Metastatic ovarian carcinoma-associated subepidermal blistering disease with autoantibodies to both the p200 dermal antigen and the gamma 2 subunit of laminin 5 showing unusual clinical features

Br J Dermatol. 2008 Jun;158(6):1354-7. doi: 10.1111/j.1365-2133.2008.08483.x. Epub 2008 Feb 22.

Abstract

Anti-p200 pemphigoid is an autoimmune subepidermal blistering disease characterized by autoantibodies to an unknown 200-kDa acidic noncollagenous glycoprotein of the lower lamina lucida, whereas antilaminin 5 mucous membrane pemphigoid is characterized by autoantibodies to a major basement membrane extracellular matrix, laminin 5. We report a 64-year-old Japanese woman with a subepidermal blistering disease associated with lymph node metastasis of ovarian clear cell carcinoma 10 years after its surgical treatment. Clinical features showed severe blisters and erosions on multiple mucous membranes (i.e. lip, oral cavity, nose, eye, genitalia and anus) and on both the periungual and subungual regions. This is the first report in which an immunoblot analysis revealed the unusual combination of autoantibodies to both the p200 antigen and the gamma 2 subunit of laminin 5.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma, Clear Cell / immunology
  • Adenocarcinoma, Clear Cell / secondary*
  • Antigens, Neoplasm / immunology*
  • Autoantibodies / immunology*
  • Blister / immunology*
  • Blister / pathology
  • Epidermolysis Bullosa Acquisita / diagnosis*
  • Epidermolysis Bullosa Acquisita / immunology
  • Epidermolysis Bullosa Acquisita / pathology
  • Fatal Outcome
  • Female
  • Humans
  • Immunity, Mucosal
  • Immunoglobulin G / immunology
  • Laminin / immunology*
  • Lymphatic Metastasis / immunology
  • Middle Aged
  • Ovarian Neoplasms / complications*
  • Ovarian Neoplasms / immunology
  • Severity of Illness Index

Substances

  • Antigens, Neoplasm
  • Autoantibodies
  • Immunoglobulin G
  • LAMC2 protein, human
  • Laminin
  • antigen p200