Gulasi Selvi
Department of Pediatrics, Adana Numune Research and Training Hospital, Adana, Turkey.
Iran J Pediatr. 2016 Jul 26;26(5):e2442. doi: 10.5812/ijp.2442. eCollection 2016 Oct.
Lamellar ichthyosis (collodion baby) is a cornification disorder classified under the category of autosomal recessive congenital ichthyosis and characterized by hyperkeratosis. Early-stage retinoid treatment has been shown to improve survival in these patients. In this article, a lamellar ichthyosis case is presented of an infant who had the symptoms at birth and was treated successfully with acitretin.
A term newborn infant presented after delivery. Physical examination showed that the skin on her outer mouth, neck, axillae, and inguinal fold areas had collodion membranes and peelings. On the third day of life, the skin all over her body became dry and seemed similar to parchment paper, with peeling in some areas, as well as ectropion and eclabium development. After her daily bath, liquid Vaseline was applied all over her body, but it did not provide enough benefits. The infant was started on acitretin treatment. On the 14 day of treatment, the skin appeared nearly normal. On the 28 day of life, the infant was discharged.
Early oral retinoid treatment facilitates increased quality of life improves survival rates for ichthyosis patients.
板层状鱼鳞病（胶样婴儿）是一种角化异常疾病，归类于常染色体隐性先天性鱼鳞病，其特征为角化过度。早期维甲酸治疗已被证明可提高这些患者的生存率。本文介绍了一名出生时即出现症状并成功接受阿维A治疗的板层状鱼鳞病婴儿病例。
一名足月儿出生后就诊。体格检查显示其口周、颈部、腋窝和腹股沟褶皱处皮肤有胶样膜和脱皮现象。出生第三天，其全身皮肤变得干燥，类似羊皮纸，部分区域有脱皮，同时出现睑外翻和唇外翻。每日洗澡后，全身涂抹液体凡士林，但效果不佳。遂开始对该婴儿进行阿维A治疗。治疗第14天，皮肤几乎恢复正常。出生第28天，婴儿出院。
早期口服维甲酸治疗有助于提高鱼鳞病患者的生活质量并改善生存率。
