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Article |

A Dermatitis Secondary to Amino-Acid Deficiency in Treated Maple Syrup Urine Disease

Susan E. Koch, MD; Thomas K. Koch, MD; Mary L. Williams, MD
Arch Pediatr Adolesc Med. 1994;148(9):993. doi:10.1001/archpedi.1994.02170090107025.
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It was with great interest that we read the article by Giacoia and Berry1 in the September 1993 issue of AJDC. While the pediatric literature eludes to the occurrence of a dermatitis in children receiving branched-chain amino acid–free formula,2 there are few detailed descriptions of this syndrome.3 We recently described multiple exacerbations of dermatitis in two children with classic maple syrup urine disease.4 While the majority of our observed episodes of dermatitis were associated with an isoleucine deficiency, one of our patients developed a well-documented exacerbation when the leucine concentration fell below normal limits and the isoleucine level became slightly elevated. The reported occurrence of a similar periorificial rash with selective arginine deficiency5 suggests that this may not be an isolated response to any one specific amino acid; rather, it may be seen with several different amino-acid deficiency states.

We believe that this clinical syndrome


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