Lamellar Ichtyosis: Case Report

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Introduction: Ichtyosiform dermatoses are a group of hereditary disorders characterized by excessive scaling on the skin. Lamellar ichtyosis is an autosomal recessive disorder. The clinical findings are seen at birth and become widespread and prominent in time; gray-brown scales are seen all over the body. Emollients and keratolytics containing salicylic acid or glycolic acid are used topically. Symptoms are improved significantly by acitretin usage. Case Report: Here we present a 5-year-old girl with lamellar ichtyosis. She was born in a collodion membrane. Besides the skin scales, ectropion and deformities of the nails and ears were observed. Significant clinical improvement was seen with acitretin (10 mg/day) therapy. Conclusion: Lamellar ichtyosis is a severe form of ichtyosiform dermatoses and topical agents may be insufficient. Systemic acitretin treatment improves the symptoms significantly but long term side effects limit its usage.

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