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Focal dermal hypoplasia

ORPHA:2092· ICD-10 Q82.8

Definition

A rare multiple congenital anomalies/dysmorphic syndrome characterized by abnormalities in ectodermal- and mesodermal-derived tissues, classically manifesting with skin abnormalities, limb defects, ocular malformations, and mild facial dysmorphism.

Prevalence
<1 / 1 000 000
Inheritance
X-linked dominant
Age of onset
Antenatal, Neonatal