Report of a family with mal de Meleda in Taiwan: a clinical, histopathological and immunological study.
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.
Jee
S H
SH
Lee
Y Y
YY
Wu
Y C
YC
Lü
Y C
YC
Pan
C C
CC
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
D005050:Etretinate