Paraneoplastic pemphigoid-pemphigus? Subepidermal bullous disease with pemphigus-like direct immunofluorescence

Int J Dermatol. 1993 Jan;32(1):48-51. doi: 10.1111/j.1365-4362.1993.tb00964.x.

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.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Complement C3 / analysis
  • Female
  • Fluorescent Antibody Technique
  • Humans
  • Immunoglobulin G / analysis
  • Male
  • Middle Aged
  • Paraneoplastic Syndromes / metabolism
  • Paraneoplastic Syndromes / pathology*
  • Pemphigoid, Bullous / metabolism
  • Pemphigoid, Bullous / pathology*
  • Pemphigus / metabolism
  • Pemphigus / pathology*

Substances

  • Complement C3
  • Immunoglobulin G