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[Acrodermatitis enteropathica-like syndrome secondary to branched-chain amino acid deficiency during treatment of maple syrup urine disease].

Authors
  • Templier, I
  • Reymond, J-L
  • Nguyen, M-A
  • Boujet, C
  • Lantuejoul, S
  • Beani, J-C
  • Leccia, M-T
Type
Published Article
Journal
Annales de Dermatologie et de Vénéréologie
Publisher
Elsevier
Publication Date
Apr 01, 2006
Volume
133
Issue
4
Pages
375–379
Identifiers
PMID: 16733455
Source
Medline
License
Unknown

Abstract

Several cases of acrodermatitis enteropathica-like eruptions resulting from therapeutic protein restriction diets have been described in infants with different aminoacidopathies. The accompanying dermatosis was associated with a raised plasma leucine/isoleucine ratio and/or isoleucine deficiency, or valine deficiency. While the exact pathogenesis of the skin lesions has not been established, these observations show that branched-chain amino acids are essential for normal growth and differentiation of keratinocytes. The essential role of isoleucine is further substantiated by the fact that its presence is critical in keratinocyte culture media, with growth arrest occurring upon its depletion.

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