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Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid.
Clin Exp Dermatol. 2010 Aug; 35(6):614-7.CE

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.

Authors+Show Affiliations

Department of Dermatology, University of Lübeck, 23538 Lübeck, Germany.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

19874345

Citation

Kasperkiewicz, M, et al. "Relapse-associated Autoantibodies to BP180 in a Patient With Anti-p200 Pemphigoid." Clinical and Experimental Dermatology, vol. 35, no. 6, 2010, pp. 614-7.
Kasperkiewicz M, Hoppe U, Zillikens D, et al. Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid. Clin Exp Dermatol. 2010;35(6):614-7.
Kasperkiewicz, M., Hoppe, U., Zillikens, D., & Schmidt, E. (2010). Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid. Clinical and Experimental Dermatology, 35(6), 614-7. https://doi.org/10.1111/j.1365-2230.2009.03731.x
Kasperkiewicz M, et al. Relapse-associated Autoantibodies to BP180 in a Patient With Anti-p200 Pemphigoid. Clin Exp Dermatol. 2010;35(6):614-7. PubMed PMID: 19874345.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid. AU - Kasperkiewicz,M, AU - Hoppe,U, AU - Zillikens,D, AU - Schmidt,E, Y1 - 2009/10/23/ PY - 2009/10/31/entrez PY - 2009/10/31/pubmed PY - 2011/2/12/medline SP - 614 EP - 7 JF - Clinical and experimental dermatology JO - Clin Exp Dermatol VL - 35 IS - 6 N2 - 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. SN - 1365-2230 UR - https://www.unboundmedicine.com/medline/citation/19874345/Relapse_associated_autoantibodies_to_BP180_in_a_patient_with_anti_p200_pemphigoid_ L2 - https://doi.org/10.1111/j.1365-2230.2009.03731.x DB - PRIME DP - Unbound Medicine ER -