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Pemphigus Vulgaris

Title               

Ñ    Pemphigus Vulgaris

Definition

Ñ    Autoimmune

Ñ    Superficial:

Intraepidermal

Ñ    Blistering

Ñ    Disease of the skin     

Pathogenesis

Causes

Ñ    Autoimmune disease

Ñ    Epidemiology:

Common in Ashkenazi Jews and in the Indian subcontinent

Ñ    Drug induced (rarely):

Penicillamine

Captopril

Rifampicin

Meprobamate

Pathological Process

Ñ    Autoantibodies against desmoglein 3

Ñ    Immunofluorescence (direct and indirect) shows intercellular staining of IgG within the epidermis

Ñ    Skin biopsy shows a superficial intraepidermal split just above the basal layer:

In pemphigus foliaceous the split is higher

Ñ    Acantholysis, intercellular clefts and eventually broad based intraepithelial blisters occur

Clinical Features

History

Age

Ñ    Middle age

Sex

Ñ    Males = Females

IS

Ñ    Rash:

Sore not itchy

Examination

IS

Ñ    Superficial, flaccid blisters 

Ñ    Rupture easily

Ñ    Leave erythematous weeping erosions

Ñ    Blisters may be extended with gentle pressure:

Nikolsky’s sign

GIT

Ñ    Superficial erosions in the mouth:

Blisters rupture easily

Investigations

Tissue Diagnosis

Skin biopsy

Ñ    Superficial intraepidermal split just above the basal layer

Ñ    Acantholysis:

Separation of individual cells

Ñ    Direct and indirect immunofluorescence:

Intercellular staining of IgG within the epidermis

Management

Control

Drugs

Ñ    Steroids:

High dose

Ñ    Immunosuppression:

Azathioprine, methotrexate, cyclophosphamide, ciclosporin, mycophenolate mofetil

Ñ    Gold salts

Lifestyle Adjustments

Ñ    Liquids drunk should not be strongly osmotic

Ñ    Soft diet

Staffing

Ñ    Expert nursing

Prognosis

Ñ    Cure may be possible

Ñ    Most patients off treatment in 2 years

Ñ    Up to 25% of patients die of the disease or as a consequence of treatment

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