Pemphigus vulgaris

Pemphigus vulgaris is a potentially fatal blistering disease occurring in all races but commoner in Ashkenazi Jews and possibly in people from the Indian subcontinent. Onset is usually in middle age and both sexes are affected equally. Prior to the development of oral steroids this condition was frequently fatal. The development of autoantibodies against the desmosomal protein, desmoglein 3, is pathogenic in this disease and they can be measured experimentally as markers of disease activity. Rarely the disease can be drug induced (e.g. penicillamine or ACE inhibitors).

Skin biopsy shows a superficial intraepidermal split just above the basal layer with acantholysis (separation of individual cells). In the rarer variant, pemphigus foliaceous (characterized by anti-desmoglein 1 autoantibodies), the split is higher in the upper epidermis. Both direct IMF of skin (perilesional) and indirect IMF using patients' serum show intercellular staining of IgG within the epidermis.

Clinical features and symptoms of Pemphigus vulgaris

Mucosal involvement (especially oral ulceration) is common and may be the presenting sign in up to 50% of cases. This may then be followed by the appearance of flaccid blisters, particularly involving the trunk. They tend to be sore rather than itchy. Blistering usually becomes widespread but the blisters rapidly denude; thus pemphigus often presents with erythematous, weeping erosions. Blisters can be extended with gentle sliding pressure (Nikolsky's sign). Flexural lesions often have a vegetative appearance. In pemphigus foliaceous the blisters and erosions often start in a seborrhoeic distribution (scalp, face and upper chest) before becoming more widespread.

Pathology of Pemphigus vulgaris

The bullae of Pemphigus vulgaris are intra-epidermal and irregular in shape with acute lateral margins. They are formed by the separation of acantholytic epidermal cells (Tzanck cells). Acantholytic cells may be in the bullae cavity. Dermis beneath the bulla shows number of inflammatory cells including a few lymphocytes and plasma cells.

Treatment and cure of Pemphigus vulgaris

This is with very high-dose oral prednisolone (60-100 mg daily) or pulsed methylprednisolone and this may need to be lifelong. Therefore other immunosuppressants such as azathioprine (or occasionally cyclophosphamide or ciclosporin) are used as steroid-sparing agents. Intravenous immunoglobulin infusions can be useful in resistant cases.

Whilst treatment is normally effective, perhaps up to 10% of patients may succumb, either because of complications of the disease or more commonly from side-effects of the treatment.

Use of azathioprine . Azathioprine can cause bone marrow suppression and an allergic hepatitis. Therefore blood count and liver biochemical tests should be regularly monitored during therapy (every 6 weeks). Pregnancy should be avoided. Long-term use with other immunosuppressants causes a slightly increased risk of malignancy, especially of the skin.

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