ABSTRACT
INTRODUCTION: Lamellar ichthyosis is a rare autosomal recessive disorder apparent at birth. Although not life threatening, it is disfiguring and causes considerable psychological stress. CASE: We report a case of 15-year-old male with generalized, thick, dry, plate-like scales associated with ectropion and eclabium. At birth, he was enclosed in a transparent membrane that spontaneously shed revealing generalized pinkish skin. Non-pruritic plaques developed that evolved into plate-like scales. There was accompanying heat intolerance and tearing of eyes. Two siblings were similarly affected. Histopathologic findings were consistent with lamellar ichthyosis. The patient was treated with retinoic acid lotion and petroleum jelly. After three weeks, 50 percent improvement was noted with decrease in number and thickness of lesions. CONCLUSION: This lifetime cutaneous disease affects the physical, mental and emotional state ofthe patient. It has no cure and treatment is directed symptomatically. We plan to continue topical treatment in this patient until significant improvement is attained.