Ogilvie P, Trautmann A, Dummer W, Rose C, Bröcker E B, Zillikens D
Universitäts-Hautklinik Würzburg.
Hautarzt. 2000 Jan;51(1):25-30. doi: 10.1007/s001050050006.
Pemphigoid gestationis (PG) is a rare pregnancy-associated autoimmune bullous disease characterized by autoantibodies to the 180 kD bullous pemphigoid antigen (BP180). The clinical spectrum of PG is polymorphic and for diagnostic purposes, a skin biopsy is usually taken demonstrating the deposition of autoantibodies.
From 2 patients, skin biopsies were obtained for histopathologic and immunofluorescence studies. Circulating autoantibodies were characterized by immunoblotting and ELISA using a recombinant form of the immunodominant BP180 NC16 A domain.
The 2 PG patients described here did not show blisters but complained about severe itching. In the first case, PG presented in the first trimester of the second pregnancy as an erythema-multiforme-like disease. The second patient developed urticarial plaques a few days after delivery. PG was diagnosed by the detection of autoantibodies against recombinant BP180 NC16 A by immunoblot and ELISA analysis and confirmed by linear deposits of C3 at the cutaneous basement membrane zone on direct immunofluorescence microscopy. Skin lesions healed with oral prednisolone.
In our two patients, non-bullous PG could be diagnosed by serological tests. Immunoblotting and ELISA might be sensitive and specific tools when screening sera of patients with pruritic skin lesions in pregnancy for the presence of autoantibodies to BP180. In some cases, these newer techniques may make a skin biopsy unnecessary.
妊娠类天疱疮(PG)是一种罕见的与妊娠相关的自身免疫性大疱性疾病,其特征为针对180kD大疱性类天疱疮抗原(BP180)的自身抗体。PG的临床谱具有多形性,为明确诊断,通常会进行皮肤活检以显示自身抗体的沉积。
从2例患者获取皮肤活检样本用于组织病理学和免疫荧光研究。使用免疫印迹法和酶联免疫吸附测定(ELISA),以重组形式的免疫显性BP180 NC16 A结构域对循环自身抗体进行特征分析。
本文描述的2例PG患者未出现水疱,但均主诉严重瘙痒。第一例患者在第二次妊娠的孕早期表现为多形红斑样疾病。第二例患者在分娩后数天出现荨麻疹样斑块。通过免疫印迹和ELISA分析检测到针对重组BP180 NC16 A的自身抗体,从而诊断为PG,并通过直接免疫荧光显微镜检查在皮肤基底膜区发现C3线性沉积得以证实。皮肤病变经口服泼尼松龙后愈合。
在我们的2例患者中,非大疱性PG可通过血清学检测进行诊断。当筛查妊娠瘙痒性皮肤病变患者的血清中是否存在针对BP180的自身抗体时,免疫印迹法和ELISA可能是敏感且特异的工具。在某些情况下,这些新技术可能无需进行皮肤活检。