(1997; 132 pages) [French] [Spanish]
Pemphigus vulgaris and a less severe variant, pemphigus foliaceous, in which the blisters are more superficial and the mucous membranes are not involved, are the most common variants of this condition. Occasionally, particularly after exposure to sunlight, the lesions become confluent and lead to a form of exfoliative dermatitis.
Pemphigus vulgaris is typically a disease of middle age. It is most prevalent in eastern India, in southeast Asia and among persons of Jewish descent. Sometimes cases occur after exposure to penicillamine or captopril and associations have also been described with thymoma, myasthenia gravis and other immunopathological conditions. Painful ulcers often develop in the mouth several months before the skin lesions appear. Fragile flaccid blisters develop in the deeper layers of the epidermis in non-inflamed skin, typically on the scalp, chest and intertriginous areas. They extend readily on digital pressure and often rupture, leaving large areas of weeping denuded skin. Ulceration of mucosal surfaces is common and can be extensive. Untreated, most patients die within 5 years from secondary sepsis, electrolyte imbalance and/or emaciation.
Pemphigus vegetans is a variant of pemphigus vulgaris, in which the skin lesions are largely limited to the scalp and intertriginous areas, and appear as crusted papillomatous lesions. However, most cases eventually progress to pemphigus vulgaris.
Brazilian pemphigus or fogo selvagem is a rapidly spreading form of pemphigus, which occurs epidemically in parts of Brazil.
Fluid and electrolyte balance should be carefully monitored. Systemic antimicrobial therapy may be needed for patients with secondary infections.
Compresses soaked in 5% aluminium diacetate, 0.005% silver nitrate, or a 0.01% potassium permanganate solution should be applied to the affected areas every 4 hours. This will facilitate the removal of skin debris from blistered areas and reduce the risk of secondary infection.
High doses of oral corticosteroids are usually required for control. The dose should be gradually reduced once stabilization occurs to the lowest dose that maintains remission. Oral prednisolone or prednisone can be used. Addition of immunosuppressive drugs, such as azathioprine or cyclophosphamide, is often useful when the patient is unable to tolerate the minimum corticosteroid dose that maintains remission. The immunosuppressive effect develops gradually and is usually undetectable until 4-6 weeks after treatment has started. Corticosteroids should be withdrawn before the immunosuppressive drug is discontinued.