Kün-Darbois J-D, Molin A, Jeanne-Pasquier C, Paré A, Bénateau H, Veyssière A
Service de chirurgie maxillo-faciale, hôpital Côte de Nacre, CHU de Caen, 14033 Caen cedex, France.
Service d'anatomopathologie, hôpital Côte de Nacre, CHU de Caen, 14033 Caen cedex, France.
Rev Stomatol Chir Maxillofac Chir Orale. 2016 Feb;117(1):51-3. doi: 10.1016/j.revsto.2015.11.007. Epub 2015 Dec 28.
We report 4 cases of Harlequin ichthyosis, which is a rare and severe congenital ichthyosis involving the face. Facial appearance consists in severe ectropion, conjonctival edema, eclabium, flattened ears, broadened nose and large, thick, plate-like skin scales. Recent advances in neonatal care have been made, such as retinoid therapy, and have led to an increased survival rate. Early surgical correction of ectropion may be required because of ocular complications.
我们报告了4例丑角鱼鳞病病例,这是一种罕见且严重的累及面部的先天性鱼鳞病。面部表现为严重的睑外翻、结膜水肿、唇外翻、耳朵扁平、鼻子变宽以及有大而厚的板状皮肤鳞屑。新生儿护理方面已有一些新进展,如维甲酸治疗,这使得存活率有所提高。由于眼部并发症,可能需要尽早对外翻进行手术矫正。