Rare Zebra

Rare disease search prototype built on Orphanet data

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ORPHA:99812

LIG4 syndrome

Also called DNA ligase IV syndrome, SCID with macrocephaly due to DNA ligase IV deficiency

Body system
Immunological diseases
Inheritance pattern
Autosomal recessive
Typical age of onset
Infancy, Neonatal
Estimated prevalence
<1 / 1 000 000 (Worldwide)
Rarity class
<1 / 1 000 000

ORPHA:99812 is classified under "Immunological diseases" in the Orphanet nomenclature.

Common signs

  • Microcephaly
  • Bird-like facies
  • Global developmental delay
  • Growth delay
  • Immunodeficiency
  • Abnormality of chromosome stability