[Pemphigus]

Pemphigus is a rare autoimmune blistering disorder involving the skin and mucosae. Three types of pemphigus have been described, including pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Diagnosis of the type of pemphigus is made by the combination of clinical features, histological and direct immunofluorescence findings, and serum analysis by indirect immunofluorescence, immunoblot analysis and enzyme-linked-immunosorbent-assay (Elisa). Most patients are treated with oral corticosteroids, sometimes associated with immunosuppressants. Patients with treatment failure or with contraindications to systemic corticosteroids and/or immunosuppressants may be treated with intravenous immunoglobulins or rituximab, an anti-CD20 monoclonal antibody.