A novel case of IgA paraneoplastic pemphigus associated with chronic lymphocytic leukemia

Paraneoplastic pemphigus is an autoimmune vesiculobullous and erosive mucocutaneous disease associated with an underlying malignancy. Reported malignancies include chronic lymphocytic leukemia, non-Hodgkin's lymphoma, Castleman's disease, sarcomas, and rarely solid tumors. Patients with paraneoplastic pemphigus develop characteristic IgG autoantibodies against several antigens including members of the plakin family, bullous pemphigoid antigen 1, and desmosomal proteins. IgA pemphigus is another recently characterized immunobullous disease that presents as a vesiculopustular eruption with neutrophilic infiltration and epidermal acantholysis. Mucous membrane involvement is rare. We report what is to our knowledge a unique case with features of both IgA pemphigus and paraneoplastic pemphigus associated with chronic lymphocytic leukemia.