A PARANEOPLASTIC MIXED BULLOUS SKIN-DISEASE ASSOCIATED WITH ANTI-SKINANTIBODIES AND A B-CELL LYMPHOMA

Background.-The full spectrum of bullous diseases associated with unde rlying cancers remains to be fully defined. Observation.-We describe a patient with a mixed bullous disease exhibiting combined features of cicatricial pemphigoid and pemphigus and associated with a B-cell lymp homa producing an IgM paraprotein to intercellular antigens in human s kin. The patient had the clinical features of cicatricial pemphigoid a nd the histologic and immunofluorescence abnormalities of both cicatri cial pemphigoid and pemphigus. These included oral and cutaneous erosi ons; ocular scarring; subbasal and acantholytic intraepidermal bullae; and circulating and tissue-fixed basement membrane zone and intercell ular antibodies. The antibodies were directed to a 140-kd antigen in d ermal extracts of skin split with 1 mol/L of sodium chloride and to an tigens with approximate molecular weights of 150,180,230, and 285 kd i n the dermal extract. In contrast to paraneoplastic pemphigus, the int ercellular antibodies did not react to mammalian bladder. The intercel lular antibodies were of the IgM class and were associated with the pa raprotein produced by the malignant B cells. Conclusions.-We believe t hat this condition represents a novel bullous disease, which we refer to as paraneoplastic mixed bullous disease. This condition illustrates that distinct bullous diseases are associated with paraneoplastic syn dromes and that at least one possible mechanism for such eruptions is the production of anti-skin antibodies by malignant B cells.