Kumar Piyush, Mondal Ashim Kumar, Lal Niharika Ranjan, Gharami Ramesh Chandra
Department of Dermatology, Katihar Medical College, Katihar, India.
Skinmed. 2013 Sep-Oct;11(5):308-9.
A 24-year-old man presented with multiple mildly itchy flesh-colored papules and plaques on both legs for the past decade. The lesions were preceded by transient vesicles that contained clear fluid. The papules and plaques used to develop on sites where vesicles had healed. Many family members in three generations had similar lesions (Figure 1). On examination, multiple discrete flesh-colored papules and plaques were found on both lower extremities, extending from the feet up to the knees (Figures 2 and 3). A few of the plaques were excoriated. No vesicles or bullae were noted, and the skin in between the lesions appeared normal. The nail of left great toe was discolored and dystrophic. The rest of the mucocutaneous examination was unremarkable. Bullous lichen planus, Neckam's disease, lichenoid amyloidosis, and epidermolysis bullosa pruriginosa (EBP) were considered as differential diagnoses. Histopathology from the plaque showed a subepidermal cleft with no inflammatory cells. The epidermis was acanthotic at places, and the dermis appeared normal (Figure 4a and 4b). Based on clinical presentation and histopathology, a diagnosis of EBP was made.
一名24岁男性在过去十年中双下肢出现多个轻度瘙痒的肉色丘疹和斑块。这些损害之前有含清亮液体的短暂水疱。丘疹和斑块过去常在水疱愈合的部位出现。三代家族中的许多成员有类似损害（图1）。检查时，在双下肢发现多个散在的肉色丘疹和斑块，从足部延伸至膝部（图2和图3）。少数斑块有抓破。未发现水疱或大疱，损害之间的皮肤外观正常。左拇趾指甲变色且营养不良。其余黏膜皮肤检查未见异常。大疱性扁平苔藓、内卡姆病、苔藓样淀粉样变和瘙痒性大疱性表皮松解症（EBP）被列为鉴别诊断。斑块的组织病理学显示表皮下裂隙，无炎症细胞。表皮在某些部位棘层肥厚，真皮外观正常（图4a和4b）。根据临床表现和组织病理学，诊断为EBP。
