title: Report of a family with mal de Meleda in Taiwan: a clinical, histopathological and immunological study.
abstract: A family in Taiwan is reported to have Mal 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.
authors.forename: S H
authors.initials: SH
authors.lastname: Jee
authors.forename: Y Y
authors.initials: YY
authors.lastname: Lee
authors.forename: Y C
authors.initials: YC
authors.lastname: Wu
authors.forename: Y C
authors.initials: YC
authors.lastname: Lü
authors.forename: C C
authors.initials: CC
authors.lastname: Pan
mesh_terms: D000293:Adolescent; D000328:Adult; D005050:Etretinate / Q000627:therapeutic use; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology; D008213:Lymphocyte Activation; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D013601:T-Lymphocytes / Q000145:classification; D013624:Taiwan
chemical_list: D005050:Etretinate