Hbibi Mohamed, Abourazzak Sana, Idrissi Mounia, Chaouki Sana, Atmani Samir, Hida Moustapha
Department of Pediatrics, University Hospital Hassan II Fès, Morocco.
Pan Afr Med J. 2015 Jan 5;20:3. doi: 10.11604/pamj.2015.20.3.5878. eCollection 2015.
Cutis laxa (CL) is a heterogeneous group of inherited and acquired connective tissue disorders characterized by a loose skin and variable systemic involvement (inguinal hernia, cardiopulmonary disease, and emphysema). Autosomal dominant, autosomal recessive and x-linked recessive patterns have been described in the inherited forms. Acquired forms of this disease have been associated with a previous inflammatory skin disorder (urticaria…). The characteristic symptomatological pattern is resulting from paucity of elastic fibers. We report an 18 months old baby boy with a congenital cutis laxa. He was admitted in pediatric unit for respiratory disorders. The diagnosis of CL syndrome is based on clinical assessment of typical skin features and the associated extracutaneous finding.
皮肤松弛症(CL)是一组异质性的遗传性和获得性结缔组织疾病,其特征为皮肤松弛及不同程度的全身受累(腹股沟疝、心肺疾病和肺气肿)。遗传性皮肤松弛症已被描述有常染色体显性、常染色体隐性和X连锁隐性遗传模式。该疾病的获得性形式与既往炎性皮肤病(荨麻疹……)有关。其特征性症状模式是由弹性纤维缺乏所致。我们报告一名18个月大患先天性皮肤松弛症的男婴。他因呼吸系统疾病入住儿科病房。CL综合征的诊断基于对典型皮肤特征及相关皮肤外表现的临床评估。