Synopsis-Abstract Acrodermatitis enteropathica is a rare familial disease of childhood, manifested by a peculiar skin eruption localized to the acral areas, and by alopecia, diarrhea, stomatitis, glossitis, paronychias, nail dystrophy, emotional disturbances, and frequent secondary infection with monilial or bacterial organisms. Fifty-eight case reports have now appeared in the world literature, and these have been reviewed. Six cases of this disorder are presented. All had been labeled previously with other diagnoses, as had many cases reported in the literature. Skin biopsies on 3 patients, necropsies on 3 patients, and laboratory studies on all of the patients failed to reveal consistent or significant abnormalities, and the disease remains among those of unknown etiology. It is suggested that some obscure absorptive defect may be responsible for the varying manifestations of acrodermatitis enteropathica. Acrodermatitis enteropathica is a rare familial disease of early childhood, first described by Danbolt and Closs in 1942.1 Their