Özyurt Banu Mutlu, Onay Özge Sürmeli, Ersoy Özlem
Mersin Women's and Children's Hospital, Neonatal Intensive Care Unit, Mersin, Turkey.
Turk Pediatri Ars. 2018 Mar 1;53(1):51-56. doi: 10.5152/TurkPediatriArs.2018.3704. eCollection 2018 Mar.
Ichthyosis is a clinical skin cornification disorder characterized by hyperkeratosis. Lamellar ichthyosis is a rare form of ichthyosis (collodion baby), which is autosomal recessive. Diagnostic clinical findings can be confirmed with skin biopsy and genetic analysis. The principles of treatment are moistening the skin, prevention of dehydration, and use of keratolytic agents. Systemic retinoic acid (0.5-1 mg/kg/day) is reported to provide dramatic benefits in the treatment of lamellar ichthyosis and congenital ichthyosiform erythroderma. In this study, the efficiency of oral retinoic acid treatment is presented to show a dramatic improvement in five patients who were diagnosed as collodion babies and admitted to The Neonatal Intensive Care Unit after birth. None had other congenital problems. Only two patients were diagnosed as having sepsis. Hair loss was the only adverse effect of treatment in all five patients. With this series of collodion baby, we wanted to the draw the attention of physicians to the promising efficacy of retinoic acid.
鱼鳞病是一种以角化过度为特征的临床皮肤角化障碍疾病。板层状鱼鳞病是鱼鳞病的一种罕见形式（胶样婴儿），为常染色体隐性遗传。诊断性临床发现可通过皮肤活检和基因分析得以证实。治疗原则是皮肤保湿、预防脱水以及使用角质溶解剂。据报道，系统性维甲酸（0.5 - 1毫克/千克/天）在治疗板层状鱼鳞病和先天性鱼鳞病样红皮病方面具有显著疗效。在本研究中，呈现了口服维甲酸治疗的效果，显示5例被诊断为胶样婴儿且出生后入住新生儿重症监护病房的患者有显著改善。所有患者均无其他先天性问题。仅2例患者被诊断为败血症。脱发是所有5例患者治疗的唯一不良反应。通过这一系列胶样婴儿病例，我们希望引起医生对维甲酸显著疗效的关注。
