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PARANEOPLASTIC PEMPHIGOID‐PEMPHIGUS? SUBEPIDERMAL BULLOUS DISEASE WITH PEMPHIGUS‐LIKE DIRECT IMMUNOFLUORESCENCE

1993; Wiley; Volume: 32; Issue: 1 Linguagem: Inglês

10.1111/j.1365-4362.1993.tb00964.x

1365-4632

Franco Rongioletti, F. Truchetet, Alfredo Rebora,

Coagulation, Bradykinin, Polyphosphates, and Angioedema

Abstract Background. Paraneoplastic pemphigus is a recently described variant of pemphigus, in which classic immunologic features of pemphigus are associated with subepidermal erythema multiforme‐like bullae. Case Reports. Two patients with underlying malignant neoplasm had a bullous disease, which had clinical and histologic features suggestive of bullous pemphigoid while direct immunofluorescence revealed intercellular deposits of IgG and C3 throughout the malpighian layer of epidermis, a pattern usually diagnostic for pemphigus. Indirect immunofluorescence studies were negative. One mg/kg/day prednisone was enough to cure the eruption in a couple of weeks without any recurrence. Conclusions. The disease differs from the recently reported paraneoplastic pemphigus and from other bullous diseases associating circulating pemphigus‐like antibodies with clinical, histologic, and often even immunologic features of bullous pemphigoid.