Strohal R, Rappersberger K, Pehamberger H, Wolff K
Department of Dermatology I, University of Vienna School of Medicine, Austria.
J Am Acad Dermatol. 1993 Aug;29(2 Pt 2):293-9. doi: 10.1016/0190-9622(93)70179-w.
We describe two patients with pruritic, mainly urticarial or eczematous lesions associated with peripheral blood eosinophilia. No vesicles or blisters developed in either patient throughout the course of the disease (29 and 38 months, respectively). To characterize the clinicopathologic features of these patients we performed histopathologic studies, direct and indirect immunofluorescence, immunoelectron microscopy (patient 2), and immunoprecipitation of both patients' serum. Histopathologic examination revealed a moderate eosinophilic infiltrate partly arranged along the basement membrane zone and focally invading the epidermis. Linear deposits of immunoglobulin and C3 along the dermoepidermal junction were localized within the lamina lucida and over the hemidesmosomal plaques. Immunoprecipitation revealed the presence of circulating autoantibodies against the 230 kd bullous pemphigoid antigen. These findings suggest that our patients had a distinct, nonbullous variant of the pemphigoid spectrum.
我们描述了两名患有瘙痒性皮疹的患者，主要表现为荨麻疹或湿疹样皮损，并伴有外周血嗜酸性粒细胞增多。在疾病过程中（分别为29个月和38个月），两名患者均未出现水疱或大疱。为了明确这些患者的临床病理特征，我们进行了组织病理学研究、直接和间接免疫荧光检查、免疫电子显微镜检查（患者2）以及两名患者血清的免疫沉淀分析。组织病理学检查显示有中度嗜酸性粒细胞浸润，部分沿基底膜带排列，并灶性侵入表皮。免疫球蛋白和C3沿真皮表皮交界处的线性沉积位于透明层内和半桥粒斑块上方。免疫沉淀分析显示存在针对230kd大疱性类天疱疮抗原的循环自身抗体。这些发现提示我们的患者患有类天疱疮谱系中一种独特的非大疱性变异型。
