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March 1963

Acrodermatitis Enteropathica: Case Report and Review of Literature

Author Affiliations

R. C. Tierney, M.D., Chief, Pediatrics, Loring Air Force Base, Maine.; CDR Andrew M. Margileth, MC, USN, Department of Pediatrics, U.S. Naval Hospital, Chelsea 50, Mass.; Chief, Department of Pediatrics, U.S. Naval Hospital.

Am J Dis Child. 1963;105(3):285-291. doi:10.1001/archpedi.1963.02080040287011

In 1942 Danbolt and Closs 1 were the first to describe 2 children with diarrhea, periorificial dermatitis, alopecia, and apathy. Since then 65 cases have been reported, 3 of which were in adults.2-4 Acrodermatitis enteropathica is a rare familial disorder with a recessive mode of transmission. Females are affected more often, and a history of familial occurrence is noted in 60% of the cases. Siblings but no parents have been affected, although consanguinity of the parents was noted in 5 cases. Because of the rarity of this disease our observations in a case and a review of the literature may be of interest to physicians caring for children with this problem.

Report of a Case  The patient, age 4 months, was admitted on Dec. 16, 1959, to Chelsea Naval Hospital because of recurrent dermatitis, diarrhea, and failure to thrive. The infant was well until 5 weeks of age when