Department of Ophthalmology, JNMC, Belagavi, Karnataka, India.
Indian J Ophthalmol. 2018 Jun;66(6):856-858. doi: 10.4103/ijo.IJO_1171_17.
Congenital lamellar ichthyosis is an autosomal recessive, heterogeneous disorder presenting at birth with generalized skin involvement. The most common ophthalmic manifestation noted is bilateral ectropion of the lower eyelids. A 1-month-old female neonate, the second born of a nonconsanguineous marriage, presented with 4 days' history of redness, discharge, and swelling in the right eye. There was severe right upper eyelid ectropion, conjunctival injection, chemosis, a subconjunctival mass on the temporal bulbar conjunctiva spontaneously draining pus and corneal haze. The anterior chamber, iris, lens and fundus appeared normal. Congenital lamellar ichthyosis was suspected because of scaling and excessive dryness of the entire body. The occurrence of a spontaneous subconjunctival abscess is not known in lamellar ichthyosis. We thus report the management of a rare case of unilateral upper eyelid ectropion, subconjunctival abscess with orbital cellulitis in congenital lamellar ichthyosis.
先天性板层状鱼鳞病是一种常染色体隐性、异质性疾病，出生时即有全身皮肤受累。最常见的眼部表现是双侧下眼睑的外翻。一名 1 个月大的女婴，非近亲结婚所生的二胎，右眼出现 4 天红肿、分泌物和肿胀。右上眼睑严重外翻、结膜充血、水肿，球结膜颞侧有一个皮下积脓的肿块，自行破溃排脓，角膜混浊。前房、虹膜、晶状体和眼底均正常。由于全身鳞屑和极度干燥，怀疑为先天性板层状鱼鳞病。然而，在板层状鱼鳞病中，自发性结膜下脓肿的发生并不常见。因此，我们报告了一例先天性板层状鱼鳞病单侧上眼睑外翻、伴眶蜂窝织炎的罕见病例。
