McCuaig C C, Chan L S, Woodley D T, Rasmussen J E, Cooper K D
Department of Dermatology, University of Michigan Medical Center, Ann Arbor.
Arch Dermatol. 1989 Jul;125(7):944-9.
Epidermolysis bullosa acquisita (EBA), rarely reported in childhood, is described in a 10-year-old black girl. The age of onset during infancy and the clinical appearance mimicked hereditary dystrophic epidermolysis bullosa. Epidermolysis bullosa acquisita was diagnosed by direct immunofluorescence of perilesional skin, indirect immunofluorescence on normal epithelium and saline-split skin, direct immunoelectron microscopy, and immunoblotting of the patient's serum sample against partially purified EBA antigen/carboxyl domain of type VII collagen. Differentiation of mechanobullous disease in children is critical in that significant clinical benefit may be achieved in EBA with prednisone and/or dapsone therapy. A search for associated immunologic abnormalities and HLA-DR typing may help our understanding of EBA.
获得性大疱性表皮松解症（EBA）在儿童中鲜有报道，本文描述了一名10岁黑人女孩的病例。其发病年龄在婴儿期，临床表现类似遗传性营养不良性大疱性表皮松解症。通过对皮损周围皮肤进行直接免疫荧光检测、对正常上皮和盐裂皮肤进行间接免疫荧光检测、直接免疫电子显微镜检查以及用患者血清样本针对部分纯化的EBA抗原/ VII型胶原羧基结构域进行免疫印迹分析，确诊为获得性大疱性表皮松解症。儿童机械性大疱病的鉴别诊断至关重要，因为使用泼尼松和/或氨苯砜治疗EBA可能会带来显著的临床益处。寻找相关的免疫异常情况以及进行HLA - DR分型可能有助于我们对EBA的理解。
