Lamellar Ichthyosis

Dry, pruritic, scaly skin was noted on a 65-year-old woman who came to the emergency department with bronchitis. She reported that her skin had had this texture all of her life. This is lamellar ichthyosis, an autosomal recessive disorder, writes Dr Sunita Puri of Decatur, Ala. The large, centrally attached, platelike, brownish yellow, rectangular scales with raised borders and superficial cracks resemble crocodile skin or a dry riverbed. Generally, the largest scales are on the legs. Involvement of the palms and soles may range from hyperlinearity to severe thickening of the skin; lips and mucous membranes usually are spared. Sweat gland function can be affected. The patient may have ectropion if scales are on the periocular area. Tight skin and thick scalp scales can lead to alopecia. There is no satisfactory treatment for this lifelong condition. Hydration, lubrication, and keratolytics may be beneficial, and long-term systemic retinoid therapy can be tried. Ichthyosis may improve in moist, humid climates.