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Dermatitis and Nutritional Deficiency: Another Acrodermatitis Enteropathica–like Eruption

Dermatitis and Nutritional Deficiency: Another Acrodermatitis Enteropathica–like Eruption Abstract In this issue of AJDC, Giacoia and Berry1 report the occurrence of an acrodermatitis enteropathica (AE)–like eruption in an infant with maple syrup urine disease (MSUD) while being fed an isoleucine-deficient formula. At least two important points emerge from this observation. The first point is the importance of providing necessary amounts of essential amino acids when infants with metabolic disorders are fed intravenously or fed special formulas. Total restriction of phenylalanine is not possible in phenylketonuria, and total restriction of branched-chain amino acids (BCAAs) is deleterious in MSUD as suggested by Giacoia and Berry. Recently, a more extensive cutaneous eruption was reported in an infant with MSUD in whom inadequate amino acid intake was associated with severely depressed levels of all three BCAAs (valine, leucine, and isoleucine).2 The authors of the second report contend that clinical differences (total body exfoliative dermatitis without mucous References 1. Giacoia GP, Berry GT. Acrodermatitis enteropathica–like syndrome secondary to isoleucine deficiency during treatment of maple syrup urine disease . AJDC . 1993;147:954-956. 2. Northrup H, Sigman ES, Hebert AA. Exfoliative erythroderma resulting from inadequate intake of branched-chain amino acids in infants with maple syrup urine disease . Arch Dermatol . 1993;129:384-385.Crossref 3. DiLiberti JH, Di George AM, Auerbach VH. Abnormal leucine/isoleucine ratio and acrodermatitis enteropathica–like rash in maple syrup urine disease (MSUD) . Pediatr Res . 1973;7:382. 4. Hansen RC, Lemen R, Revsin B. Cystic fibrosis manifesting with acrodermatitis-like eruption . Arch Dermatol . 1983;119:51-55.Crossref 5. Darinstadt GL, Schmidt CP, Wechsler DS, Tunnessen WW, Rosenstein BJ. Dermatitis as a presenting sign of cystic fibrosis . Arch Dermatol . 1992;128:1358-1364.Crossref 6. DeRaeve L, DeMeirleirL, Blecker U, et al. Acrodermatitis enteropathica–like lesions in organic aciduria . Pediatr Dermatol . 1992;9:191. Abstract. 7. Shepherd ME, Raimer SS, Tyring SK, Smith EB. Treatment of necrolytic migratory erythema in glucagonoma syndrome . J Am Acad Dermatol . 1991;25:925-928.Crossref 8. Glover MT, Atherton DJ, Levinsky RJ. Syndrome of erythroderma failure to thrive and diarrhea in infancy: a manifestation of immunodeficiency . Pediatrics . 1988;81:66-70. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Dermatitis and Nutritional Deficiency: Another Acrodermatitis Enteropathica–like Eruption

Dermatitis and Nutritional Deficiency: Another Acrodermatitis Enteropathica–like Eruption

Abstract

Abstract In this issue of AJDC, Giacoia and Berry1 report the occurrence of an acrodermatitis enteropathica (AE)–like eruption in an infant with maple syrup urine disease (MSUD) while being fed an isoleucine-deficient formula. At least two important points emerge from this observation. The first point is the importance of providing necessary amounts of essential amino acids when infants with metabolic disorders are fed intravenously or fed special formulas. Total restriction of...
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Publisher
American Medical Association
Copyright
Copyright © 1993 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1993.02160330030011
Publisher site
See Article on Publisher Site

Abstract

Abstract In this issue of AJDC, Giacoia and Berry1 report the occurrence of an acrodermatitis enteropathica (AE)–like eruption in an infant with maple syrup urine disease (MSUD) while being fed an isoleucine-deficient formula. At least two important points emerge from this observation. The first point is the importance of providing necessary amounts of essential amino acids when infants with metabolic disorders are fed intravenously or fed special formulas. Total restriction of phenylalanine is not possible in phenylketonuria, and total restriction of branched-chain amino acids (BCAAs) is deleterious in MSUD as suggested by Giacoia and Berry. Recently, a more extensive cutaneous eruption was reported in an infant with MSUD in whom inadequate amino acid intake was associated with severely depressed levels of all three BCAAs (valine, leucine, and isoleucine).2 The authors of the second report contend that clinical differences (total body exfoliative dermatitis without mucous References 1. Giacoia GP, Berry GT. Acrodermatitis enteropathica–like syndrome secondary to isoleucine deficiency during treatment of maple syrup urine disease . AJDC . 1993;147:954-956. 2. Northrup H, Sigman ES, Hebert AA. Exfoliative erythroderma resulting from inadequate intake of branched-chain amino acids in infants with maple syrup urine disease . Arch Dermatol . 1993;129:384-385.Crossref 3. DiLiberti JH, Di George AM, Auerbach VH. Abnormal leucine/isoleucine ratio and acrodermatitis enteropathica–like rash in maple syrup urine disease (MSUD) . Pediatr Res . 1973;7:382. 4. Hansen RC, Lemen R, Revsin B. Cystic fibrosis manifesting with acrodermatitis-like eruption . Arch Dermatol . 1983;119:51-55.Crossref 5. Darinstadt GL, Schmidt CP, Wechsler DS, Tunnessen WW, Rosenstein BJ. Dermatitis as a presenting sign of cystic fibrosis . Arch Dermatol . 1992;128:1358-1364.Crossref 6. DeRaeve L, DeMeirleirL, Blecker U, et al. Acrodermatitis enteropathica–like lesions in organic aciduria . Pediatr Dermatol . 1992;9:191. Abstract. 7. Shepherd ME, Raimer SS, Tyring SK, Smith EB. Treatment of necrolytic migratory erythema in glucagonoma syndrome . J Am Acad Dermatol . 1991;25:925-928.Crossref 8. Glover MT, Atherton DJ, Levinsky RJ. Syndrome of erythroderma failure to thrive and diarrhea in infancy: a manifestation of immunodeficiency . Pediatrics . 1988;81:66-70.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Sep 1, 1993

References