Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid

Clin Exp Dermatol. 2010 Aug;35(6):614-7. doi: 10.1111/j.1365-2230.2009.03731.x. Epub 2009 Oct 23.

Abstract

Anti-p200 pemphigoid and bullous pemphigoid (BP) are autoimmune subepidermal blistering diseases characterized by autoantibodies to a 200-kDa dermal antigen (p200) and two hemidesmosomal proteins (BP180 and BP230), respectively. We report a 70-year-old man with haemorrhagic blisters, widespread crusted erosions, and the immunopathological characteristics of anti-p200 pemphigoid. Treatment with doxycycline, topical corticosteroids and immunoadsorption led to rapid clinical remission. However, 19 weeks later, a relapse occurred with generalized itchy urticarial erythema and tense blisters. At this time, both strong dermal and epidermal IgG staining was detected by indirect immunofluorescence microscopy on salt-split skin, and autoantibodies against both p200 and the 16th noncollagenous (NC16A) domain of BP180 were found. Interestingly, the relapse was associated not only with the detection of autoantibodies to a second autoantigen (BP180), but also with an altered clinical phenotype. This case was a unique occasion to directly monitor the emergence of intermolecular epitope spreading during the course of an autoimmune bullous disorder.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Anti-Bacterial Agents / therapeutic use*
  • Autoantibodies / immunology*
  • Autoantigens / immunology*
  • Clobetasol / therapeutic use
  • Collagen Type XVII
  • Doxycycline / therapeutic use
  • Humans
  • Male
  • Non-Fibrillar Collagens / immunology*
  • Pemphigoid, Bullous / drug therapy
  • Pemphigoid, Bullous / immunology*
  • Pemphigoid, Bullous / pathology
  • Recurrence
  • Steroids, Chlorinated / therapeutic use*
  • Time Factors
  • Treatment Outcome

Substances

  • Anti-Bacterial Agents
  • Autoantibodies
  • Autoantigens
  • Non-Fibrillar Collagens
  • Steroids, Chlorinated
  • Clobetasol
  • Doxycycline