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.