Bruckner-Tuderman L, Vogel A, Rüegger S, Odermatt B, Tönz O, Schnyder U W
Department of Dermatology, University Hospital of Zürich, Switzerland.
J Am Acad Dermatol. 1989 Aug;21(2 Pt 2):425-32. doi: 10.1016/s0190-9622(89)80052-0.
A patient with epidermolysis bullosa simplex with mottled pigmentation is described. Clinical features include blistering of the skin, especially of the extremities; healing without scars; slight atrophy of the skin; and striking mottled pigmentation of the trunk. Histologic examination of a biopsy specimen from freshly frictioned, clinically uninvolved skin indicated a split inside the basal keratinocytes, focal hyperpigmentation of the basal cells, and pigment incontinence without an inflammatory infiltrate. Indirect immunofluorescence demonstrated focal discontinuity of the basement membrane zone. Electron microscopic examination revealed basal keratinocytes with few intact intracellular organelles, aggregated tonofilaments, and subnuclear splitting with the basal parts of the cells adhering to the basement membrane. Both normal basement membrane and zones of irregular and interrupted structures were seen. Hemidesmosomes and anchoring fibrils appeared to be normal.
本文描述了一名患有单纯性大疱性表皮松解症伴斑驳色素沉着的患者。临床特征包括皮肤起水疱，尤其是四肢；愈合后无瘢痕；皮肤轻度萎缩；以及躯干有明显的斑驳色素沉着。对新鲜摩擦后临床上未受累皮肤的活检标本进行组织学检查显示，基底角质形成细胞内出现裂隙、基底细胞局灶性色素沉着增加以及无炎症浸润的色素失禁。间接免疫荧光显示基底膜带局灶性不连续。电子显微镜检查发现基底角质形成细胞内细胞器完整的较少，张力丝聚集，细胞核下分裂，细胞基部附着于基底膜。可见正常的基底膜以及不规则和中断结构区域。半桥粒和锚定原纤维似乎正常。
