Sacher C, König C, Scharffetter-Kochanek K, Krieg T, Hunzelmann N
Department of Dermatology, University of Cologne, Germany.
Dermatology. 2001;202(1):54-7. doi: 10.1159/000051588.
Bullous pemphigoid is an autoimmune disease of the skin characterized by the production of antibodies directed at structures of the basement membrane zone (BMZ) leading to subepidermal blisters. Several causative triggers have been described in the literature, among them UV light. Here, we report on a 73-year-old Caucasian female with disseminated morphea who developed blisters on her extremities after receiving whole-body UVA-1 phototherapy. The initial differential diagnosis of a phototoxic versus photoallergic reaction was ruled out as the lesions continued to spread after discontinuation of phototherapy. Histological and direct immunofluorescence examination showing a subepidermal blister and linear IgG deposits along the BMZ along with detection of circulating anti-BMZ antibodies led to the diagnosis of bullous pemphigoid. Immunosuppressive therapy resulted in regression of all blisters. After ruling out other possible causes, such as neoplasias or drugs, we conclude that UVA-1 has to be regarded as the most likely trigger of the disease.
大疱性类天疱疮是一种皮肤自身免疫性疾病，其特征是产生针对基底膜带（BMZ）结构的抗体，导致表皮下水疱。文献中描述了几种致病诱因，其中包括紫外线。在此，我们报告一例73岁的白种女性，患有播散性硬斑病，在接受全身UVA - 1光疗后，其四肢出现水疱。由于停止光疗后皮损仍继续扩散，排除了光毒性与光变应性反应的初步鉴别诊断。组织学和直接免疫荧光检查显示表皮下水疱和沿BMZ的线性IgG沉积，同时检测到循环抗BMZ抗体，从而诊断为大疱性类天疱疮。免疫抑制治疗使所有水疱消退。在排除其他可能原因，如肿瘤或药物后，我们得出结论，UVA - 1必须被视为该疾病最可能的诱因。
