Pemphigoid gestationis with predominant involvement of oral mucous membranes and IgA autoantibodies targeting the C-terminus of BP180.
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.
Shimanovich
Iakov
I
Department of Dermatology, University of Würzburg, Würzburg, Germany.
Skrobek
Christian
C
Rose
Christian
C
Nie
Zhuxiang
Z
Hashimoto
Takashi
T
Bröcker
Eva-Bettina
EB
Zillikens
Detlef
D
D000328:Adult; D000888:Antibodies, Anti-Idiotypic / Q000032:analysis*; D001324:Autoantigens / Q000276:immunology*; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D015151:Immunoblotting; D007070:Immunoglobulin A / Q000032:analysis / Q000276:immunology*; D007163:Immunosorbent Techniques; D009061:Mouth Mucosa / Q000276:immunology*; D024041:Non-Fibrillar Collagens; D006559:Pemphigoid Gestationis / Q000276:immunology*; D010391:Pemphigoid, Bullous / Q000276:immunology*; D011247:Pregnancy; D000097805:Collagen Type XVII
D000888:Antibodies, Anti-Idiotypic; D001324:Autoantigens; D007070:Immunoglobulin A; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII