| Senter syndrome | |
|---|---|
| Classification and external resources | |
| OMIM | 148210 |
| DiseasesDB | 32841 |
Senter syndrome (also known as "Desmons' syndrome")[1] is a cutaneous condition characterized by similar skin changes and congenital hearing impairment to keratitis–ichthyosis–deafness syndrome, but is associated with glycogen storage leading to hepatomegaly, hepatic cirrhosis, growth failure and mental retardation.[2]