Is pemphigus herpetiformis an entity?

Clinical, histologic, and immunofluorescence studies were performed in 15 patients with pemphigus herpetiformis. The initial diagnosis was dermatitis herpetiformis, IgA linear bullous dermatosis or bullous pemphigoid. The histology varied depending on the character of skin lesions, and showed eosinophilic spongiosis or slight acantholysis and/or polymorphonuclear papillary microabscesses. Direct immunofluorescence showed invariably intercellular IgG staining, and indirect immunofluorescence on monkey esophagus substrate was positive, at some periods, in five cases. About half of the patients responded to therapy with sulfones and prednisone, and only one patient responded to sulfones alone. Half of the patients required combined therapy with prednisone and cyclophosphamide or with higher doses of prednisone. In consecutive relapses, nine patients retained the pattern of pemphigus herpetiformis: in the others, lesions were mostly of pemphigus seborrheicus-foliaceus type.