General Pediatric Surgery unit, Mother and Child Department, Mohamed VI Teaching Hospital, Cadi Ayyad University, Marrakech, Morocco.
Pan Afr Med J. 2024 Jun 9;48:52. doi: 10.11604/pamj.2024.48.52.43784. eCollection 2024.
Aplasia cutis congenita (ACC) is a rare congenital disorder defined as a congenital skin defect, characterized by the absence of all skin layers at birth. The most frequent presentation is a small erythematous ulcerated or scar-like alopecic ectodermal lesion on the scalp vertex. However, extensive cutis aplasia of the trunk is extremely uncommon. Clinical and radiological evaluation defined the appropriate treatment. We herein report a rare case of a large aplasia cutis congenita of the trunk occurring in a male newborn managed with sulfadiazine silver 1% dressing, complete healing was achieved in about a month. The report highlights that conservative treatment is a highly effective and practical option for managing non-scalp extensive ACC.
先天性皮肤发育不全(ACC)是一种罕见的先天性疾病,定义为先天性皮肤缺陷,其特征是出生时所有皮肤层缺失。最常见的表现是头皮顶点处有一个小的红斑性溃疡性或瘢痕性无毛的外胚层病变。然而,广泛的躯干皮肤发育不全极为罕见。临床和影像学评估确定了适当的治疗方法。本文报告了一例罕见的男性新生儿躯干大面积先天性皮肤发育不全的病例,采用磺胺嘧啶银 1%敷料治疗,约一个月即可完全愈合。该报告强调,对于非头皮广泛 ACC,保守治疗是一种高度有效和实用的选择。