ICD-11 classes
14 Diseases of the skin
Skin disorders involving specific cutaneous structures
Disorders of the epidermis and epidermal appendages
Disorders of hair
EC21 — Genetic defects of hair or hair growth
EC21.3 — Genetic syndromes with alopecia or hypotrichosis

ICD-11 EC21.3 — Genetic syndromes with alopecia or hypotrichosis

Hereditary syndromes in which sparse or absent hair is a component

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code EC21.3 contains 2 clarifying diagnoses:

  1. 5C50.A0 — Argininosuccinic aciduria
  2. LD27.03 — Hidrotic ectodermal dysplasia, Clouston type

The diagnosis is coded elsewhere:

The diagnosis is included in 15 other classes.

  • Argininosuccinic aciduria (5C50.A0)
  • Hidrotic ectodermal dysplasia, Clouston type (LD27.03)
  • Severe T-cell immunodeficiency - congenital alopecia - nail dystrophy (4A01.1Y)
  • Ichthyosis – hypotrichosis syndrome (LD27.2)
  • Neonatal sclerosing cholangitis – ichthyosis – hypotrichosis syndrome (DB96.2Y)
  • Odonto-onycho-dermal dysplasia (LD27.0Y)
  • Woolly hair – hypotrichosis – everted lower lip – outstanding ears (LD27.0Y)
  • Autosomal dominant palmoplantar keratoderma and congenital alopecia (LD27.0Y)
  • Autosomal recessive palmoplantar keratoderma and congenital alopecia (LD27.0Y)
  • Alopecia - contractures - dwarfism - intellectual deficit (LD27.0Y)
  • Alopecia – psychomotor epilepsy – periodontal pyorrhoea – intellectual disability syndrome (LD90.Y)
  • Cataract - alopecia - sclerodactyly (LD27.0Y)
  • Odonto-onycho dysplasia - alopecia (LD27.0Y)
  • Schöpf-Schulz-Passarge syndrome (LD27.0Y)
  • Macrocephaly – alopecia – cutis laxa – scoliosis syndrome (LD28.2)

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