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Syndromic recessive X-linked ichthyosis

ORPHA:281090· ICD-10 Q80.1

Definition

A rare genetic skin disease belonging to the Mendelian Disorders of Cornification (MeDOC) characterized by a generally mild cutaneous desquamation in association with extracutaneous manifestations as part of a syndrome.

Prevalence
1-9 / 100 000
Inheritance
X-linked recessive
Age of onset
Childhood