DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20150646

Bilateral ectropion in a 3 months old baby with lamellar ichthyosis: a rare case report

Sweta S. Kumar, Shailly Raj, Ifsa Sami, Sonali Singh

Abstract


Ichthyosiform dermatoses are a group of hereditary disorders characterized by dryness and roughness of the skin with excessive accumulation of epidermal scales. Four main types of Ichthyosis are Ichthyosis vulgaris, x-linked recessive, lamellar ichthyosis and epidermolytic hyperkeratosis. Lamellar ichthyosis is the rarest form with an incidence of 1 in 3, 00,000. It has autosomal recessive inheritance. Ocular manifestations include exposure keratitis secondary to ectropion, unilateral megalocornea, and enlarged corneal nerve, and blepharitis, absence of the meibomian gland, trichiasis, madarosis and absence of lacrimal puncta. Ectropion of both upper and lower lids have been documented. We are reporting a rare case of lamellar ichthyosis with bilateral upper eyelid ectropion in a child.


Keywords


Lamellar ichthyosis, Ectropion, Collodion membrane

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References


Duke Elder S. System of Ophthalmology Vol. XIII, Henry Kimpton, London, p.p.257-259,1968.

Ahmad IL, Rizvi R, Ahmad SS, Ahmad RU. Bilateral Severe Ectropion and Mature Cataract in Lamellar ichthyosis. J North Zone Ophthalmolog Soc. 2004;14:34-6.

Lentz CL, Attman J Arch Derm. 1968;97:3-13.

Bloom D, Goodfried MS. Arch Derm. 1962;86:336-42.

Kenneth MG, Seth AA. Ichthyosis: Overview; e Medicine Ophthalmology. Available from: http://www.emedicine,Medscape.com/article. Last updated 2007 Dec 12.