Acrodermatitis enteropathica in three siblings

Authors

  • Dr. Arfan-ul-Bari
  • Muhammad Abbas
  • Simeen ber Rahman

Abstract

Acrodermatitis enteropathica is an autosomal recessive disease due to a partial disorder of intestinal zinc uptake. Signs usually appear within the first months of life, with a characteristic cutaneous rash that is symmetrical, located around the body orifices, behind the ears, and on hands, feet, and head. We report three siblings with characteristic features of the disease and laboratory evidence of low serum zinc levels. Symptoms in all three patients reversed remarkably on supplementation of zinc. A brief history, clinical profile, pathophysiology and treatment of the disease have also been discussed in this article for better understanding of this disorder.

References

Danbolt N, Closs K. Arodermatitis enteropathica. Acta Derm Venereol 1942; 22: 17.

Portnoy B, Molokhia M. Acrodermatitis enteropathica treated by zinc. Br J Dermatol 1974; 91: 701-3

Wells BT, Winkelmann RD. Acrodermatitis enteropathica: report of six cases. Arch Dermatol 1961; 84: 40-52.

Stevenson JR, Fidone GS, Leland LS. Acrodermatitis enteropathica. Arch Dermatol 1964; 89: 224-8.

Moynahan EJ. Acrodermatitis enteropathica: a lethal inherited human zinc deficiency disorder (letter). Lancet 1974; 2(7877): 399-400.

Sivasubramanian KN, Henkin RI. Behavioral and dermatologic changes and low serum zinc and copper concentrations in two premature infants after parenteral alimentation. J Pediatr 1978; 93: 847-51.

Bilinski DL, Ehrenkranz RA, Cooley-Jacobs J. Symptomatic zinc deficiency in a breast-fed, premature infant. Arch Dermatol 1987; 123: 1221-4.

Kuramoto Y, Igarashi Y, Tagami H. Acquired zinc deficiency in breast-fed infants. Semin Dermatol 1991; 10: 309-12.

Piela Z, Szuber M, Mach B. Zinc deficiency in exclusively breast-fed infants. Cutis 1998; 61: 197-200.

Arakawa T, Tamura T, Igarashi Y. Zinc deficiency in two infants during total parenteral alimentation for diarrhea. Am J Clin Nutr 1976; 29: 197-204.

Garretts M, Molokhia M. Acrodermatitis enteropathica without hypozincemia. J Pediatr 1977; 91: 492-4.

Gordon EF, Gordon RC, Passal DB. Zinc metabolism: basic, clinical, and behavioral aspects. J Pediatr 1981; 99: 341-9.

Walravens PA, Hambidge KM, Neldner KH. Zinc metabolism in acrodermatitis enteropathica. J Pediatr 1978; 93: 71-3.

Leupold D, Poley JR, Meigel WN. Zinc therapy in acrodermatitis enteropathica. Helv Paediatr Acta 1976; 31: 109-15.

Bhutta ZA. The role of zinc in health and disease: relevance in child health in developing countries. J Pak Med Assoc 1997; 47: 68-73.

Dallaha CJ, Lorincz AL, Aarnik ON. Acrodermatitis enteropathica: review of the literature and report on a case successfully treated with diodoquin. JAMA 1953; 152: 509-12.

Grider A, Mouat MF. The acrodermatitis enteropathica mutation affects protein expression in human fibroblasts: analysis by two-dimensional gel electrophoresis. J Nutr 1998; 128: 1311-4.

Pugh EW, Griffen S. Homozygosity mapping places the acrodermatitis enteropathica gene on chromosomal region 8q24.3. Am J Hum Genet 2001; 68: 1055-60.

Lonnerdal B, Stanislowski AG, Hurley LS. Isolation of a low molecular weight zinc binding ligand from human milk. J Inorg Biochem 1980; 12: 71-8.

Prasad AS. Zinc: an overview. Nutrition 1995; 11 (Suppl 1): 93-9.

Bhutta ZA, Nizami SQ, Isani Z. Zinc supplementation in malnourished children with persistent diarrhea in Pakistan. Paediatrics 1999; 103: 42.

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Published

2017-01-03

How to Cite

1.
Arfan-ul-Bari D, Abbas M, Rahman S ber. Acrodermatitis enteropathica in three siblings. J Pak Assoc Dermatol [Internet]. 2017Jan.3 [cited 2026Feb.26];13(3):148-52. Available from: https://www.jpad.com.pk/index.php/jpad/article/view/751

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Case Reports

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