PARANEOPLASTIC PEMPHIGUS WITH A PEMPHIGUS VEGETANS-LIKE PLAQUE AS THEONLY CUTANEOUS MANIFESTATION

Paraneoplastic pemphigus (PNP) is a recently recognized autoimmune muc ocutaneous disease associated with a few unusual lymphoreticular neopl asms. Intractable stomatitis is the most constant clinical feature but the cutaneous presentations are characteristically variable. We descr ibe a patient with PNP associated with chronic lymphocytic leukemia wh o presented with severe oral, laryngeal, and pharyngeal involvement. T he only cutaneous finding was a pemphigus vegetans-like plaque, a prev iously undescribed manifestation. The plaque evolved from a flaccid bl ister that formed at a prior intravenous catheter site. The mucosal bi opsy specimen demonstrated faint intercellular staining with IgG, wher eas results for the cutaneous plaque were negative. Indirect immunoflu orescence testing was positive on bath monkey esophagus and rat bladde r epithelium. Immunoprecipitation (IP) studies demonstrated a complex of 4 proteins with molecular weights of 250, 230, 210, and 190 kd, con firming the diagnosis of PNP.