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.
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