[Pemphigus]

Pemphigus is a rare autoimmune bullous disease of the skin and mucosae. It has predisposing genetic factors and, in the case of pemphigus foliaceus, environmental factors; some therapeutic drugs may trigger off pemphigus. The disease often begins with painful buccal erosions, followed by flaccid bullae on healthy skin. Some lesions are erythematous plaques involving the seborrheic areas. Cytology shows acantholysis and histology, intraepidermal cleavage. Direct immunofluorescence confirms the diagnosis by showing a network deposit of immunoglobulins and complement. The titres of circulating antibodies to the intracellular substance vary with the course of the disease. The loss of cohesion between keratinocytes is induced by autoantibodies directed against antigens of the epidermal cell junction zone; the molecular weight of these antibodies is 130 and 85 kD for pemphigus vulgaris and 160 and 85 for superficial pemphigus. Paraneoplastic pemphigus is a recently individualized entity. Treatment consists of systematic corticosteroid therapy, sometimes associated with immunosuppressants; it has improved the prognosis of this once lethal disease.