McGrath J A, Burrows N P, Russell Jones R, Eady R A
St. John's Institute of Dermatology, St. Thomas's Hospital, London, UK.
Dermatology. 1993;186(1):68-71. doi: 10.1159/000247306.
A 46-year-old woman with the Dowling-Meara variant of epidermolysis bullosa simplex (DM-EBS) presented with worsening recurrent, pruritic, circinate crops of clear and haemorrhagic herpetiform blisters affecting her trunk and limbs. Electron microscopy showed tonofilament aggregation and an intra-epidermal level of blister formation confirming a diagnosis of DM-EBS rather than an acquired immunobullous disorder. Antihistamines failed to control the intense pruritus, but dapsone (up to 150 mg daily) was beneficial. Mutations of basal cell keratin genes (K14 and K5) are thought to be of aetiopathological significance in this form of epidermolysis bullosa, but the underlying cellular mechanisms responsible for the clinical deterioration and severe itching in this adult patient are not yet clear.
一名患有单纯性大疱性表皮松解症Dowling-Meara变异型（DM-EBS）的46岁女性，反复出现瘙痒性、环形分布的清亮和出血性疱疹样水疱，累及躯干和四肢，且症状逐渐加重。电子显微镜检查显示张力丝聚集以及表皮内水疱形成，从而确诊为DM-EBS而非获得性免疫性大疱病。抗组胺药未能控制剧烈瘙痒，但氨苯砜（每日剂量高达150毫克）有效。基底细胞角蛋白基因（K14和K5）的突变被认为在这种形式的大疱性表皮松解症中具有病因病理意义，但导致该成年患者临床病情恶化和严重瘙痒的潜在细胞机制尚不清楚。
