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Paraneoplastic pemphigoid-pemphigus? Subepidermal bullous disease with pemphigus-like direct immunofluorescence
F Rongioletti1, F Truchetet, A Rebora
1Department of Dermatology, University of Genoa, Italy.
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.