Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid.
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.
Kasperkiewicz
M
M
Department of Dermatology, University of Lübeck, 23538 Lübeck, Germany.
Hoppe
U
U
Zillikens
D
D
Schmidt
E
E
D000368:Aged; D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D002990:Clobetasol / Q000627:therapeutic use; D004318:Doxycycline / Q000627:therapeutic use; D006801:Humans; D008297:Male; D024041:Non-Fibrillar Collagens / Q000276:immunology*; D010391:Pemphigoid, Bullous / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D012008:Recurrence; D013258:Steroids, Chlorinated / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome; D000097805:Collagen Type XVII
D000900:Anti-Bacterial Agents; D001323:Autoantibodies; D001324:Autoantigens; D002990:Clobetasol; D004318:Doxycycline; D024041:Non-Fibrillar Collagens; D013258:Steroids, Chlorinated; D000097805:Collagen Type XVII