in many patients
• psychological support from family, doctor and support groups

Other treatment options include:

• topical or intralesional corticosteroids
• topical minoxidil
• phototherapy
• dithranol
• contact immunotherapy
• wigs

Alopecia totalis: loss of all scalp hair and eyebrows
Alopecia universalis: complete loss of body hair
Trichotillomania: psychological disorder where patients are compelled to pull out their hair.

Acanthosis nigricans
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Describes symmetrical, brown, velvety plaques that are often found on the neck, axilla and groin (occasionally on dorsum of the hand)

Causes

• internal malignancy (usually GI especially adenocarcinoma of stomach)
• insulin-resistant diabetes mellitus
• obesity
• acromegaly
• Cushing's disease
• hypothyroidism
• polycystic ovarian syndrome
• familial
• Prader-Willi syndrome
• drugs: oral contraceptive pill, nicotinic acid

Skin disorders associated with SLE
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Skin manifestations of systemic lupus erythematosus

• photosensitive 'butterfly' rash
• discoid lupus
• scarring alopecia
• livedo reticularis: net-like rash

Skin disorders associated with diabetes
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Note whilst pyoderma gangrenosum can occur in diabetes mellitus it is rare and is often not included in a differential of potential causes

Necrobiosis lipoidica

• shiny, painless areas of yellow/red/brown skin typically on the shin
• often associated with surrounding telangiectasia

Infection

• candidiasis
• staphylococcal

Neuropathic ulcers

Vitiligo

Lipoatrophy

Granuloma annulare*

• papular lesions that are often slightly hyperpigmented and depressed centrally

*it is not clear from recent studies if there is actually a significant association between diabetes mellitus and granuloma annulare, but it is often listed in major textbooks

Erythema chronicum migrans

Cause

• Lyme disease: tick-borne infection caused by Borrelia burgdorferi

Features

• Presents as a small papule which develops into a spreading large erythematous ring, with central fading
• usually 7-10 days (range 1-33 days) after a tick bite
• It lasts from 48h to 3 months
• May be multiple

Skin complications

• acrodermatitis chronica atrophicans (ACA; skin is as thin as cigarette paper)
• borellia lymphocytoma manifests eg as a blue/red discolouration of the earlobe

CNS

• Cognition↓
• meningitis
• ataxia
• amnesia
• cranial nerve palsies eg Bell's palsy
• neuropathy
• lymphocytic meningoradiculitis (Bannwarth's syndrome).

Heart

• myocarditis
• heart block

others

• Lymphadenopathy
• arthralgia/arthritis

Diagnosis

• Clinical ± serology; if -ve do, PCR/LP.

Treatmrnt

• Skin rash: doxycycline 100mg/12h PO (amoxicillin or penicillin V if <8yrs or pregnant) for 14-21d.
• Later complications: high-dose IV benzylpenicillin, ceftriaxone.

Prevention

• Keep limbs covered
• use insect repellent
• tick collars for pets
• heck skin often when in risky areas
• Vaccination if living in high-risk areas.
• prophylaxis after a tick bites. A single dose of doxycycline 200mg PO given within 72h of a bite

Removing ticks

• Suffocate tick with, eg petroleum jelly, then remove by grasping close to mouth parts and twisting off; then clean skin.

Ixodes tick

Cutaneous larva migrans (creeping eruption)

• The infection acquired by direct contact with dog and cat faeces – often acquired when sunbathing on contaminated sand. The larvae burrow in the dermoepidermal junction

Cause

• Ancylostoma braziliense (animal hookworm, most common)
• human nematodes: Strongyloides stercoralis, Necator americanus Ancylostoma duodenale

Features

• pruritis
• raised, serpiginous erythematous rash migrates at a rate of up to 1 cm/day

Treatment

• topical thiobendazole
• oral albendazole

Granuloma annulare

• common condition of unknown cause

Associations

• diabetes mellitus
• lymphoma
• HIV infection
• solid tumours

Features

• more common in children and young adults
• Women : men = 2:1
• ring of small smooth papules, which enlarge centrifugally
• pearly white, skin-colored, red, or purple
• occur singly or in groups
• on tops of the hands and feet, elbows, and knees
• usually does not peel or itch
• usually resolve over 2–3 years

Diagnosis

• Clinical

Treatment

• Stubborn lesions respond to intralesional trimacinolone or systemic immunosuppressants.

Skin disorders associated with TB
sqweqwesf erwrewfsdfs adasd dhe
Possible skin disorders

• lupus vulgaris (accounts for 50% of cases)
• erythema nodosum
• scarring alopecia
• scrofuloderma: breakdown of skin overlying a tuberculous focus
• verrucosa cutis
• gumma

Lupus vulgaris

• most common form of cutaneous TB
• due to spread from an endogenous source
• seen in the Indian subcontinent.
• > 80% on the face and neck, common around the nose and mouth
• Lesions begin as papules and coalesce to form an erythematous flat plaque
• The centre of lesion consists of scar tissue
• apple-jelly nodules are classically described at margins of lesions
• gradually becomes elevated and may ulcerate later.
• Treatment is the same as that for pulmonary tuberculosis

Henoch-Schonlein purpura
• sqweqwesf erwrewfsdfs adasd dhe
IgA mediated small vessel vasculitis.
• degree of overlap with IgA nephropathy (Berger's disease).
• usually seen in children following an infection.
• The most likely precipitant is a Group A Streptococcal infection

Features

• palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs
• abdominal pain
• Gastrointestinal bleeding
• Polyarthritis
• Perivascular immunoglobulin A (IgA) deposits
• features of IgA nephropathy may occur e.g. haematuria, renal failure

Skin disorders associated with malignancy
sqweqwesf erwrewfsdfs adasd dhe
Paraneoplastic syndromes associated with internal malignancies:

• acanthosis nigricans - gastrointestinal cancer
• acquired ichthyosis - lymphoma
• acquired hypertrichosis lanuginosa - gastrointestinal and lung cancer
• dermatomyositis - bronchial and breast cancer
• erythema gyratum repens - lung cancer
• erythroderma: lymphoma
• migratory thrombophlebitis - pancreatic cancer
• necrolytic migratory erythema - glucagonoma
• pyoderma gangrenosum (bullous and non-bullous forms) - myeloproliferative disorders
• Sweet's syndrome - haematological malignancy e.g. myelodysplasia - tender, purple plaques. also known as acute febrile neutrophilic dermatosis has a strong association with acute myeloid leukaemia

Vitamin C deficiency

• follicular keratosis
• coiling of hair.
• perifollicular haemorrhages
• bleeding gums
• purpura over the legs.
• anaemia
• plasma ascorbic acid levels ↓

Lichen planus
sqweqwesf erwrewfsdfs adasd dhe
Lichen planus is a skin disorder of unknown aetiology, most probably being immune mediated

Association

• Vitiligo
• Alopecia areata
• Ulcerative colitis

Features

• violaceous or lilac-coloured, intensely itchy, flat-topped papules often polygonal in shape, fine white streaks on the surface of the lesions if viewed through a hand lens (Wickham's striae)
• most common on the palms, soles, genitalia and flexor surfaces of arms
• + mucous membrane involvement
• Koebner phenomenon seen: refers to the appearance of lesions along a site of injury. This phenomenon is seen in e.g., lichen planus, warts, molluscum contagiosum, psoriasis, lichen nitidus, and the systemic form of juvenile rheumatoid arthritis.
• Genital skin is similarly affected in the absence of oral, nail or other skin findings
• Oral lesions: asymptomatic white lacy lines, dots or plaques. 50%
• nails: thinning of nail plate, longitudinal ridging and destruction of the nail bed. (not onycholysis) 10%
• usually resolves spontaneously in a few months

Lichenoid drug eruptions - causes:

• thiazides
• β-blockers
• phenothiazines
• antimalarials
• methyldopa
• gold
• quinine

Treatment

• Potent topical steroids are the treatment of choice: clobetasol proprionate and betamethasone proprionate ointments for 4 -6 week

Lichen sclerosus
sqweqwesf erwrewfsdfs adasd dhe
Overview

• inflammatory condition which often affects genitals
• more common in elderly females
• leads to atrophy of epidermis, white plaques may form increases risk of vulval cancer

Zinc deficiency
sqweqwesf erwrewfsdfs adasd dhe
Features

• perioral dermatitis: red, crusted lesions
• acrodermatitis: Inflamation of the skin of the extremeties
• alopecia
• short stature
• hypogonadism
• hepatosplenomegaly
• geophagia (ingesting clay/soil)
• cognitive impairment

Contact dermatitis
sqweqwesf erwrewfsdfs adasd dhe
There are two main types of contact dermatitis

• irritant contact dermatitis: common - non-allergic reaction due to weak acids or alkalis (e.g. detergents). Often seen on the hands. Erythema is typical, crusting and vesicles are rare
• allergic contact dermatitis: type IV hypersensitivity reaction. Uncommon - often seen on the head following hair dyes. Presents as an acute weeping eczema which predominately effects the margins of the hairline rather than the hairy scalp itself. Topical treatment with a potent steroid is indicated

Cement is a frequent cause of contact dermatitis. The alkaline nature of cement may cause an irritant contact dermatitis whilst the dichromates in cement also can cause an allergic contact dermatitis

Pruritus
sqweqwesf erwrewfsdfs adasd dhe
Causes of generalised pruritus

• liver disease
• iron deficiency anaemia and polycythaemia
• chronic renal failure
• hyper- and hypothyroidism
• diabetes
• pregnancy
• malignancy: Hodgkin's, other underlying malignancy
• drugs: morphine
• 'senile' pruritus

Pityriasis rosea
sqweqwesf erwre
Overview

• cause unknown, herpes hominis virus 7 (HHV-7) a possibility
• tends to affect young adults

Features

• herald patch (usually on trunk): a single scaling patch often appears 1-20 days before the general rash. It is an oval plaque 2-5 cm in diameter, with a scale trailing just inside the edge of the lesion
• followed by erythematous, oval, scaly patches which follow the line of the ribs like a fir tree. They have a dry surface and may have an inner circlet of scaling. The plaques may be a faint pink to a deep red. They may be very itchy, but in most cases they don't itch at all.

Management

• self-limiting, usually disappears after 4-6 weeks

Pityriasis versicolor
sqweqwesf erwrewfsdfs adasd dhe
Pityriasis versicolor, also called tinea versicolor, is a superficial cutaneous fungal infection caused by Malassezia furfur

Cause

• infection with a yeast, Pityrosporum orbiculare, which we all have on our skin as a harmless commensal. Under certain conditions, the yeast produces hyphae and becomes pathogenic when it is known as Malassezia globosa

Features

• young adults
• most commonly affects trunk, neck, and/or arms
• patches may be hypopigmented, pink or brown (hence versicolor)
• small, less than 1 cm in diameter
• usually round
• scale is common (when scratched)
• mild pruritus

Predisposing factors

• occurs in healthy individuals
• immunosuppression
• malnutrition
• Cushing's

Management

• topical antifungal e.g. terbinafine or selenium sulphide
• if extensive disease then consider oral itraconazole

Erythema nodosum

• inflammation of subcutaneous tissue (panniculitis)
• Peak incidence 20–30 year
• females: males 3–6:1

Pathology

• vasculitis of small venules and panniculitis

Causes

• infection: streptococci (most common), upper respiratory tract infections, TB, histoplasmosis, coccidioidomycosis, psittacosis, cat scratch fever, Yersinia infection, brucellosis, Salmonellosis, Chlamydial infection, infectious mononucleosis
• systemic disease: sarcoidosis, inflammatory bowel disease, Behcet's
• malignancy/lymphoma
• drugs: penicillins, sulphonamides, oral contraceptive pill, aspartame, bromides, iodides
• pregnancy

Features

• typically causes tender, erythematous, nodules
• usually occurs over shins, may also occur elsewhere (e.g. forearms, thighs)
• usually resolves within 6 weeks
• lesions heal without scarring

Management

• Oral NSAID whilst investigations are carried out
• in severe cases: colchicine, dapsone or potassium iodide
• Systemic steroids: rapid response, should only be given when diagnosis is known, and infection is excluded

Venous ulceration
sqweqwesf erwrewfsdfs adasd dhe
Venous ulceration is typically seen above the medial malleolus

Investigations

• ankle-brachial pressure index is important in none-healing ulcers to assess for poor arterial flow which could impair healing

Management

• compression bandaging, usually four layer (only treatment shown to be of real benefit)
• oral pentoxifylline, a peripheral vasodilator, improves healing rate
• small evidence base supporting use of flavinoids
• little evidence to suggest benefit from hydrocolloid dressings, topical growth factors, ultrasound therapy and intermittent pneumatic compression

Koebner phenomenon

• appearance of a rash in a scar or in the site of an injury
• usually 7 to 14 days after the injury
• pseudo Koebner is used for conditions such as molluscum contagiosum and viral warts where the phenomenon arises from the spread of an infective agent.
• The reverse Koebner also exists where trauma to a lesion results in its resolving.
sqweqwesf erwrewfsdfs adasd dhe
Koebner phenomenon is seen in

• psoriasis (20%)
• lichen planus
• vitiligo
• warts
• lichen sclerosus
• molluscum contagiosum

Systemic mastocytosis
sqweqwesf erwrewfsdfs adasd dhe
Systemic mastocytosis results from a neoplastic proliferation of mast cells

Features

• young person
• urticaria pigmentosa - produces a wheal on rubbing (Darier's sign)
• flushing
• abdominal pain
• monocytosis on the blood film

DD

• Carcinoid syndrome: average age is 61 years

Diagnosis

• urinary histamine

Erythema multiforme (EM)

Causes

• idiopathic
• bacteria: Mycoplasma, Streptococcus
• viruses: herpes simplex virus (commonest), Orf
• drugs: Barbiturates, penicillin, sulphonamides, carbamazepine, allopurinol, NSAIDs, oral contraceptive pill, nevirapine
• connective tissue disease e.g. systemic lupus erythematosus, PAN
• ulcerative colitis-not Crohn's
• sarcoidosis
• malignancy

Features

• preceding upper respiratory infection
• symmetrical, non-pruritic, erythematous maculopapular rash occur in crops on the limbs and trunk
• annular plaques: classic target lesion has 3 zones of color: central dusky purpura or a central bulla, a surrounding edematous pale zone, and a surrounding macular erythema. (characteristic)
• May develop into vesicles or bullae
• Involves skin and mucus membranes (one mucosal surface)
• Lesions resolve over 3-6 weeks but may recur.

Treatment

• identify and remove the cause
• Usually no treatment, simple dressings→ symptomatic relief
• Corticosteroids are best avoided

Stevens-Johnson syndrome (SJS)
sqweqwesf erwrewfsdfs adasd dhe
Causes

• as erythema multiforme

Features

• Systemic symptoms: such as fever, cough, or sore throat, may appear 1-3 days prior to any cutaneous lesions
• Target lesion
o central dusky purpura or a central bulla, with surrounding macular erythema.
o Lesions begin symmetrically on the face and the upper part of the torso and extend rapidly
o epidermal detachment is limited to less than 10% of the BSA
• Mucous membrane involvement
o two or more mucous membranes
o Painful oral erosions cause severe crusting of the lips, increased salivation.
o Lesions have been reported in the oropharynx, the tracheobronchial tree, the esophagus, the GI tract, the genitalia, and the anus

• Ocular lesions
o Initially, the conjunctivae are erythematous and painful
o Keratitis, corneal erosions, and a siccalike syndrome

Treatment
• Admission to hospital
• Stop all medications
• Fluid replacement.
• Nutritional supplementation
• Sterile technique
• Wound care: Debridement of all necrotic epidermis with replacement by using biologic dressings, such as collagen-based substitutes or porcine xenografts
• Oral care and application of antiseptics: Chlorhexidine mouthwashes
• Frequently applied eye drops with daily blunt disruption of synechiae.
• environmental temperature should be increased to 30-32°C. Heated antiseptic baths, heat shields, infrared lamps, and air-fluidized beds may decrease heat losses.

Toxic epidermal necrolysis (TEN)

• Idiosyncratic
• potentially life-threatening skin disorder
• most commonly is drug induced
• blistering and peeling of the top layer of skin
• a severe form of Stevens-Johnson syndrome
• very rare

Causes

• Drugs
• Antibiotics
o Sulphonamides
o Penicillins
o Macrolides
o Quinolones
• Allopurinol
• NSAIDs
• Anticonvulsants (eg Carbamazepine)
• Infection
• Malignancy
• Vaccinations
• Idiopathic (one-third)

Features

2-3 days of flu-like symptoms followed by the critical phase (8-12 days)

• Persistent fever
• Conjunctivitis (1-3 days before skin lesions)
• a painful, red area that spreads quickly
• the top layer of skin may peel without blistering
• scalded-looking raw areas of flesh
• Cracked, bleeding lips that form crusts
• Extreme pain

Complications

• dehydration and malnutrition
• Bacterial skin infections
• Conjunctival sloughing, blindness
• Pneumonia
• renal failure
• septicaemia
• Shock and multiple organ failure.

SCORTEN

an illness severity score that has been developed to predict mortality in SJS and TEN cases. One point is scored for each

• Age >40 years
• Presence of a malignancy (cancer)
• Heart rate >120
• Initial percentage of epidermal detachment >10%
• Serum urea level >10 mmol/L
• Serum glucose level >14 mmol/L
• Serum bicarbonate level <20 mmol/L

Mortality rates have been predicted to be as follows:

• SCORTEN 0-1 > 3.2%
• SCORTEN 2 > 12.1%
• SCORTEN 3 > 35.3%
• SCORTEN 4 > 58.3%
• SCORTEN 5 or more > 90%

DD

• Staphylococcal scalded skin syndrome: skin biopsy distinguish these two diseases
o SJS/TEN: Full-thickness epidermal necrosis
o Staphylococcal scalded skin syndrome: Subcorneal split

Treatment

• Any potentially responsible drug should be withdrawn as quickly as possible
• Fluid and electrolyte resuscitation
• Intravenous antibiotics for infection
• Pain management
• Nutritional support
• Wound care
• nursing on air-fluidised mattresses, as the skin is so fragile
• Surgical debridement (removal) of dead tissue
• Plasmapheresis

DD of TEN, SJS, EM

Toxic epidermal necrolysis (TEN)
• acute dermatologic disease
• Nearly all cases are induced by medications
• widespread erythematous macules and targetoid lesions
• involvement of more than 30% of the cutaneous surface
• full-thickness epidermal necrosis
• Commonly, the mucous membranes are also involved
• mortality rate 40%.
Stevens-Johnson syndrome (SJS)
• dermatologic emergency
• As with TEN, medications are important inciting agents, although Mycoplasma infections may induce some cases
• purpuric macules and targetoid lesions
• full-thickness epidermal necrosis
• with lesser detachment of the cutaneous surface
• mucous membrane involvement.
• The mortality rate is much lower and approaches 5%
Erythema multiforme (EM)
• recurrent benign process
• target or targetoid lesions, with or without blisters, in a symmetric acral distribution.
• Oral lesions are common
• Severe presentations may have widespread involvement of the mucous membranes and epidermal detachment with a loss of less than 10% of the cutaneous surface.
• Most cases are secondary to prior infection with a herpes virus.
• low morbidity and no mortality

Hirsuitism and hypertrichosis
sqweqwesf erwrewfsdfs adasd dhe
Hirsuitism is often used to describe androgen-dependent hair growth in women, with hypertrichosis being used for androgen-independent hair growth

Causes of hirsuitism

• polycystic ovarian syndrome
• Cushing's syndrome
• congenital adrenal hyperplasia
• androgen therapy
• adrenal tumour
• androgen secreting ovarian tumour

Causes of hypertrichosis

• drugs: minoxidil, ciclosporin, phenytoin, diazoxide
• congenital hypertrichosis lanuginosa, congenital hypertrichosis terminalis
• porphyria cutanea tarda
• anorexia nervosa

Actinic keratoses
sqweqwesf erwrewfsdfs adasd dhe
Actinic (solar) keratoses is a common premalignant skin lesion that develops as a consequence of chronic sun exposure

Features

• small, crusty or scaly, lesions
• may be pink, red, brown or the same colour as the skin
• typically on sun-exposed areas e.g. temples of head
• multiple lesions may be present

Management options include

• 5-FU cream: typically a 1 to 3 week course. The skin will become red and inflammed - sometimes topical hydrocortisone is given following 5-FU to help settle the inflammation
• prevention of further risk: e.g. sun avoidance, sun cream
• cryotherapy
• curettage and cautery

Eczema

diagnosis
sqweqwesf erwrewfsdfs adasd dhe
UK Working Party Diagnostic Criteria for Atopic Eczema

An itchy skin condition in the last 12 months

Plus three or more of

• onset below age 2 years*
• history of flexural involvement**
• history of generally dry skin
• personal history of other atopic disease***
• visible flexural dermatitis

*not used in children under 4 years
**or dermatitis on the cheeks and/or extensor areas in children aged 18 months or under
***in children aged under 4 years, history of atopic disease in a first degree relative may be included

Pemphigus vulgaris
sqweqwesf erwrewfsdfs adasd dhe
Overview

• autoimmune disease against desmoglein-3
• more common in the Ashkenazi Jewish population
• seen predominantly in elderly

Association

• HLA DR4
• HLA DRw6
• Myasthenia gravis
• Thymoma

Features

• mucosal ulceration, Most patients first present with lesions on the mucous membranes such as the mouth and genitals (in some, the only manifestation)
• truncal blistering (Several months later): thin walled flaccid blisters filled with clear fluid that easily rupture causing painful erosions
• Nikolsky’s sign is +ve: fluid filled blisters spread with lateral pressure

Diagnosis

• acantholysis on biopsy: rounded-up separated keratinocytes within the blisters just above the basal layer of the epidermis.
• confirmed by direct immunofluorescence to reveal deposition of IgG directed against intercellular cement - resulting in a 'chicken wire' appearance
• In most cases, circulating antibodies can be detected by a blood test (indirect immunofluorescence test

Causes of intraepidermal blistering

• friction
• eczema – multilocular
• infection
• toxic epidermal necrolysis
• Pemphigus vulgaris

Causes of subepidermal blistering

• Burns
• Pemphigoid
• dermatitis herpetiformis
• erythema multiforme
• insect bites
• porphyria cutanea tarda
• epidermolysis bullosa

Management

• Steroids: high-dose steroids (Prednisolone 70 mg od) to induce remission and then may require long-term lower dose steroids for maintenance, usually for around 2 years
• Immunosuppressants: to minimise steroid use. These include:
o Azathioprine
o Cyclophosphamide
o Dapsone
o Tetracyclines
o Nicotinamide
o Plasmapheresis
o Gold
o Mycophenolate mofetil
o Intravenous immunoglobulin
o Anti-CD20 monoclonal antibody (rituximab)

Prognosis

• The mortality reduced to 5–15%.

Bullous pemphigoid

• autoimmune condition
• Blistering at subepidermal level – deeper than blisters of pemphigus vulgaris
• IgG autoantibodies against components of basement membrane (hemidesmosomal proteins BP180 and BP230)
• more common in elderly (>60 y)

Features

• itchy, tense blisters typically around flexures
• mouth is usually spared
• Nikolsky’s sign is negative

Skin biopsy

• immunofluorescence shows IgG and C3 at the dermoepidermal junction

Management

• referral to dermatologist for biopsy and confirmation of diagnosis
• Oral corticosteroids are the mainstay of treatment
• topical corticosteroids, immunosuppressants and antibiotics are also used

Photosensitive skin disorders
sqweqwesf erwrewfsdfs adasd dhe
Diseases aggravated by exposure to sunlight

• systemic lupus erythematosus, discoid lupus
• porphyria (not acute intermittent)
• herpes labialis (cold sores)
• pellagra
• xeroderma pigmentosum
• solar urticaria
• polymorphic light eruption

Drugs causing photosensitivity
sqweqwesf erwrewfsdfs adasd dhe
Causes of drug-induced photosensitivity

• thiazides
• tetracyclines, sulphonamides, ciprofloxacin
• amiodarone
• NSAIDs e.g. piroxicam
• psoralens
• sulphonylureas

Porphyria cutanea tarda

• most common hepatic porphyria.

Cause

• inherited defect in uroporphyrinogen decarboxylase
• hepatocyte damage e.g. alcohol, oestrogens, iron

Features

• classically presents with photosensitive rash with blistering and skin fragility (erosions) on the face and dorsal aspect of hands (most common feature). lesions heal slowly, leaving scars
• hypertrichosis
• hyperpigmentation
• scarring alopecia following resolution of bullae on the scalp

DD

• Phototoxic drug reaction: caused by all quinolones including ciprofloxacin, well-demarcated erythema and large blisters after sun exposure
• Polymorphic light eruption: affects all exposed areas and often results in blistering e.g. on the face. It is usually preceded by a long history of sun intolerance.

Investigations

• urine: elevated uroporphyrinogen and pink fluorescence of urine under Wood's lamp

Management

• Chloroquine: promotes porphyrin excretion
• Venesection: (450ml/week) until haemoglobin 12g/d

Discoid LE

• chronic, scarring, photosensitive dermatosis.
• small number may later develop SLE and DLE can occur as a feature of SLE
• usually occurs as a skin manifestation alone without significant systemic features

Features

• red scaly patches which leave postinflammatory pigmentation and white scars.
• It may be localised or widespread
o predominantly affects the cheeks, nose and ears, but sometimes involves the upper back, V of neck, and backs of hands.
o Hypertrophic LE results in thickened and warty skin
o palmoplantar LE rare
o scalp: adherent scale and then bald areas
o lips and inside the mouth, causing ulcers and scaling. These lesions may predispose to squamous cell carcinoma.
• somrtimes positive autoantibodies (Rh factor) +/– low complement levels.
• Arthralgia
• pruritis at the site of the lesions

Treatment

• sun protection
• topical corticosteroids
• oral antimalarial agents
• systemic immunosuppressive therapy in severe cases

Yellow nail syndrome
sqweqwesf erwrewfsdfs adasd dhe

Triad

• thickened, curved and yellow nails: due to Slowing of nail growth
• congenital lymphoedema
• pleural effusions

Others

• bronchiectasis 40%
• chronic sinus infections
• COPD
• Pulmonary neoplasm.
• nephrotic syndrome
• penicillamine use

Erythema gyratum repens

Causes

• malignancy
o in up to 80%
o Lung cancer is the most common particularly in a patient who is a smoker
o also breast, bladder, cervical, stomach, and prostate cancer
o Most patients develop the eruption before the symptoms of underlying malignancy. The time interval for this may be up to 6 years.
• pulmonary TB
• SLE
• CREST syndrome
• Psoriasis
• as a drug reaction to azathioprine in a patient with type I autoimmune hepatitis.
• Idiopathic

Features

• concentric mildly scaling bands of flat-to-raised erythema forming a wood-grain appearance (Characteristic)
• Fairly rapid migration (up to 1 cm/d)
• Intense pruritus
• On arms, legs and trunk
• Associated ichthyosis and palmoplantar hyperkeratosis
• In most patients, symptoms disappear with the resolution of underlying disease

[/b]

_________________
DR ALRAZI

DR ALRAZI · Posted: Sat Feb 28, 2009 2:38 am Post subject:

DERMATOLOGY 2
Nickel dermatitis
sqweqwesf erwrewfsdfs adasd dhe
Nickel is a common cause allergic contact dermatitis and is an example of a type IV hypersensitivity reaction. It is often caused by jewellery such as watches

It is diagnosed by a skin patch test

Scabies

• caused by the mite Sarcoptes scabiei
• spread by prolonged skin contact
• typically affects children and young adults.

pathology

• The scabies mite burrows into the skin, laying its eggs in the stratum corneum.
• The intense pruritus associated with scabies is due to a delayed type IV hypersensitivity reaction to mites/eggs which occurs about 30 days after initial infection

Features

• widespread pruritus: at night when the patient is warm
• linear burrows (pathgnomic): S-shaped papules 3–5 mm in length on side of fingers, interdigital webs and flexor aspects of the wrist
• Generalised rash: hypersensitivity reaction in the form of tiny red intensely itchy bumps on the limbs and trunk
• nodules:Itchy lumps or nodules in the armpits and groins or along the shaft of the penis
• Acropustulosis: Blisters and pustules on the palms and soles are characteristic of scabies in infants
• Secondary infection: impetiogo, cellulitis
• in infants the face and scalp may also be affected
• secondary features due to scratching: excoriation, infection

Crusted scabies (Norwegian scabies)

• affect individuals with a poor immune system, neurological diseases, the elderly or those with mental incompetence.
• It is the usual cause of severe outbreaks of scabies in an institution such as a hospital, rest home or prison

Features

• very contagious in which there are thousands or even millions of mites,
• very little itch.
• generalised scaly rash. (misdiagnosed as psoriasis).
• may affect the scalp. .
• People in contact become very itchy, with tiny red spots and blisters on exposed areas;

Management

• anti-scabetic (Permethrin cream, topical Benzyl Benzoate or malathion) to ALL skin from neck downward for 24 hr - include head if < 2 years old
• treat all members of household
• clothing and bedding should be cleaned by hot washing
• pruritus persists for up to 4-6 weeks post eradication

Tinea capitis

• Infection of the scalp with a dermatophyte fungus
• most between 3 and 7 years
• slightly more common in boys than girls
• suspected if there is a combination of scale and bald patches

Causes

• The causative fungi belong to three genera (Microsporum (M), Trichophyton (T), Epidermophyton)
• they can originate from the soil (geophilic) or animals (zoophilic), or be confined to human skin (anthropophilic).

Types

• Ectothrix infection: (outside the hair shaft) species of fungi, such as M. audouinii (anthropophilic),; M. canis (from dogs and cats) can be identified by green fluorescence with Wood's light
• Endothrix infection (within the hair shaft) infections, e.g. T. tonsurans,. There is no fluorescence under Wood's light.
• Favus: It is caused by T. schoenleinii infection, which results in a honeycomb destruction of the hair shaft.

Clinical features

• Tinea capitis may present in several ways
o Dry scaling – like dandruff but usually with moth-eaten hair loss
o Black dots – the hairs are broken off at the scalp surface, which is scaly
o Smooth areas of hair loss
o Kerion – very inflamed mass, like an abscess
o Favus – yellow crusts and matted hair
o Carrier state no symptoms and only mild scaling (T. tonsurans).
• Swollen lymph glands at the sides of the back of the neck
• Permanent scarring (bald areas) in untreated kerion and favus
• Id reaction (allergic rash at a distant site), especially just after starting antifungal treatment

Diagnosis

• If the child has an anthropophilic infection, all family members should be examined for signs of infection
• confirmed by microscopy and culture of skin scrapings and hair pulled out by the roots

Treatment

• Oral terbinafine, itraconazole (4-6 weeks) or griseofulvin (3 months)
• Antifungal shampoo as adjunct
o 2.5% selenium sulfide
o 1-2 % zinc pyrithione
o Povidone-iodine
o 2% ketoconazole
• Arachis oil: to remove crusting
• Oral corticosteroids: to reduce the inflammation in Kerions + oral antifungal
• Treatment of carriers (no symptoms)
o Antifungal shampoo twice weekly for four weeks
o Oral treatment: if cultures remain positive

Tinea corporis (Ringworm)

(involvement of the body)

Cause

• M. canis and T.verrucosum (from dogs and cattle respectively).

Features

Classically, the lesions are

• Erythematous
• annular
• scaly
• well-defined edge
• central clearing
• single or multiple
• usually asymmetrical

Treatment

• Topical terbinafine or imidazoles (e.g. ketoconazole, clotrimazole)

Tinea incognito

• Tinea corporis in which the clinical appearance has been altered by inappropriate treatment, usually a topical steroid cream

Features

Compared with an untreated tinea corporis, tinea incognito:

• less raised margin
• less scaly,
• More pustular
• More extensive
• more irritable

There may also be secondary changes caused by long term use of a topical steroid such as:

• Atrophy (thin skin, stretch marks (striae) in the skin folds).
• Purpura (bruising) and telangiectasia (broken blood vessels).

Treatment

• The topical steroid should be discontinued
• Bland antipruritic lotions
• Standard antifungal treatment

Tinea cruris (groin involvement)

Cause

• Infection often comes from the feet or nails
• spread by scratching or the use of an infected towel
• T. rubrum

Features

• rash has a scaly raised red border
• can be very itchy
• spreads down the inner thighs from the groin or scrotum*
• may be on buttocks, penis or vulva or around the anus
• In acute infections, the rash may be moist and exudative

* The scrotum is usually spared in distinct contrast with infections of this area by Candida

Tinea pedis (athlete's foot)

• most common form of ringworm in the UK and USA

Cause

anthropophilic fungi such as

• T. rubrum,
• T. interdigitale, previously called T. mentagrophytes
• Epidermophyton floccosum

Clinical features

• Chronic hyperkeratotic tinea: patchy fine dry scaling on the sole of the foot
• Moccasin tinea: extensive hyperkeratotic tinea, in which the skin of the entire sole, heel and sides of the foot is dry but not inflamed. The affected area does not include the top of the foot. This is usually caused by T. rubrum.
• Athlete's foot i.e. moist peeling irritable skin between the toes, most often in the cleft between the fourth and fifth toes.
• Clusters of blisters or pustules on the sides of the feet or insteps (more likely with T. interdigitale).
• Round dry patches on the top of the foot (ringworm like tinea corporis).

Diagnosis

• In all cases of suspected dermatophyte infection, the diagnosis should be confirmed by KOH treated skin scraping or nail clippings

Management

• topical (terbinafine or miconazole cream) or
• systemic (terbinafine, griseofulvin or itraconazole).

Impetigo

• common superficial infection of the epidermis
• outbreaks in nurseries and schools are not rare
• eczema and infestations, predispose to it.

Non-bullous impetigo

• young children, often in late summer.
• usually due to Staphylococcus aureus, a group A beta-haemolytic streptococcus or a mixture of both.
• very contagious
• usually affect the face and the limbs
• typically: thin-walled vesicle which ruptures rapidly with the formation of golden crust on an erythematous base
• less commonly: glazed erythema
• Lesions may be single but often rapidly become multiple and coalesce

Bullous impetigo

• primarily caused by Staphylococcus
• most frequently in children
• Bullae (often quite large) last for 2-3 days
• initially clear and then cloudy.
• blisters are caused by a staphylococcal epidermolytic toxin.
• Once the blisters have burst, crusts develop

Diagnosis

• Swab for microbiological

Management (based on current British National Formulary guidelines)

• small, localised patches: Topical antibiotics eg 2% fusidic acid (Fucidin), 2% mupirocin (Bactroban), or 0.3% neomycin ointment
• extensive disease: Oral flucloxacillin (erythromycin if penicillin allergic)
• Oral penicillin V is useful in parts of the world where impetigo is due to a group A beta-streptococcus, to prevent acute glomerulonephritis

Erythrasma

Cause

• bacterial infection: Corynebacterium minutissimum

Features

• no symptoms
• slowly enlarging area of pink or brown dry skin.
• in the skin folds such as under the arms, in the groin and between the toes

Diagnosis

• coral pink fluorescence with Wood's light due to porphyrins released by the bacteria.
• confirmed by a swab or scraping for microscopy and culture

Treatment

• Antiseptic or
• topical antibiotic such as:
o Fusidic acid cream
o Clindamycin solution
o Whitfield's ointment
• oral antibiotics: erythromycin or tetracycline, in extensive infection
• Antibacterial soap: to prevent recurrence.

Cellulitis

Cause

• Staphylococcus aureus & Streptococci (commonest)
• Group B Streptoccus has a prediliction for diabetic patients

Erysipelas

Cause

• Streptococcus pyogenes infection of deep dermis and subcutis.

Complications

• Sepsis
• cerebral abscess
• venous sinus thrombosis

Treatment

• IV antibiotics such as Benzylpenicillin and Erythromycin if penicillin allergic

Cutaneous anthrax

• commonest form of infection in humans
• usually due to contact with infected animals or animal products
• Anthrax endemic to herd animals in some parts of world

Cause

• Bacillus anthracis

Features

• evolves over a period of ~2 weeks into a papule, pustule, vesicle
• eventually an ulcer with a central black eschar
• surrounding skin usually boggy oedematous.
• Lesions are usually painless with tender regional lymph nodes.
• 80-90% heal spontaneously, 10-20% become bacteraemic – associated a high mortality

Treatment

• Penicillin

Albinism

• a group of inherited abnormalities of melanin synthesis characterized by a congenital reduction or absence of melanin pigment in association with specific developmental changes in optic system resulting from hypopigmentation
• on fundus exam: The albino macula is always hypoplastic

Classification

• Oculocutaneous albinism (OCA) types 1–4
• Ocular albinism
• Chediak–Higashi syndrome
• Hermansky–Pudlak syndrome
• Griscelli syndrome

Features

• OCA 1A (Tyrosinase-related)
o complete absence of melanin in the hair, the skin and the eyes
o complete lack of tyrosinase activity
o photophobia
o reduction in visual acuity (moderate to severe)
o severe nystagmus
o both irises have a pink hue
• OCA 1B (Tyrosinase-related)
o moderate pigmentation in these same tissues
o reduced activity of tyrosinase
o associated eye problems are not apparent
• The remaining forms of OCA reflect an increased presence of melanin in the tissues
o P-gene related OCA2: most common type of albinism,
o frequent among African Americans (1 in 10,000)and Africans.
o 1 in 36,000 in Caucasians
• Ocular albinism
o almost normal skin and hair colour (tend to be lighter than their siblings especially in dark skin)
o iris transillumination defects
o congenital motor nystagmus
o reduced visual acuity
o refractive errors
o fundus hypopigmentation
o lack of foveal reflex
o strabismus

• Chediak-Higashi Syndrome
o Autosomal recessive
o silvery sheen skin
o blue brown irises
o increased susceptibility to infection
o hepatosplenomegaly
o lymphadenopathy
o predisposition to development of a lymphoma-like condition.

• Hermansky-Pudlak Syndrome
o Autosomal recessive.
o absence of platelet dense-bodies, resulting in a loss of secondary aggregation of platelets after stimulation a predisposition to bruising and bleeding which
o can be severe.
o higher frequency in Puerto Rico.

DD

• Homocystinuria
o inherited autosomal recessive defect
o defective methionine metabolism.
o long thin extremities
o arachnodactyly
o pale skin and hair
• Phenylketonuria
o inherited autosomal recessive condition
o defect in phenylalanine metabolism.
o now routinely screened for at birth
o fair hair and skin

_________________
DR ALRAZI

DR ALRAZI · Posted: Sat Feb 28, 2009 2:40 am Post subject:

DERMATOLOGY 3

Keratoacanthoma (KA)

• relatively common low-grade malignancy
• originates in pilosebaceous glands
• resembles squamous cell carcinoma (SCC) pathologically
• rapid growth over a few weeks to months, followed by spontaneous resolution over 4-6 months in most cases.
• Lesions typically are solitary
• begin as firm, roundish, skin-colored or reddish papules
• rapidly progress to dome-shaped nodules with a smooth shiny surface and a central crateriform ulceration or keratin plug that may project like a horn

Mycobacterium marinum

• cause of ‘Fish Tank Granuloma’.
• Lesions are ovoid
• usually occur on hands (following contact with fish).
• Fishermen, fishmongers tropical fish enthusiasts are susceptible

Pyogenic granuloma (lobular capillary haemangioma)

• benign vascular lesion of skin and mucosa.
• cause unknown
• Pathologically, it an inflammatory lesion composed of granulation tissue and chronic inflammatory cells
• usually solitary
• glistening red papule or nodule
• prone to bleeding and ulceration.
• often grow rapidly (over weeks)
• frequently at sites of trauma
• commonly involve digits, arms, head and face

Kaposi’s sarcoma

• cancer of blood vessels

Cause

• Infection with human herpes virus 8 in patients with HIV

Features

• red to purplish spots (macules) and raised bumps (papules and nodules)
• generally first seen on the skin, commonly on legs or feet. Initially, the lesions are small and painless but they can ulcerate and become painful.
• occur in the mouth.
• occur internally; in the gut, lungs, genitals and lymphatic system. These internal lesions may cause symptoms e.g. discomfort with swallowing, bleeding, shortness of breath, swollen legs, etc.

Treating localised lesions

• Freezing with liquid nitrogen (cryotherapy)
• radiation
• Surgical removal
• Injection with anti-cancer drugs such as vinblastine
• Topical application of alitretinoin gel (Panretin)

Treating extensive or internal lesions

• Photodynamic therapy (a combination of a photosensitiser and light energy)
• Isotretinoin (a vitamin-A derivative)
• Cytokine inhibitors
• The pregnancy hormone, human chorionic gonadotropin (HCG); Kaposi sarcoma lesions disappear in some women when they become pregnant.
• Ganciclovir and foscarnet (antiviral medications)

Mycosis fungoides

• Cutaneous T-cell lymphoma which is usually confined to skin
• Itchy, red plaques – pre-malignant stage
• Telangiectasiae
• Areas of 'cigarette paper' atrophy (poikiloderma atrophicans vasculare).
• Nodular lesions, become necrotic – malignancy
• Sézary syndrome is a variant which is also associated with erythroderma

Malignant melanoma

• malignant tumour of the melanocytes
• ♀ : ♂ ≈ 1.5 : 1
• UK incidence: 3500/yr, with 800 deaths/ yr (up ≥80% in last 20yrs)
• metastasise early
• Sunlight is a major cause, particularly in the early years.
• Two-thirds arise from normal skin and one-third arise from a pre-existing mole

Diagnosis

if there are ≥3 points on the Glasgow scale (2 for major feature, 1 for minor feature),

Major

• Change in size
• Change in shape
• Change in colour

Minor

• Inflammation, crusting, or bleeding
• Sensory change
• Diameter >7mm (unless growth is in the vertical plane: beware)

Less helpful signs

• Asymmetry
• Irregular colour
• Elevation
• Irregular border

If smooth, well-demarcated and regular, it is unlikely to be a melanoma.

NB: Superficial spreading malignant melanomas occur as malignant melanocytes migrate laterally along the dermo-epidermal junction, and have a good prognosis

DD

• Pigmented basal cell carcinoma: heavily pigmented nodules with rolling edges.

Prognostic factors
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The invasion depth of a tumour (Breslow's depth) is the single most important factor in determining prognosis of patients with malignant melanoma

Breslow Thickness Approximate 5 year survival
< 1 mm 95-100%
1 - 2 mm 80-96%
2.1 - 4 mm 60-75%
> 4 mm 50%

Treatment

• urgent excision

Basal cell carcinoma (rodent ulcer)

Cause

• UV exposure

Features

• most commonly occur in middle-aged or elderly fair-skinned individuals
• usually on the face on a sun-exposed site, but sometimes on limbs and trunk
• Typically, a pearly firm nodule with rolled telangiectatic edge
• It slowly increases in size when the centre may ulcerate and crust─causes local destruction if left untreated.
• Lesions on the trunk can appear as red scaly plaques with a raised smooth edge
• Metastases are very rare

Treatment

• complete excision with a margin of normal skin

Dermatitis artefacta

• skin lesions are inflicted by the patient on themselves.

Causes

• underlying psychological problem
• form of emotional release from distressful situations
• attention-seeking behaviour particularly when the patient is lonely

Features

• more common in women than in men.
• lesions tend to have unusual shapes and may have a linear or geometric pattern.
• clearly demarcated from surrounding skin
• usually appear overnight
• Different methods may be used to injure the skin (nails, caustic soda, cigarettes).
• tend to be on exposed skin that is readily accessible to the patient’s hands

Hereditary haemorrhagic telangiectasia

• also known as Osler–Weber–Rendu disease
• autosomal dominantly inherited condition
• HHT1 (Chromosome 9) and HHT 2 (Chromosome 12)

Features

• Telangiectases of the skin and mucous membranes (Small red macules and papules)
• on the lips, tongue and fingers
• Bleeding tendency eg nose bleeds and GI bleeding from early teens and worsening after age of 50
• Larger lesions may affect the nasopharynx, central nervous system, lung, liver, and spleen, as well as the urinary and gastrointestinal tracts
• AV malformations of brain, lung, (more frequently in HHT1) GI tract.

Treatment

• oral iron: for Anaemia
• pulse dye laser: very effective at removing skin lesions if unsightly
• Nasal skin grafts: for persistent epistaxis

Tuberous sclerosis

• genetic condition of autosomal dominant inheritance(one third)
• sporadic new mutations of the tuberin protein gene occurring in the early stages of life(other cases)
• As is neurofibromatosis, the majority of features seen are neuro-cutaneous
• usually occurs between 2-6 years

Cutaneous features (60-70%)

• depigmented 'ash-leaf' spots which fluoresce under UV light, 3 or more white spots at birth suggests diagnosis of TS
• Shagreen patches: roughened, Flesh coloured orange-peel connective tissue naevi of varying sizes, usually on the lower back
• adenoma sebaceum(Angiofibromas): Facial rash that appears as a spread of small pink or red spots across the cheeks and nose in a butterfly distribution
• subungual fibromata: Smooth, firm, flesh-coloured lumps that emerge from the nail folds
• café-au-lait spots* may be seen
he

Severe angiofibromas
Moderate angiofibromas
Mild angiofibromas

Periungual fibromas
Periungual fibromas

Solitary ashleaf mark
Solitary ashleaf mark

Neurological features

• developmental delay
• epilepsy (infantile spasms or partial)e 70%
• intellectual impairment, learning difficulties

Also

• retinal hamartomas: dense white areas on retina (phakomata)
• rhabdomyomas of the heart
• Fibromas may also develop within CNS, where they calcify typically in periventricular area
• gliomatous changes can occur in the brain lesions
• polycystic kidneys, renal angiomyolipomata

*these of course are more commonly associated with neurofibromatosis. However a 1998 study of 106 children with TS found café-au-lait spots in 28% of patients

Neurofibromatosis type 1 (von Recklinghausen’s disease)

Criteria for diagnosis

• six or more café-au-lait spots
• two or more neurofibromas
• axillary freckling
• two or more Lisch nodules (iris hamartoma)
• optic glioma
• a parent or sibling with neurofibromatosis

associated abnormalities

• skeletal rib notching and other bony defects
• honeycomb lung
• intellectual disability
• kyphoscoliosis
• hypertension (renal artery stenosis, phaeochromocytoma)
• Lisch nodules (iris hamartoma).

Café-au-lait mark
Café-au-lait mark
Neurofibroma

Neurofibromas
Freckling in the armpit
Plexiform neurofibroma

Café-au-lait spots
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Hyperpigmented lesions that vary in colour from light brown to dark brown, with borders that may be smooth or irregular

Causes

• neurofibromatosis type I & II
• tuberous sclerosis
• Fanconi anaemia
• McCune-Albright syndrome

_________________
DR ALRAZI

DR ALRAZI · Posted: Sat Feb 28, 2009 2:56 am Post subject:

DERMATOLOGY 4
Erythroderma
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Causes of erythroderma

• eczema 40 %
• psoriasis 20%
• drugs e.g. gold
• lymphoma, leukaemia
• pityriasis rubra pilaris *
• idiopathic

* papulosquamous disorder of unknown aetiology which presents as red-orange
plaques

Features

• any inflammatory skin disease that affects more than 90% of the body surface
• fever, shivering and malaise
• metabolic disturbances
• hypothermia
• high-output cardiac failure
• may continue for months or years
• may relapse.

Treatment

• in hospital with careful monitoring of fluid balance and temperature

Erythrodermic psoriasis

• may result from progression of chronic disease to an exfoliative phase with plaques covering most of the body. Associated with mild systemic upset
• more serious form is an acute deterioration. This may be triggered by a variety of factors such as withdrawal of systemic steroids. Patients need to be admitted to hospital for management

Bullous disorders
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Causes of skin bullae

• congenital: epidermolysis bullosa
• autoimmune: bullous pemphigoid, pemphigus
• insect bite
• trauma/friction
• drugs: barbiturates, frusemide

Facial flushing

Causes

• Menopause
o Women
o Sweating
o waking them at night
• Anxiety
• Hyperthyroidism
• Calcium-channel blockers eg diltiazem: continuous and persistent
• Rosacea
• Mitral valve disease
• Dermatomyositis
• SLE

Urticaria

• not an allergic process
• Mast cell degranulation → release of histamine,kinins, leukotrienes, prostaglandins, and complement
• → oedema, vasodilatation, and a cellular infiltrate of lymphocytes, polymorphs, and histiocytes
• no place for blood tests

Causes

• Idiopathic (most common): Autoimmune due to production of antibodies that cross-link the IgE receptor on mast cells (chronic idiopathic urticaria)
• Angio-oedema
o oedema of the subcutaneous tissues
o may involve the mucous membranes.
o Hereditary angio-oedema is a rare form
o Laryngeal oedema is the most serious complication
• Physical urticarias
o Dermatographism—exaggerated release of histamine from stroking the skin firmly with a hard object
o Pressure urticaria—by sustained pressure from clothing, hard seats, and footwear
o Cold urticaria
o Heat urticaria
o Solar urticaria
o Cholinergic urticaria—after exertion, stress, or exposure to heat.
o Aquagenic urticaria—on contact with water
• Non-physical urticaria
o Food allergies—fish, eggs, dairy products, chocolate, nuts, strawberries, pork, tomatoes
o Food additives—tartrazine dyes, sodium benzoates
o Salicylates—both in medicines and foods
o Infection—bacterial, viral, and protozoal
o Systemic disorders—autoimmune and “collagen” diseases; reticuloses, carcinoma, and dysproteinaemias
o Contact urticaria—from contact with meat, fish, vegetables, plants, and animals
o Papular urticaria—persistent itching papules at the site of insect bites; it is also sometimes applied to urticaria from other causes
o Inhalants—house dust, animal danders

Urticarial vasculitis

• Hepatitis B
• Systemic lupus erythematosus
• Idiopathic

Features

• Itching red weals
• < 24 hours (> 24 hours—urticarial vasculitis)
• acute if < 6 weeks, chronic if > 6 weeks

Treatment

• Eliminate possible causative factors
• Non-sedative histamine blockers eg loratadine, fexofenadine or cetirizine 10 mg bd For 2 weeks, the effects wear off after 14 hours so, regular dose, give some relief
• If fail, another non-sedative antihistamine and H2 blockers eg cimetidine
• Adrenaline for acute attacks, particularly if there is angio-oedema of the respiratory tract
• Systemic corticosteroids may be needed for acute urticarial vasculitis. Prednisolone works far too slowly

Fixed drug eruption

• A violent local erythema that blisters and recurs at the same site
• skin biopsy—diagnostic, eosinophilic infiltrate
• DD: other blistering skin conditions— more extensive, multiple blisters

_________________
DR ALRAZI

DR ALRAZI · Posted: Sat Feb 28, 2009 3:00 am Post subject:

DERMATOLOGY 5
Drug hypersensitivity syndrome

• serious hypersensitivity reaction
• 3–6 weeks after commencing certain drugs, particularly anticonvulsants and antimicrobials

Features

• fever
• facial oedema
• generalised papulopustular or exanthematous rash
• lymphadenopathy, or hepatitis
• nephritis, pneumonitis, myocarditis
• hypothyroidism
• eosinophilia and mononucleosis.

Treatment

• oral steroid treatment

Scleroderma

Sclerosis of skin and internal organs. Increased production of collagen and widespread vascular damage, with early obliteration of capillaries and later involvement of progressively larger arteries.

• Tissue fibrosis
• Raynaud’s phenomenon (small blood vessel vasculopathy)
• Autoimmunity

1. LIMITED CONNECTIVE TISSUE DISEASE

• Raynauds
• capillary changes
• finger bowing

2. LOCALISED SCLERODERMA (Morphoea)

localised thickening of the dermis due to excess collagen with loss of appendages (sweat glands and hair follicles)

• after skin trauma (eg cosmetic piercings) and radiation
• peak incidence 20–40 years
• more common in females
• firm oval plaques with a shiny smooth surface
• The edge can be purple or brown
• It feels thickened compared to the surrounding skin
• either pale or hyperpigmented
• absent hair and sweating
• risk of developing further lesions at sites of subsequent trauma
• Autoantibodies are usually negative

Extensive morphoea may mimic systemic sclerosis, but differs in the absence of

• systemic disease
• Raynaud’s phenomenon
• hand lesions.

Treatment

• may improve spontaneously
• local steroids: in inflammatory stage
• vitamin D analogues: topical and oral
• light therapy

3. CHEMICALLY INDUCED

• toxic oil syndrome which affected 20,000 people in Madrid

4. SYSTEMIC SCLEROSIS

Features

• microstomia
• facial telangiectasia
• beaking of nose
• ANA positive in 95%
• Rheumatoid factor positive in 25%
• Dyspepsia
• pulmonary hypertension: is generally secondary to reduced cross section of pulmonary vasculature, this being due to abnormal proliferation of pulmonary vascular cells. Pulmonary hypertension therefore most often occurs in the absence of interstitial fibrosis

a. Limited cutaneous systemic sclerosis

• lower risk of early visceral involvement.
• CREST: Calcinosis, Raynaud’s phenomenon, oEsophageal dysmotility, Sclerodactyly and Telangiectasia
• Anticentromere antibody (positive in up to 90 %, 93% 10yr survival)

b. Diffuse cutaneous systemic sclerosis

• both cutaneous and visceral involvement,
• rapid skin thickening on the extremities, face and trunk
• Antitopoisomerase (Scl-70) antibody (positive in up to 60%, 66% 10yr survival)

Anti- RNA polymerase (30% 10yr survival)

DD

• Systemic lupus erythematosus (SLE)
• Eosinophilic fasciitis (elevated eosinophil count)
• Polymyositis (elevated creatine kinase levels)

Necrolytic migratory erythema

• With glucagonoma

Features

• widespread exfoliative erythema
• affecting trunk and limbs, worse in the flexures
• moving crusted edge

Features of glucagonoma
• normochromic normocytic anemia
• Stomatitis
• Abdominal pains, watery diarrhoea
• weight loss
• diabetes
• hypoaminoacidemia
• cheilosis
• venous thrombosis
• neuropsychiatric features.
• At least 50% are metastatic at presentation so prognosis poor

DD

• protein and zinc deficiency (acrodermatitis enteropathica)
o with Alcoholics at risk of zinc deficiency because of poor diet and also because of the direct effect of alcohol increasing the urinary excretion of zinc
o zinc level ↓(normal range 11–1 Cool

o albumin level ↓ (normal 35–51 g/l)
o glucagon level normal
• Hepatic cirrhosis
• Coeliac disease
• Cystic fibrosis causing intestinal malabsorption

_________________
DR ALRAZI

DR ALRAZI · Posted: Sat Feb 28, 2009 3:02 am Post subject:

DERMATOLOGY 6
Dermatomyositis

• autoimmune skin condition

Features

• insidious, symmetrical, proximal muscle weakness resulting from muscle inflammation (eg difficulty brushing hair, standing up from a chair)
• Autoantibodies to striatal muscle eg Anti-Jo antibodies (most common)
• pulmonary fibrosis
• The skin signs are characteristic:
o heliotrope rash: violaceous or erythematous rash (sometimes oedema) in a symmetrical distribution involving periorbital skin (may be mild discoloration along eyelid margin)
o Gottren’s papules: lilac atrophic papules over knuckles
o malar erythema and facial oedema
o poikiloderma (ie, variegated telangiectasia, hyperpigmentation) in a photosensitive distribution
o violaceous erythema on extensor surfaces
o erythema, telangiectasia and hypertrophy of cuticle with small hemorrhagic infarcts of nail folds
• The skin signs can appear much earlier than the muscle symptoms.
• Malignancy in up to 30% of patients above 40 years. The neoplasias most commonly seen are breast, lung, ovary and stomach.

Investigations

• elevated CK
• abnormal EMG (showing spontaneous fibrillation)

Treatment

• high-dose steroids and immunosuppressants. Cause remission of symptoms, even an underlying malignancy
• Management of the driving malignancy often leads to resolution of the cutaneous symptoms

Behçet’s disease

• chronic systemic vasculitic disorder involving arteries and veins
• in the Mediterranean, Middle East and Japan
• HLA B5 associated with ocular disease
• HLA B12 associated with recurrent oral ulcers

Classical triad

• recurrent oral ulceration
• recurrent genital ulceration
• iritis

Features

• commonly relapsing and remitting course
• painful oral aphthous ulcers 90%
• genital aphthous ulcers 70%
• ocular inflammation 50%:
o anterior or posterior uveitis.
o In most cases, follow the oral and genital ulcers by 3–4 years
o initial manifestation in about 20%
• gastrointestinal symptoms 50%: intestinal erosions and ulcers may cause abdominal pain, melena, and perforation
• arthralgias/arthritis 50%: asymmetrical migratory non-erosive oligoarthritis
• CNS involvement eg meningoencephalitis
• skin lesions
o erythema nodosum
o folliculitis
o acneiform lesions on the face
o thrombophlebitis
o cutaneous hypersensitivity eg development of a pustule after skin puncture
• Vascular complications 7-40 %
o venous and arterial thromboses
o vessel occlusions and stenoses
o aneurysm formation
o Venous involvement typically includes superficial thrombophlebitis or deep venous thrombosis, usually of the lower extremities
DD
• Crohn’s disease
Treatment
• steroids or colchicine

Retention hyperkeratosis
• common condition found on elderly care wards
• a build up of keratin resulted from bandages that were changed infrequently as treatment for varicose eczema in the past ─This interferes with the normal process of desquamation

Treatment

• Soaking in arachis oil
• Gentle debridement: revealing normal skin underneath.
• Emollients to prevent recurrence

Acne rosacea

• chronic skin disease of unknown aetiology
• typically affects nose, cheeks and forehead
• more common in females

Features

• flushing: is often first symptom
• later develops into
o persistent erythema
o telangiectasia
o papules
o pustules

Complication
• rhinophyma: hypertrophy of the nose with follicular dilatation, resulting from
o hyperplasia of the sebaceous gland
o fibrosis
o increased vascularity
o lymphoedema
• blepharitis
• chronic lymphoedema of the face

DD

• Ciclosporin treatment: Sebaceous hyperplasia is a recognised side-effect, particularly on the skin of renal transplant patients when exposed to sunlight
• Steroid rosacea: a result of topical steroids to the face
• Acne: papules, pustules and comedones

Management

• topical metronidazole: for mild symptoms
• oral antibiotics e.g. oxytetracycline: in active and more severe disease
• high-factor sunscreen: daily
• camouflage creams: help conceal redness
• laser therapy: for prominent telangiectasia

Acne vulgaris
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Acne vulgaris is a common skin disorder which usually occurs in adolescence.

Features

• comedones, inflammation and pustules
• It typically affects the face, neck and upper trunk

Epidemiology

• affects around 80-90% of teenagers, 60% of whom seek medical advice
• acne may also persist beyond adolescence, with 10-15% of females and 5% of males over 25 years old being affected
• Acne presenting at a later stage (beyond aged 20 years) should always prompt investigating a possible secondary cause

Pathophysiology is multifactorial

• follicular epidermal hyperproliferation resulting in the formation of a keratin plug → obstruction of the pilosebaceous follicle
• Activity of sebaceous glands may be controlled by androgen, although levels are often normal in patients with acne
• colonisation by the anaerobic bacterium Propionibacterium acnes
• inflammation

Acne may be classified into mild, moderate or severe:

• mild: open and closed comedones with or without sparse inflammatory lesions.
• moderate acne: widespread non-inflammatory lesions and numerous papules and pustules
• severe acne: extensive inflammatory lesions, which may include nodules, pitting, and scarring

Management

Acne vulgaris is a common skin disorder which usually occurs in adolescence. It typically affects the face, neck and upper trunk and is characterised by the obstruction of the pilosebaceous follicle with keratin plugs which results in comedones, inflammation and pustules.

A simple step-up management scheme often used in the treatment of acne is as follows:

• single topical therapy (topical retinoids, benzyl peroxide)
• topical combination therapy (topical antibiotic, benzoyl peroxide, topical retinoid)
• oral antibiotics: e.g. oxytetracycline, doxycycline. Improvement may not be seen for 3-4 months. Minocycline is now considered second line treatment due to the possibility of irreversible pigmentation. Gram negative folliculitis may occur as a complication of long-term antibiotic use - high-dose oral trimethoprim is effective if this occurs
• oral isotretinoin: only under specialist supervision

There is no role for dietary modification in patients with acne

Isotretinoin

• Oral retinoid
• Two-thirds of patients have a long term remission or cure
• It significantly reduces
o elevated sebum production
o comedogenesis
o colonisation with Propionibacterium acnes.
• should be prescribed for patients with moderate or severe acne who are failing to respond to conventional therapy

Adverse effects

• teratogenicity: negative pregnancy test must be obtained prior to treatment (Beta-HCG), females MUST be using two forms of contraception (e.g. combined oral contraceptive pill and condoms)
• Liver function tests ↑
• raised triglycerides
• dry skin, eyes and lips: the most common side-effect of isotretinoin
• low mood
• hair thinning
• nose bleeds (caused by dryness of the nasal mucosa)
• benign intracranial hypertension: isotretinoin treatment should not be combined with tetracyclines for this reason

Psoriasis

Basics

• can be divided into type 1 and 2

Type 1

• presents < 40 years old
• positive family history
• associated with HLA-CW6

Type 2

• presents > 50 years old
• no family history

Features

• purplish plaque with silvery scales (diagnostic)
• scalp, extensor surfaces elbows/knees, sacrum
• nail signs: pitting, onycholysis
• arthritis
• Psoriasis in the genitalia
o difficult to distinguish from tinea and eczema
o skin changes elsewhere
o not scaly but glistening and well demarcated
o treatment is moderately potent topical steroids in the first instance
• Guttate psoriasis
o may often follow upper respiratory tract infection especially Streptococcal
o drop like ' Guttate"
o common in children and young adults

Drug-induced psoriasis

• Reactions may occur form less than one month to one year after medication initiated

Cause

• Beta blockers
• Lithium
• Antimalarials
• NSAIDs
• Lisinopril: rare

Treatment

• withdrawal of all medications, unless absolutely necessary
• Skin punch biopsy may be performed to exclude other forms of erythroderma or pustulosis
• Bed rest
• bland topical compresses
• low potency topical steroids
• Frequent emollient
• Etretinate
• methotrexate,
• phototherapy

Nail changes

• affect both fingers and toes
• do not reflect the severity of psoriasis
• association with psoriatic arthropathy

Cause

• abnormal T cell activity stimulates keratinocyte proliferation (rather than an actual primary keratinocyte disorder)
• mediated by type 1 helper T cells

Nail changes seen in psoriasis

• pitting
• onycholysis
• subungual hyperkeratosis
• loss of nail

Management
sqweqwesf erwrewfsdfs adasd dhe
Topical

• simple emollients
• coal tar: probably inhibit DNA synthesis
• topical corticosteroids*: particularly if flexural disease
• calcipotriol: vitamin D analogue which reduces epidermal proliferation and restores a normal horny layer. ointment is used for bd topical treatment
• dithranol: inhibits DNA synthesis, wash off after 30 mins, SE: burning, staining

Phototherapy

• narrow band ultraviolet B light (311-313nm) is now the treatment of choice
• photochemotherapy is also used - psoralen + ultraviolet A light (PUVA)
• adverse effects: skin ageing, squamous cell cancer (not melanoma)

Systemic therapy

• methotrexate: useful if associated joint disease. LFTs and FBC must be monitored regularly – SE: hepatitis and liver cirrhosis, lung fibrosis
• infliximab (TNF-alpha antibody): highly effective, particularly if joint disease
• ciclosporin: causes nephrotoxicity and U+Es and creatinine must be checked regularly as well as blood pressure (may precipitate hypertension)
• systemic retinoids

* Oral Steroids are contraindicated in psoriasis and although one may see an initial improvement, a very serious rebound effect may be seen.

EBV

EBV infection associated a rash if ampicillin administered

_________________
DR ALRAZI

DR ALRAZI · Posted: Sat Feb 28, 2009 1:24 pm Post subject:

--------------------------------------------------------------------------------

Hi Dr

waiting for ur responses and suggestions.

Best regards.

Sad

_________________
DR ALRAZI

mrsidhwa · Posted: Sun Mar 01, 2009 10:10 pm Post subject:

hey guys how are you guys starting studies with sanjay sharma who is giving part 2 written in july let me know any guidance guys any one thinking about reading text books How about masterclass any suggestions

DR ALRAZI · Posted: Mon Mar 02, 2009 2:14 am Post subject: Dear Dr

What I can say is that sharma is excellent as it directs you to the most important points that are usually covered in the exam.
Onexamination is good also as it represents the type of questions that you will see in the real exam. Its only problem is that it contains about 1200 questions only, so the chance for the questions to be repeated is low (given that in each exam diet there is about 270 questions). If you remember the number of questions in part 1 was about 3600 questions so the chance of the questions being repeated is higher.
I believe if you want to do something else other than the onexamination, it could be a good idea to try the pastest (I heard that it is at least comparable to onexamination).
PACES
MRCP
Tim Hall
Foreward by
Sir Graeme Catto
2009 or 2008

An Aid to the
MRCP short cases
REJ Ryder/ MA Mir /EA freeman
2009 or 2008

MRCP 2
Success in paces
Philip Kelly thomos powles paul jenkius
Pastest

2009 or 2008

Rapid Review of Clinical Medicine for MRCP: Part 2

by Sanjay Sharma

Product details
Paperback: 352 pages
Publisher: Manson Publishing Ltd; 2nd Edition edition (13 Jun 2006)
Language English
ISBN-10: 1840760702
ISBN-13: 978-1840760705

And best of luck to everybody.

Salam

_________________
DR ALRAZI

hamnee · Guest

great work alrazi
m going 2 appear in july
combined study on line is not a dream
any 1 can do
waiting for an enthusiastic partner 4 this lazy 1

guest 83 · Guest

hi everyone..i have started reading sanjay sharma..the data interpretation ques are slightly difficult expecially in answering the exact explanation.
any senior plz guide wat is the best way to answer the way it is written in the book ..and also how we should time ourself for each ques? how much time on one ques ?each paper has 100 ques to do in three hours?
as the ques are longer than in part 1 and has many parts ...

thanks

mrsidhwa · Posted: Thu Mar 05, 2009 6:59 pm Post subject:

I WOULD SUGGEST YOU TO DO MOCK TESTS ON ONEXAMINATION PASTEST AND MASTERCLASS AND TIME TOUR SELF TRY TO DO 100 QS IN 2 HRS AND 45 MIN AND KEEP 15 MIN FOR RECHECKING

DR ALRAZI · Posted: Sun Mar 08, 2009 10:20 am Post subject: NEPHROLOGY

Renal physiology

Renal blood flow is 20-25% of cardiac output

Renal cortical blood flow > medullary blood flow (i.e. tubular cells more prone to ischaemia

Sterile pyuria

Causes
• partially treated UTI
• renal TB
• urethritis and sexually transmitted diseases
• appendicitis
• bladder/renal cell cancer
• calculi
• acute glomerulo-nephritis
• tubulo-interstitial diseases
• adult polycystic kidney disease

Proteinuria

Microalbuminuria
• defined as an albumin excretion of 30 - 300 mg/day

Albumin:creatinine excretion ratio (ACR)
• used in clinical practice to quantify degree of proteinuria
• first morning urine sample
• urine albumin (mg) / creatinine (mmol)
• normal ACR < 2.5
• microalbuminuric range = 2.5 - 33

Renal stones:

Risk factors
• dehydration
• hypercalciuria, hyperparathyroidism, hypercalcaemia
• cystinuria
• high dietary oxalate
• renal tubular acidosis
• medullary sponge kidney, polycystic kidney disease
• beryllium or cadmium exposure

Risk factors for urate stones
• associated with hyperuricaemia and hyperuricosuria
• gout
• ileostomy: loss of bicarbonate and fluid results in acidic urine, causing the precipitation of uric acid
• dehydration
• thiazide diuretics
• high purine load (high protein diet)
• high cell turnover (e.g. haematological malignancy)
• Primary polycythaemia

Drug causes
• drugs that promote calcium stones: loop diuretics, steroids, acetazolamide, theophylline
• thiazides can prevent calcium stones (increase distal tubular calcium resorption)
Imaging

The table below summarises the appearance of different types of renal stone on x-ray
he
Type Frequency Radiograph appearance
Calcium oxalate 40% Opaque
Mixed calcium oxalate/phosphate stones 25% Opaque
Triple phosphate stones 10% Opaque
Calcium phosphate 10% Opaque
Urate stones 5-10% Radio-lucent
Cystine stones 1% Semi-opaque, 'ground-glass' appearance
Xanthine stones <1% Radio-lucent

management
Calcium stones
• high fluid intake
• low animal protein, low salt diet (a low calcium diet has not been shown to be superior to a normocalcaemic diet)
• thiazide diuretics (increase distal tubular calcium resorption)
• stones < 5 mm will usually pass spontaneously
• lithotripsy, nephrolithotomy may be required

Oxalate stones
• cholestyramine reduces urinary oxalate secretion
• pyridoxine reduces urinary oxalate secretion

Uric acid stones
• allopurinol
• urinary alkalinization e.g. oral bicarbonate

Glomerulonephritides

Knowing a few key facts is the best way to approach the difficult subject of glomerulonephritis:

Membranous glomerulonephritis

• commonest type of glomerulonephritis in adults
• third most common cause of end-stage renal failure (ESRF)
• The immune complexes develop in situ or, less likely, by the deposition of circulating immune complexes
• most patients are older than 30 years at diagnosis

Features:
• It usually presents as nephrotic syndrome or proteinuria, haematuria
• BP↑
• renal impairment.
• 5-10% have renal vein thrombosis

Renal biopsy demonstrates:
• thickened BM
• IF +ve for IgG & C3
• subepithelial deposits on EM.

Causes
• idiopathic
• infections: hepatitis B, malaria , syphilis; leprosy; filiariasis
• malignancy: lung cancer, lymphoma, leukaemia
• drugs: gold, penicillamine, NSAIDs, captopril
• autoimmune: RA; SLE; thyroid disease

Prognosis - rule of thirds
• one-third: spontaneous remission
• one-third: remain proteinuric
• one-third: develop ESRF

Management
• immunosuppression: chlorambucil or cyclophosphamide, either alone or with steroids (methylprednisolone), are more effective than symptomatic treatment or treatment with steroids alone in inducing remission of the nephrotic syndrome
• BP control
• consider anticoagulation

IgA nephropathy (Berger's disease, mesangioproliferative GN)
Basics
• commonest cause of glomerulonephritis worldwide
• pathogenesis unknown, ?mesangial deposition of IgA immune complexes

Differentiating between IgA nephropathy and post-streptococcal glomerulonephritis
In post-streptococcal glomerulonephritis
• low complement levels
• main symptom is proteinuria (although haematuria can occur)
• typically an interval between URTI and the onset of renal problems

Presentations
• young male
• recurrent episodes of macroscopic haematuria
• typically associated with mucosal infections e.g., URTI
• Recovery is usually rapid between attacks
rarer
• nephrotic syndrome
• renal failure

Associated conditions
• alcoholic cirrhosis
• coeliac disease/dermatitis herpetiformis

Diagnosis:
Renal biopsy: mesangial hypercellularity, +ve immunofluorescence for IgA & C3

Management

• symptomatic treatment
• steroids/immunosuppressants not be shown to be useful

Prognosis
• 25% of patients develop ESRF over ~20yrs.

Henoch-Schonlein purpura

• IgA mediated small vessel vasculitis
• There is a degree of overlap with IgA nephropathy (Berger's disease).
• HSP is usually seen in children following an infection

Features
• palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs
• Flitting polyarthritis of the large joints
• features of IgA nephropathy may occur e.g. haematuria, renal failure
• abdominal pain

Diagnosis:
• Usually clinical
• May be confirmed by finding positive IF for IgA and C3 in skin lesions or renal biopsy (identical to IgA nephropathy)

Prognosis:
• 50% remission
• 15-20% impaired renal function
• 3-5% renal failure.

Diffuse proliferative glomerulonephritis
• classical post-streptococcal glomerulonephritis in child
• presents as nephritic syndrome / ARF

Minimal change glomerulonephritis

Commonest cause of nephrotic syndrome in children, accounting for 75% of cases in children and 25% in adults

The majority of cases are idiopathic, but in around 10-20% a cause is found:
• drugs: NSAIDs, rifampicin
• Hodgkin's lymphoma, thymoma
• infectious mononucleosis

Features
• nephrotic syndrome
• normotension - hypertension is rare
• highly selective proteinuria
• renal impairment
• especial vulnerability to renal effects of NSAIDs.
• renal biopsy: electron microscopy shows fusion of podocytes

Management
• majority of cases (80%) are steroid responsive
• cyclophosphamide is the next step for steroid resistant cases

prognosis

• 1% progress to ESRF.

Focal segmental glomerulosclerosis

• high recurrence rate in renal transplants

Causes
• idiopathic
• reflux or IgA nephropathy
• diffuse proliferative GN
• vasculitis
• HIV
• heroin
• Alport's syndrome
• sickle-cell

Presentations
• Nephrotic syndrome; proteinuria; haematuria
• ↓ renal function
• BP↑.

Renal biopsy
• Segmental areas of glomerular sclerosis
• hyalinization of glomerular capillaries
• positive IF for IgM and C3.

Treatment:
• symptomatic treatment
• Poor response to corticosteroids (10-30%)
• Cyclophosphamide or ciclosporin (=cylosporin) may be used in steroid-resistant cases.

Prognosis:
• 30-50% progress to ESRF.

Rapidly progressive glomerulonephritis (crescentic glomerulonephritis)

ESRF develops over weeks or months.

Causes:
• Primary systemic vasculitis (eg Wegener's granulomatosis, polyarteritis, Churg-Strauss syndrome, polyarteritis nodosa, giant cell arteritis, Takayasu's arteritis).
• Antiglomerular basement membrane (GBM) disease (Goodpasture's).
• Systemic disorders: SLE, mixed cryoglobulinaemia; Henoch-Schonlein purpura; relapsing polychondritis, Behcet's disease, rheumatoid arthritis.
• Primary GN (IgA nephropathy, mesangiocapillary GN, membranous GN).
• Infection: (post-streptococcal, IE/SBE, visceral abscess, shunt nephritis).
• Malignancy (carcinoma, lymphoma).
• Drugs (penicillamine, hydralazine, rifampicin).

Clinical features:

• Symptoms and signs of renal failure
• There may be loin pain, haematuria,
• systemic symptoms (fever, malaise, myalgia, weight loss).

Renal biopsy:

• Focal necrotizing GN with crescent formation (crescentic GN).

Lung function tests:

• Gas transfer (KCO) for pulmonary haemorrhage.

Treatment:
• High-dose corticosteroids; cyclophosphamide
• ± plasma exchange
• renal transplantation

Prognosis:

• Poor if initial serum creatinine >600 µmol/L.

Goodpasture's syndrome

• rare condition
• caused by anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen. (and the alveolar membrane)
• more common in men (sex ratio 2:1)
• has a bimodal age distribution (peaks in 20-30 and 60-70 ).
• associated with HLA DR2

Features
• pulmonary haemorrhage
• followed by rapidly progressive glomerulonephritis

Factors which increase likelihood of pulmonary haemorrhage
• young males
• smoking
• lower respiratory tract infection
• pulmonary oedema
• inhalation of hydrocarbons

Investigations
• renal biopsy: linear IgG deposits along basement membrane (crescentic nephritis)
• CXR: infiltrates, often in the lower zones
• raised transfer factor secondary to pulmonary haemorrhages

Prognosis:

• Many die in the first 6 months

Management
• plasma exchange
• steroids
• cyclophosphamide

Mesangiocapillary glomerulonephritis (membranoproliferative)
• 8% of children and 14% of adults with nephrotic syndrome
• Biopsy shows large glomeruli with mesangial proliferation and 'double' BM
• type 1(subendothelial deposits): SLE; post-strep; endocarditis; visceral abscess; shunt nephritis; HBV; HCV; leprosy; schistosomiasis; filariasis; mixed cryoglobulinaemia; sickle-cell disease; carcinoma; α1-antitrypsin deficiency
• type 2(intramembranous deposits, dense deposit disease): partial lipodystrophy; candidiasis
• ↓ serum C3 and C3 nephritic factor (an antibody against C3bBb) are found in some patients (type II (found in 70%)more than type I).
• 50% develop ESRF

Glomerulonephritis and low complement
Disorders associated with glomerulonephritis and low serum complement levels
• post-streptococcal glomerulonephritis
• subacute bacterial endocarditis
• systemic lupus erythematous
• mesangiocapillary glomerulonephritis

Nephrotic syndrome

Triad of
1. Proteinuria (> 3g/24hr) causing
2. Hypoalbuminaemia (< 30g/L) and
3. Oedema

Loss of antithrombin-III, proteins C and S and a associated rise in fibrinogen levels predispose to thrombosis. Loss of TBG lowers total, but not free thyroxine levels
Causes
Glomerulonephritis (GN, c. 80%)
• minimal change GN (causes 80% in children, 30% in adults)
• membranous GN
• focal segmental glomerulosclerosis

Systemic disease (c. 20%)
• DM
• amyloidosis (e.g. RA)
• SLE

Drugs
• gold / penicillamine (RA)
• captopril (heart failure)

Others
• congenital
• neoplasia: carcinoma, lymphoma, leukaemia, myeloma
• infection: bacterial endocarditis, hepatitis B, malaria
• renal vein thrombosis

Complications
• increased risk of infection due to urinary immunoglobulin loss
• increased risk of thromboembolism related to loss of antithrombin III and plasminogen in the urine
• hyperlipidaemia
• hypocalcaemia (vitamin D and binding protein lost in urine)
• acute renal failure

Renal vascular disease

Renal vascular disease
Causes
• atherosclerosis (> 95% of patients)
• fibromuscular dysplasia: more common in young women and characteristically has a 'string of beads' appearance on angiography.
risk factors: cause atheroma elsewhere in the body
• smoking
• hypertension
• hyperlipidaemia
Features
• Coexistent cardiovascular, cerebrovascular, or peripheral vascular disease
• deterioration in renal function after ACE-I
• hypertension
• 'flash' pulmonary oedema
• chronic renal failure
• Abdominal, carotid, or femoral bruits
• absent leg pulses
• grade III-IV hypertensive retinopathy
• asymmetrical kidneys
Investigation
• MR angiography is now the investigation of choice
• CT angiography
• conventional renal angiography is less commonly performed used nowadays, but may still have a role when planning surgery
• renal vein renin ratio
• captopril challenge test (peripheral renin levels after captopril)
• isotope renography
Treatment:
• Percutaneous transluminal renal angioplasty or surgery

Cholesterol emboli

• Suspect in any arteriopath with eosinophilia, purpura (eg of toes) ± ↑ urea
• Prevalence: 0.3%
• cholesterol emboli may break off causing renal disease

Risk:
• Atheroma
• ↑ cholesterol
• aneurysms
• thrombolysis
• arterial procedures

Features
• eosinophilia
• purpura
• progressive renal failure
• livedo reticularis
• GI bleeding
• Myalgia
• Cyanosis
• Often spontaneous
• cholesterol clefts seen in renal or colonic biopsies; they induce evolving fibrosis

Treatment:
• Statins are tried
• avoid anticoagulants and instrumentations

Prognosis:
• Often progressive and fatal
• A few have regained renal function after dialysis.

Renal tubular disease

Fanconi syndrome

A disturbance of renal tubular function resulting in:
• Generalized aminoaciduria
• Phosphaturia
• Glycosuria
• Rickets (children) or osteomalacia (adults)
• Renal tubular acidosis type 2 (proximal).
Inherited causes:

• Cystinosis
• Galactosaemia
• Glycogen storage disease type 1
• Fructose intolerance
• Lowe's syndrome
• Tyrosinaemia type 1
• Wilson's disease.

Acquired causes:

• Renal (acute tubular necrosis, hypokalaemic nephropathy, myeloma, Sjogren's syndrome, transplant rejection)
• Hyperparathyroidism
• Kwashiorkor
• drugs (out-of-date tetracycline, iphosphamide)
• heavy metals (lead, mercury, cadmium, uranium).

Idiopathic Fanconi syndrome:

• Autosomal dominant
• Presents in adulthood
• rickets, osteomalacia
• progressive renal failure
• Treatment: calcitriol; K+, NaHCO3, PO43- supplements.

Cystinosis

• Deposition of L-cystine crystals in the proximal renal tubules, retucloendocelial system, cornea
• Autosomal recessive inheritance
• The severe infantile form presents with
o failure to thrive
o polyuria, polydipsia
o rickets
o corneal crystals, retinopathy
o hypothyroidism
o renal failure.
• Treatment:
o vigorous hydration
o calcitriol; K+, NaHCO3, and PO43- supplements
o thyroxine for hypothyroidism
o dialysis or transplantation for end stage renal failure (ESRF)
o Cysteamine reduces leucocyte cystine and slows glomerular deterioration.

Renal tubular acidosis (RTA)

All types are associated with hyperchloraemic (normal anion gap) acidosis with no evidence of gastrointestinal disturbance

Type 4 RTAhyporeninaemic hypoaldosteronism
most common of these disorders

Pathogenesis: ↓ Na reabsorption in the distal tubule → ↓ secretion of both K and acid

Causes: with hypoaldosteronism or failure of aldosterone action, eg
• Addison's disease
• inborn errors of steroid metabolism
• DM (Gordon's syndrome shares biochemical abnormalities but differs in having normal GFR and hypertension)
• chronic tubulointerstitial disease
• Renal transplant rejection
• drugs (ACE-i, β-blockers, K+ sparing diuretics, NSAIDs).

Features:

• Hyperkalaemia
• hyperchloraemic metabolic acidosis
• Urinary pH <5.4
• Plasma renin and aldosterone are found to be low, with Subnormal response to stimulation
• Low basal 24-hour urinary aldosterone

Treatment
• Fludrocortisone 0.05-0.15mg PO daily, if acidotic or hyperkalaemic
• sodium bicarbonate
• diuretics
• ion exchange resins to remove potassium

Type 1 ('distal') RTA

Rare

Pathogenesis: failure of H+ excretion in the distal tubule

Cauaes
• Congenital
• Hyperglobulinaemia Autoimmune connective tissue diseases, e.g. SLE
• Toxins and drugs, e.g. toluene, lithium, amphotericin

Features
• hyperchloraemic metabolic acidosis → Hyperventilation
• hypokalaemia → muscle weakness
• inability to lower the urine pH below 5.3 despite systemic acidosis
• low urinary ammonium production. (urinary acid is excreted as ammonium chloride)
• low urinary citrate (owing to increased citrate absorption in the proximal tubule where it can be converted to bicarbonate)
• hypercalciuria: acidosis leads to mobilisation of calcium from bone and consequent osteomalacia (→ bone pain) with hypercalciuria, stone formation (→ UTI) and nephrocalcinosis

Treatment
• If Acute: correct hypokalaemia before acidosis
• sodium bicarbonate (1-3mmol/kg/d PO)
• potassium supplements and citrate
• Thiazide diuretics are useful by causing volume contraction and increased proximal sodium bicarbonate reabsorption.

Type 2 ('proximal') RTA
very rare in adult practice

Pathogenesis: failure of sodium bicarbonate reabsorption in the proximal tubule.

Cauaes
• Congenital, e.g. Fanconi's syndrome, cystinosis, Wilson's disease
• Paraproteinaemia, e.g. myeloma
• Amyloidosis
• Hyperparathyroidism
• Heavy metal toxicity
• Drugs, e.g. carbonic anhydrase inhibitors, ifosfamide

Features
• hyperchloraemic metabolic acidosis
• hypokalaemia
• inability to lower the urine pH below 5.3 despite systemic acidosis: In severe acidosis an acid urine can occur once the plasma bicarbonate has fallen below 16 mmol/l, since distal H+ secretion mechanisms are intact.
• appearance of bicarbonate in the urine despite a subnormal plasma bicarbonate.
• frequently associated with urinary wasting of amino acids, phosphate and glucose (Fanconi's syndrome) as well as bicarbonate and potassium.

Treatment

High doses of bicarbonate may be required to overcome the renal 'leak'. (≥ 3mmol/kg/d)

Diagnosis of renal tubular acidosis
• Plasma HCO3- < 21 mmol/L, urine pH > 5.3 = renal tubular acidosis
• To differentiate between proximal (very rare) and distal (rare) requires bicarbonate infusion test
• Plasma HCO3- > 21 mmol/L but suspicion of partial renal tubular acidosis (e.g. nephrocalcinosis-associated diseases): acid load test required as follows:
 Give 100 mg/kg ammonium chloride by mouth
 Check urine pH hourly and plasma HCO3- at 3 hours
 Plasma HCO3- should drop below 21 mmol/L unless the patient vomits (in which case the test should be repeated with an antiemetic)
 If urine pH remains > 5.3 despite a plasma HCO3- of 21 mmol/L, the diagnosis is confirmed

Type 3 RTA
• vanishingly rare
• a combination of type 1 and type 2.
• Inherited type 3 RTA is caused by mutations resulting in carbonic anhydrase type II deficiency, which is characterized by
 Osteopetrosis
 RTA of mixed type
 cerebral calcification
 mental retardation.
 Typical radiographic features of osteopetrosis are present
 histopathological study of the iliac crest reveals unreabsorbed calcified primary spongia.

Liddle's syndrome

Overview
• autosomal dominant condition
• causes hypertension and hypokalaemic alkalosis
• thought to be caused by a disorder sodium channels in the distal tubules leading to increased reabsorption of sodium he
• treatment is with amiloride

Bartter's syndrome

• usually autosomal recessive (mutations eg in genes encoding the Na-K-2Cl cotransporter (NKCC2))
• hyperplasia of the juxtaglomerular apparatus in most cases
• defective chloride absorption at the Na+ K+ 2Cl- cotransporter in the ascending loop of Henle → urine K+ loss
• secondary stimulation of prostaglandin synthesis, which activates the renin angiotensin aldosterone system which exacerbates the renal potassium wasting.
• Presents in childhood with failure to thrive, polyuria, and polydipsia. muscle weakness, constipation, cramps or carpopedal spasms
• BP is normal and there is no oedema
• Hypokalaemia (<2.5mmol/L)
• hypochloraemic metabolic alkalosis
• ↑ urinary K+ and Cl-
• ↑ Plasma rennin, hyperammonaemia with hyperaldosteronism
• DD: liquorice, laxative, or diuretic use, persistent vomiting or diarrhoea, pyelonephritis, or diabetes insipidus. (↓ urine Cl)
• Treatments: K+ replacement, NSAIDs, amiloride, captopril.

Gitelman’s syndrome

Overview
• cause: mutations in the distal tubular thiazide-sensitive Na+ Cl cotransporter gene, SLC12A3 on 16q13

Features
• hypokalaemia
• hypomagnesaemia
• hypocalciuria
• metabolic alkalosis
• normotension

Inherited kidney diseases

Adult polycystic kidney disease (APKD)
• Prevalence: 1 : 1000 (most common inherited kidney disease)
• Inheritance: Autosomal dominant
• Two disease loci have been identified, PKD1 and PKD2, which code for polycystin-1 and polycystin-2 respectively
ADPKD type 1 ADPKD type 2
85% of cases 15% of cases
Chromosome 16 Chromosome 4
Presents with ESRF earlier

Features
• Renal enlargement with cysts
• abdominal pain
• renal stones
• haematuria
• recurrent UTIs
• hypertension
• CRF
Extra-renal manifestations
• liver cysts (70%)
• berry aneurysms (8%) ; subarachnoid haemorrhage
• CVS: mitral valve prolapse, mitral/tricuspid incompetence, aortic root dilation, aortic dissection
• cysts in other organs: pancreas, spleen, thyroid
• abdominal herniae

Screening
The screening investigation for relatives is abdominal ultrasound:
he
Ultrasound diagnostic criteria (in patients with positive family history)
• two cysts, unilateral or bilateral, if aged < 30 years
• two cysts in both kidneys if aged 30-59 years
• four cysts in both kidneys if aged > 60 years
or magnetic resonance angiography for 1st-degree relatives of those with stroke
Treatment:
• Monitor U&E & BP treating ↑ BP is most important
• Treat infections
• dialysis or transplantation for ESRF
• genetic counselling.

Infantile polycystic kidney disease

• Prevalence 1 : 40,000
• much less common than autosomal dominant disease
• Autosomal recessive (chromosome 6)
• Diagnosis may be made on prenatal ultrasound or in early infancy with abdominal masses and renal failure.
• Signs: renal cysts; congenital hepatic fibrosis (portal and interlobular fibrosis)
• End-stage renal failure develops in childhood.

Alport's syndrome

• Prevalence: 1 : 5000.
• hereditary condition, usually X-linked dominant but may be autosomal recessive or dominant.
• due to a defect in the gene which codes for type IV collagen resulting in an abnormal glomerular-basement membrane (GBM
• more severe in males with females rarely developing renal failure
• usually presents in childhood.
• Pathology: thickened GBM with splitting of the lamina densa.
he

Features:
• microscopic haematuria
• progressive renal failure
• bilateral sensorineural deafness
• retinitis pigmentosa
• lenticonus: protrusion of the lens surface into the anterior chamber (seen on slit-lamp examination)

Treatment: Control BP; supportive management of renal failure; dialysis; transplantation.

NB: A favourite question in the MRCP is an Alport's patient with a failing renal transplant. This may be caused by the presence of anti-GBM antibodies leading to a Goodpasture's syndrome like picture

SLE: renal complications

WHO classification
• class I: normal kidney
• class II: mesangial glomerulonephritis
• class III: focal (and segmental) proliferative glomerulonephritis
• class IV: diffuse proliferative glomerulonephritis
• class V: diffuse membranous glomerulonephritis
• class VI: sclerosing glomerulonephritis

Class IV (diffuse proliferative glomerulonephritis) is the most common and severe form
Management
• treat hypertension
• corticosteroids if clinical evidence of disease
• immunosuppressants e.g. azathiopine/cyclophosphamide

HIV: renal involvement
Renal involvement in HIV patients may occur as a consequence of treatment or the virus itself. Protease inhibitors such as indinavir can precipitate intratubular crystal obstruction
he
HIV-associated nephropathy (HIVAN) accounts for up to 10% of end-stage renal failure cases in the United States. Antiretroviral therapy has been shown to alter the course of the disease. There are five key features of HIVAN:
• massive proteinuria
• normal or large kidneys
• focal segmental glomerulosclerosis with focal or global capillary collapse on renal biopsy
• elevated urea and creatinine
• normotension

Acute Renal Failure (ARF)

Causes

• Pre-renal:
o Hypovolaemia (gastroenteritis, burns, sepsis, haemorrhage, Nephrotic Syndrome).
o Circulatory failure.
• Renal:
o Vascular: HUS, vasculitis, embolus, renal vein thrombosis.
o Tubular: acute tubular necrosis, ischaemic, toxic, obstructive.
o Glomerular: glomerulonephritis.
o Interstitial: interstitial nephritis, pyelonephritis.
o Acute chronic renal failure.
• Post-renal:
obstruction, either congenital or acquired. Although Alport's Syndrome is associated with end stage renal failure, this usually progresses gradually so that it occurs in adult life.

ATN vs. prerenal uraemia

Prerenal uraemia - kidneys hold on to sodium to preserve volume

Pre-renal uraemia Acute tubular necrosis
Urine sodium < 20 mmol/L > 30 mmol/L
Fractional sodium excretion* < 1% > 1%
Fractional urea excretion** < 35% >35%
Urine:plasma osmolality > 1.5 < 1.1
Urine:plasma urea > 10:1 < 8:1
Specific gravity > 1020 < 1010
Urine 'bland' sediment brown granular casts
Response to fluid challenge Yes No

*fractional sodium excretion = (urine sodium/plasma sodium) / (urine creatinine/plasma creatinine) x 100

**fractional urea excretion = (urine urea /blood urea ) / (urine creatinine/plasma creatinine) x 100

Acute vs. chronic renal failure
Best way to differentiate is renal ultrasound - most patients with CRF have bilateral small kidneys

Exceptions
• autosomal dominant polycystic kidney disease
• diabetic nephropathy
• amyloidosis
• scleroderma
• rapidly progressive glomerulonephritis

Other features suggesting CRF rather than ARF
• hypocalcaemia (due to lack of vitamin D)
• LVH is probably more likely to be seen in chronic renal failure but is not reliable

Chronic Renal Failure
Major pathophysiological abnormalities of chronic renal failure:
• Accumulation of nitrogenous waste products.
• Acidosis: bicarbonate wasting, decreased ammonia secretion, decreased acid excretion.
• Sodium wasting: solute diuresis, tubular damage.
• Sodium retention: Nephrotic Syndrome, CCF, anuria, excess sodium intake.
• Urinary concentrating defect: nephron loss, solute diuresis.
• Hyperkalaemia: decreased GFR, acidosis, hyperaldosteronism.
• Renal osteodystrophy: decreased intestinal calcium absorption, impaired 12-dihydroxy Vitamin D production, secondary hyperparathyroidism.
• Growth retardation: protein calorie deficiency, renal osteodystrophy, acidosis, anaemia.
• Anaemia: decreased erythropoeitin production, low grade haemolysis, inadequate intake.
• Bleeding tendency: thrombocytopenia, decreased platelet function.
• Infection: defective granulocyte function.
• Neurology: uraemia, aluminium toxicity results in fatigue, poor concentration, headache, memory loss, slurred speech, muscle weakness and cramps, seizures and coma.
• GI ulceration: gastric acid hypersecretion.
• Hypertension: sodium and water overload, hyperammonaemia.
• Hypertriglyceridaemia: decreased plasma lipoprotein lipase activity.
• Pericarditis and cardiomyopathy: cause unknown.
• Glucose intolerance: tissue insulin resistance.

Chronic kidney disease: anaemia

Patients with chronic kidney disease (CKD) may develop anaemia due to a variety of factors, the most significant of which is reduced erythropoietin levels. This is usually a normochromic normocytic anaemia and becomes apparent when the GFR is less than 35 ml/min (other causes of anaemia should be considered if the GFR is > 60 ml/min). Anaemia in CKD predisposes to the development of left ventricular hypertrophy - associated with a three fold increase in mortality in renal patients

Causes of anaemia in renal failure
• reduced erythropoietin levels - the most significant factor
• reduced erythropoiesis due to toxic effects of uraemia on bone marrow
• reduced absorption of iron
• anorexia/nausea due to uraemia
• reduced red cell survival (especially in haemodialysis)
• blood loss due to capillary fragility and poor platelet function
• stress ulceration leading to chronic blood loss

Management
• the 2006 NICE guidelines suggest a target haemoglobin of 10.5 - 12.5 g/dl
• determination and optimisation of iron status should be carried out prior to the administration of erythropoiesis-stimulating agents (ESA). Many patients, especially those on haemodialysis, will require IV iron
• ESAs such as erythropoietin and darbepoetin should be used in those 'who are likely to benefit in terms of quality of life and physical function'

Chronic kidney disease: bone disease

Basic problems in chronic kidney disease
• low vitamin D (1-alpha hydroxylation normally occurs in the kidneys)
• high phosphate
• low calcium: due to lack of vitamin D, high phosphate
• secondary hyperparathyroidism: due to low calcium, high phosphate and low vitamin D

Several clinical manifestations may result:
he
Osteitis fibrosa cystica
• aka hyperparathyroid bone disease

Adynamic
• may be due to over treatment with vitamin D

Osteomalacia
• due to low vitamin D

Osteosclerosis

Osteoporosis

Erythropoietin
Erythropoietin is a haematopoietic growth factor that stimulates the production of erythrocytes. The main uses of erythropoietin are to treat the anaemia associated with chronic renal failure and that associated with cytotoxic therapy
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Side-effects of erythropoietin
• accelerated hypertension --> encephalopathy, seizures (blood pressure increases in 25% of patients)
• bone aches
• skin rashes, urticaria, flu-like symptoms
• pure red cell aplasia (due to antibodies against erythropoietin)
• raised PCV increases risk of thrombosis (e.g. fistula)
• iron deficiency 2nd to increased erythropoiesis

There are a number of reasons why patients may failure to respond to erythropoietin therapy
• iron deficiency
• inadequate dose
• concurrent infection/inflammation
• hyperparathyroid bone disease
• aluminium toxicity

Drugs in renal failure

Questions regarding which drugs to avoid in renal failure are common in the MRCP
Drugs to avoid in renal failure
• antibiotics: tetracycline, nitrofurantoin
• NSAIDs
• lithium

Drugs likely to accumulate in renal failure - need dose adjustment
• most antibiotics including penicillins, cephalosporins, vancomycin, streptomycin
• digoxin, atenolol
• methotrexate
• sulphonylureas
• frusemide

Drugs relatively safe - use in normal dose
• antibiotics: erythromycin, rifampicin
• diazepam
• warfarin
Papillary necrosis

Causes
• chronic analgesia use
• sickle cell disease
• TB
• acute pyelonephritis
• diabetes mellitus

Features
• fever, loin pain, haematuria
• IVU - papillary necrosis with renal scarring - 'cup & spill'

_________________
DR ALRAZI

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