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Acrodermatitis Enteropathica

Abstract

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NORD is very grateful to John McGrath, MD, St John's Institute of Dermatology, Guy's Hospital, London, for assistance in the preparation of this report.

Synonyms of Acrodermatitis Enteropathica

  • AE
  • Brandt syndrome
  • Danbolt-Cross syndrome
  • zinc deficiency, congenital

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Acrodermatitis enteropathica (AE) is a disorder of zinc metabolism that occurs in one of two forms: an inborn (congenital) form and an acquired form. The inborn form of AE is a rare genetic disorder characterized by intestinal abnormalities that lead to the inability to absorb zinc from the intestine. The lack of zinc presents, characteristically, as: (1) skin inflammation with pimples (pustular dermatitis) occurring around the mouth and/or anus, (2) diarrhea, and (3) abnormal nails (nail dystrophy). In the acute phase, irritability and emotional disturbances are evident due to wasting (atrophy) of the brain cortex. It is important to recognize and treat this disorder.

The acquired form of this disorder generates similar symptoms. Acquired AE sometimes results from special intravenous nutritional programs that are prepared without the appropriate amount of zinc.

Supplemental zinc usually eliminates the symptoms.

Organizations related to Acrodermatitis Enteropathica

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