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Home :: Skin Disorders :: Pemphigus Vulgaris

Pemphigus Vulgaris

Pemphigus vulgaris (PV) is a serious, acute or chronic, bullous, autoimmune disease of skin and mucous membranes that is often fatal unless treated with immunosuppressive agents. It is the prototype of the pemphigus family, a group of autoimmune acuntholytic blistering diseases.

Causes of Pemphigus Vulgaris

A loss of the normal cell-to-cell adhesion in the epidermis occurs as a result of circulating antibodies of the IgG class; these antibodies bind to cell surface glycoproteins (pemphigus antigens; desmoglein 3, a member of the cadherin superfamily) of the epidermis and induce acantholysis, probably by the activation of serine proteases.

Signs and symptoms of Pemphigus Vulgaris

Pemphigus Vulgaris usually starts in the oral mucosa, and months may elapse before skin lesions occur; lesions may be localized for 6 to 12 months, after which generalized bullae occur. Less frequently there may be a generalized, acute eruption of bullae from the beginning. No pruritus, but burning and pain. Painful and tender mouth lesions may prevent adequate food intake. Epistaxis, hoarseness, dysphagia. Weakness, malaise, weight loss (with prolonged mouth involvement).


Can be a difficult problem if only mouth lesions are present. Biopsy of the skin and mucous membrane, direct immunofluorescence, and demonstration of circulating autoantibodies confirm a high index of suspicion.

Treatment of Pemphigus Vulgaris

The primary aim of treatment is to decrease blister formation, prevent infections and promote healing of blisters and erosions. Oral corticosteroids are the mainstay of medical treatment for controlling the disease. Since their use, many deaths from pemphigus vulgaris have been prevented (mortality rate dropped from 99% to 5-15%)

Systemic corticosteroid therapy is effective in reducing or eliminating the clinical manifestations of pemphigus vulgaris, although doses of prednisone may have to be as high as 400 mg. daily for patients with severe involvement. Topical corticosteroids can be used as an adjunct therapy if the bullae are confined to oral mucosa.

Glucocorticoids 2 to 3 mg/kg of body weight of prednisone until cessation of new blister formation and disappearance of Nikolsky's signs. Then rapid reduction to about half the initial dose until patient is almost clear, followed very slow tapering of dose to minimal effective maintenance dose.

Clinical, for improvement of skin lesions and development of drug-related side effects. Laboratory monitoring of pemphigus antibody titers and for hematologic and metabolic indicators of glucocorticoid- and/or immunosuppressive-induced adverse effects.

Prevention of Pemphigus Vulgaris
  • Be sure to consult your doctor if you are thinking about ceasing to take your corticosteroids. It is dangerous to do so suddenly.
  • Patients should minimize activities that may traumatise the skin and mucous membranes during active phases of the disease.
  • If you have erosions in your mouth it may be sensible to avoid eating heavily spiced or hard foods.
  • Patients with paraneoplastic pemphigus may have to forgo treatment of their vesiculobullous disease until the underlying neoplasm is controlled, although supportive therapy can be instituted.

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