A family in Taiwan is reported to have Mai de Meleda with possibly autosomal recessive inheritance among 5 of its members. The glove- and sock-like erythrokeratosis and the hyperkeratotic plaques of the knees and elbows are the common features. Erythema of the nose, cheeks, and the perioral area in 4 affected members, actinic elastosis in 2 severely affected members, and partially impaired cell-mediated immunity in vitro in both affected and unaffected members are notable findings. Tigason (etretinate) treatment was beneficial in regard to hyperkeratinization, but not to erythema.
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