Senter Syndrome

**Semantic type:** Disease or Syndrome

**Definition:** A cutaneous condition associated with glycogen storage dysfunction that is characterized by vascular keratitis, congenital ichthyosis, neurosensorial deafness, hepatomegaly, hepatic cirrhosis, growth failure and intellectual disability.

**Synonyms:** - Keratitis-Ichthyosis-Deafness Syndrome - Senter syndrome