Tay Y K, Weston W L
Department of Dermatology, University of Colorado Health Sciences Center, Denver 80262, USA.
Pediatr Dermatol. 1996 Jul-Aug;13(4):306-9. doi: 10.1111/j.1525-1470.1996.tb01246.x.
A 6-year-old girl with epidermolysis bullosa simplex (EBS) is described. Clinical features include generalized herpetiform blistering of the skin, healing without scars, intraoral blistering, nail dystrophy, palmoplantar keratoderma, and improvement with age. An unusual feature was the presence of a striking mottled pigmentation involving the arms, trunk, and legs. Histology, immunofluorescence, and electron microscopy of a fresh lesion showed an intraepidermal split within the basal keratinocytes, focal hyperpigmentation of the basal cells without an inflammatory infiltrate, and tonofilament clumping. Our patient illustrates the clinical features of Dowling-Meara EBS with the pigmentary changes of EBS with mottled pigmentation. The relationship between EBS of Dowling-Meara and EBS with mottled pigmentation is discussed.
本文描述了一名患有单纯性大疱性表皮松解症（EBS）的6岁女孩。临床特征包括全身性疱疹样皮肤水疱、愈合后无瘢痕、口腔内水疱、指甲营养不良、掌跖角化病以及随年龄增长病情改善。一个不寻常的特征是双臂、躯干和腿部出现明显的斑驳色素沉着。对一个新鲜皮损进行组织学、免疫荧光和电子显微镜检查，结果显示基底角质形成细胞内表皮内裂隙、基底细胞局灶性色素沉着增加且无炎症浸润以及张力细丝聚集。我们的患者表现出Dowling-Meara型EBS的临床特征以及伴有斑驳色素沉着的EBS的色素变化。本文还讨论了Dowling-Meara型EBS与伴有斑驳色素沉着的EBS之间的关系。
