Wakelin S H, Allen J, Wojnarowska F
Department of Dermatology, Churchill Hospital, Oxford, U.K.
Br J Dermatol. 1995 Oct;133(4):615-8. doi: 10.1111/j.1365-2133.1995.tb02715.x.
Bullous pemphigoid (BP) is an acquired bullous disorder which predominantly affects the elderly. It is rare in children, and may be clinically indistinguishable from other immunobullous disorders. As routine histology may be non-specific, a definitive diagnosis of childhood BP usually depends on the results of direct and indirect immunofluorescence investigations. We report a 5-year-old girl who developed bullous pemphigoid, associated with atypical immunofluorescence findings. Indirect immunofluorescence on split-skin showed a pure dermal pattern of IgG binding. This is usually suggestive of epidermolysis bullosa acquisita, but Western immunoblotting was positive with epidermal extracts, confirming a diagnosis of BP. Dermal binding on split-skin occurs in about 5% of adult cases of BP, and has not been reported previously in childhood BP.
大疱性类天疱疮(BP)是一种获得性大疱性疾病,主要影响老年人。在儿童中罕见,在临床上可能与其他免疫性大疱性疾病难以区分。由于常规组织学可能不具有特异性,儿童BP的确诊通常取决于直接和间接免疫荧光检查结果。我们报告一名5岁女孩患大疱性类天疱疮,伴有非典型免疫荧光表现。对分离皮肤进行间接免疫荧光显示IgG结合呈单纯真皮模式。这通常提示获得性大疱性表皮松解症,但用表皮提取物进行的蛋白质免疫印迹呈阳性,证实为BP诊断。分离皮肤的真皮结合在约5%的成人BP病例中出现,此前在儿童BP中尚未见报道。