Conflict of interest: none declared.
Clinical dermatology • Concise report
Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid
Article first published online: 23 OCT 2009
© 2009 The Author(s). Journal compilation © 2009 British Association of Dermatologists
Clinical and Experimental Dermatology
Volume 35, Issue 6, pages 614–617, August 2010
How to Cite
Kasperkiewicz, M., Hoppe, U., Zillikens, D. and Schmidt, E. (2010), Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid. Clinical and Experimental Dermatology, 35: 614–617. doi: 10.1111/j.1365-2230.2009.03731.x
- Issue published online: 2 JUL 2010
- Article first published online: 23 OCT 2009
- Accepted for publication 12 June 2009
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.