title: Acrodermatitis enteropathica-like cutaneous lesions in organic aciduria.
abstract: Cutaneous lesions resembling acrodermatitis enteropathica were present in two infants with methylmalonic acidemia and in one infant with propionic acidemia. All three infants were being fed a low-protein diet limited in branched-chain amino acids when the skin lesions developed. A deficiency in plasma levels of essential amino acids, particularly isoleucine, was confirmed. Supplementation of the diet with isoleucine in one of the patients led to a prompt improvement of the skin lesions. We conclude that dietary deficiencies associated with the treatment of organic aciduria should be added to the causes of acrodermatitis enteropathica-like cutaneous lesions.
authors.affiliation: Department of Dermatology, Academisch Ziekenhuis, Vrije Universiteit Brussel, Belgium.
authors.forename: L
authors.initials: L
authors.lastname: De Raeve
authors.forename: L
authors.initials: L
authors.lastname: De Meirleir
authors.forename: J
authors.initials: J
authors.lastname: Ramet
authors.forename: Y
authors.initials: Y
authors.lastname: Vandenplas
authors.forename: E
authors.initials: E
authors.lastname: Gerlo
mesh_terms: D000169:Acrodermatitis / Q000209:etiology*; D000592:Amino Acid Metabolism, Inborn Errors / Q000097:blood / Q000150:complications* / Q000178:diet therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007532:Isoleucine / Q000172:deficiency*; D007930:Leucine / Q000172:deficiency; D008297:Male; D008764:Methylmalonic Acid / Q000097:blood / Q000652:urine*; D008854:Microscopy, Electron; D011422:Propionates / Q000097:blood*; D012867:Skin / Q000473:pathology; D014633:Valine / Q000172:deficiency
chemical_list: D011422:Propionates; D007532:Isoleucine; D008764:Methylmalonic Acid; D007930:Leucine; D014633:Valine