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Hypotrichosis-lymphedema-telangiectasia-renal defect syndrome

ORPHA:69735· ICD-10 Q82.0

Definition

An extremely rare syndromic lymphedema disorder characterized by early-onset hypotrichosis, childhood-onset lymphedema, and variable telangiectasia, particularly of the palms.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant, Autosomal recessive
Age of onset
Infancy, Neonatal