Rare Zebra

Rare disease search prototype built on Orphanet data

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ORPHA:83617

Agammaglobulinemia-microcephaly-craniosynostosis-severe dermatitis syndrome

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

ORPHA:83617 is classified under "Skin diseases" in the Orphanet nomenclature.

Common signs

  • Decreased total B cell count
  • Cleft palate
  • Microcephaly
  • Micrognathia
  • Low-set ears
  • Underdeveloped nasal alae