Abstract | INTRODUCTION: CASE-REPORT: DISCUSSION: 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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Authors | I Templier, J-L Reymond, M-A Nguyen, C Boujet, S Lantuejoul, J-C Beani, M-T Leccia |
Journal | Annales de dermatologie et de venereologie
(Ann Dermatol Venereol)
Vol. 133
Issue 4
Pg. 375-9
(Apr 2006)
ISSN: 0151-9638 [Print] France |
Vernacular Title | Pseudo-acrodermatite entéropathique secondaire à un déficit en acides aminés ramifiés au cours du traitement d'une leucinose. |
PMID | 16733455
(Publication Type: Case Reports, English Abstract, Journal Article)
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Chemical References |
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Topics |
- Acrodermatitis
(etiology)
- Female
- Humans
- Infant
- Isoleucine
(deficiency)
- Maple Syrup Urine Disease
(therapy)
- Syndrome
- Valine
(deficiency)
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