Pemphigoid gestationis with predominant involvement of oral mucous membranes and IgA autoantibodies targeting the C-terminus of BP180 - 01/09/11
Abstract |
Pemphigoid gestationis (PG) is an autoimmune pregnancy-associated subepidermal blistering disease. It usually affects skin and, rarely, mucous membranes. In the vast majority of patients with PG, the autoimmune response is directed to the membrane-proximal NC16A domain of the 180-kd bullous pemphigoid (BP) antigen (BP180) and is mediated by IgG1 and IgG3 autoantibodies. We report the case of a patient with PG associated with extensive lesions on oral mucous membranes. Immunoblotting studies demonstrated the presence of circulating IgA autoantibodies in the patient's serum that were exclusively directed to a 49 amino acid stretch on the C-terminal portion of the BP180 ectodomain located 800 amino acids downstream from NC16A. This C-terminal stretch of BP180 has previously been demonstrated to localize to the lamina lucida/lamina densa interface and to be recognized by IgG and IgA antibodies in a subgroup of patients with cicatricial pemphigoid as well as by IgG autoantibodies in some BP sera. Our patient's lesions healed without scarring within 6 weeks after delivery of a healthy child. The findings in this patient extend the clinical and immunopathologic spectrum of PG. (J Am Acad Dermatol 2002;47:780-4.)
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Supported by grant 98.073.2 from the Wilhelm Sander-Stiftung, Munich, Germany (to D. Z.). |
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Reprint requests: Detlef Zillikens, MD, Department of Dermatology, University of Würzburg, Josef-Schneider-Str 2, 97080 Würzburg, Germany. E-mail: zillikens-d.derma@mail.uni-wuerzburg.de. |
Vol 47 - N° 5
P. 780-784 - novembre 2002 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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