Abstract
Introduction: Ichthyosis is a heterogeneous group of skin disease characterized by generalized scaling. Lamellar ichthyosis is an autosomal recessive disorder with a mutation in the TGM 1 gene encodes the transglutaminase I protein was found. The scales reflect changes in epidermal differentiation. Lamellar ichthyosis is the one of the rarest form of ichtyosis where the infant is usually born with a collodion membrane.
Case: A 10-year-old girl was consulted by the ophthalmologic department with a chief complaint of generalized scales on the body since birth. Both patients also complained of sore eyes because they can not close tightly. According to the parent, at the time of birth the patient was enveloped by membranes where the eyes could not close tightly. The patient was also unable to breastfeed. Physical examination showed ichtyiosiform scales arranged lamellar in a mosaic pattern throughout the body, Eclabium on the lips and ectropion on the eyes were found. Histopathological examination results support the diagnosis of lamellar ichthyosis. The patient was given emollient and supportive therapy. A good improvement shown by markedly diminished scales and skin stiffness was seen after six weeks of treatment.
Conclusion: Lamellar ichthyosis is a rare type of ichtyosis. Treatment aims to prevent excessive transepidermal water loss, prevent complications such as secondary infections and to reduce clinical symptoms.

Key words: ectropion, emollient, lamellar ichthyosis