5C64.20

ICD-11 MMS 2026-01

Acrodermatitis enteropathica

Description

Acrodermatitis enteropathica is an uncommon autosomal recessive disorder of intestinal zinc absorption. Signs usually appear within the first months of life with an exudative and crusted erythema located predominantly around body orifices (mouth, anogenital) and on the scalp and distal extremities. The signs are often misdiagnosed as being due to infection. The condition responds rapidly to zinc supplementation which must be continued throughout life.

Index terms

  • Acrodermatitis enteropathica
  • Brandt syndrome
  • Danbolt-Closs syndrome
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  • primary zinc malabsorption syndrome

Exclusions from above levels

  • 5B57 – Vitamin D deficiency
  • 5B5K – dietary mineral deficiency
  • http://id.who.int/icd/entity/962023213 – Disorders of the parathyroids or parathyroid hormone system
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  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 5A71.01 – Congenital adrenal hyperplasia
  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5C64.21 – Zinc deficiency syndromes
  • 5C64.2Y – Other specified disorders of zinc metabolism
  • 5C64.2Z – Disorders of zinc metabolism, unspecified

Foundation entity

Acrodermatitis enteropathica – http://id.who.int/icd/entity/1813939482

Part of: 5C64.2: Disorders of zinc metabolism › 5C64: Disorders of mineral absorption or transport › 05: Endocrine, nutritional or metabolic diseases