Paraneoplastic Pemphigus Is a Distinct Neoplasia-Induced Autoimmune Disease

A relationship between pemphigus and internal malignancy has been recognized for a long time. Some authors have suggested that patients with pemphigus have a greater incidence of malignancy, perhaps related to immunosuppressive treatment,1while others have noted a strong statistical association with both thymic and nonthymic neoplasms.2Immunoglobulins isolated from tumors have been shown to have reactivity similar to pemphigus intercellular substance antibodies by indirect immunofluorescence.3The importance of these findings was unclear until Anhalt and colleagues4published their seminal observations regarding five patients with a distinct blistering disorder and underlying malignancy. Serum samples obtained from their patients all showed unique banding patterns by immunoprecipitation that corresponded to autoantibodies to desmoplakins I and II, the major bullous pemphigoid antigen, and an as yet unidentified 190-kd protein. Clinical findings included a blistering eruption with stomatitis and conjunctivitis. Treatment results were disappointing, as four of the five patients were known to die of their disease.