Lamellar ichthyosis : A case report 12 years follow-up
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Abstract
Lamellar ichthyosis is an autosomal recessive disorder characterized by onset of ichthyosis at birth, involving the entire body surface. The newborn was born encased in a translucent colloidian membrane, which is taut and impair respiration and sucking. During second weeks of life, the membrane broke up and peeled off leaving fissures, with impairment of the barrier to infection and water loss lead to difficulties in thermal regulation, an increased risk of infection. The colloidian baby survived because of fully supportive care in neonatal intensive care unit.
Now he is over twelve years old with generalized scale that is accentuated in flexural areas as well as on the forehead and lower extremities. Eclabium subsides but ectropion remains and scarring alopecia at the periphery of the scalp, small and deformed ear are observed as a sequelae of excessively taut skin, and heat intolerance occurs due to obstruction of sweat duct by plates of scale. The treatment with a low dose oral retinoid (Acitretin) results in an improvement which is manifested by the decrease in the scale and thickness of lesions and heat intolerance. Side effect were not observed.
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References
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