Johnson Miguel, Mowatt Caleisia, Scott Kimberley
Cornwall Regional Hospital, Department of Plastic Reconstructive & Aesthetic Surgery, St. James, Jamaica.
University Hospital of the West Indies, Division of Plastic Surgery, Department of Surgery, Radiology, Anaesthesia and Intensive Care, University of the West Indies, Mona, Kingston, Jamaica.
J Surg Case Rep. 2023 Jan 4;2023(1):rjac552. doi: 10.1093/jscr/rjac552. eCollection 2023 Jan.
Congenital Ichthyosis represents a very rare group of congenital skin disorders characterized by some degree of scaling and thickening of the stratum corneum with skin inflammation. Severe forms such as Harlequin and Lamellar forms may present with limb and organ compromise, respectively. Patients with Lamellar subtype presents with severe ectropion which may lead to corneal ulceration, rupture and blindness. Treatment includes a multidisciplinary approach allowing for early intervention and treatment, which optimize the outcomes. The timing for intervention is not clearly defined in the literature. This is most likely due to a paucity in data due to the relative infrequency in these conditions. However, using principles similar to early treatment of ectropion in burnt patients may be employed. A case of Lamellar Ichthyosis that presented to our outpatient clinic which was successfully managed with early ectropion release and full thickness skin graft is discussed.
先天性鱼鳞病是一类非常罕见的先天性皮肤疾病，其特征是角质层有一定程度的脱屑和增厚，并伴有皮肤炎症。严重类型如丑角样鱼鳞病和板层状鱼鳞病可能分别出现肢体和器官损害。板层状亚型的患者会出现严重的睑外翻，这可能导致角膜溃疡、破裂和失明。治疗包括多学科方法，以便早期干预和治疗，从而优化治疗效果。文献中并未明确界定干预时机。这很可能是由于这些病症相对罕见，数据匮乏所致。然而，可以采用与烧伤患者睑外翻早期治疗类似的原则。本文讨论了一例在我们门诊就诊的板层状鱼鳞病病例，该病例通过早期睑外翻松解和全厚皮片移植成功得到治疗。
