Paraneoplastic immunobullous disease with an epidermolysis bullosa acquisita phenotype: two cases demonstrating remission with treatment of gynaecological malignancy

Australas J Dermatol. 2004 May;45(2):136-9. doi: 10.1111/j.1440-0960.2004.00068.x.

Abstract

Two cases of paraneoplastic immunobullous disease occurring in women with gynaecological malignancies are reported. Both cases demonstrated mechanobullous mucocutaneous blistering as is typically seen in epidermolysis bullosa acquisita. Their immunopathology, however, favoured a dermal-binding mucous membrane pemphigoid (MMP) (or possibly bullous pemphigoid) for patient 1 and laminin-5 MMP for patient 2. Both patients showed resolution of blistering within 1 year of treatment of their malignancies; uterine and ovarian carcinoma, respectively. These cases are of interest because of their paraneoplastic nature; as well as overlapping clinicoimmunopathological features. In addition, patient 2 is, as far as we are aware, the first report of ovarian-carcinoma-associated laminin-5 MMP.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Carcinoma / complications*
  • Carcinoma / surgery
  • Carcinoma, Endometrioid / complications
  • Carcinoma, Endometrioid / surgery
  • Cell Adhesion Molecules / analysis
  • Epidermolysis Bullosa Acquisita / etiology*
  • Epidermolysis Bullosa Acquisita / therapy
  • Female
  • Humans
  • Kalinin
  • Ovarian Neoplasms / complications*
  • Ovarian Neoplasms / surgery
  • Paraneoplastic Syndromes / etiology*
  • Paraneoplastic Syndromes / therapy
  • Pemphigoid, Bullous / diagnosis
  • Phenotype
  • Remission Induction
  • Uterine Neoplasms / complications*
  • Uterine Neoplasms / surgery

Substances

  • Cell Adhesion Molecules