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Hypohidrotic ectodermal dysplasia-immunodeficiency-osteopetrosis-lymphedema syndrome

ORPHA:69088· ICD-10 Q78.2

Definition

This syndrome is characterized by severe immunodeficiency, osteopetrosis, lymphedema and anhidrotic ectodermal dysplasia.

Prevalence
<1 / 1 000 000
Inheritance
X-linked recessive
Age of onset
Infancy, Neonatal