Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition Ichthyosis-alopecia-eclabion-ectropion-intellectual disability syndrome is an ectodermal dysplasia syndrome characterized by severe generalized lamellar icthyosis at birth with alopecia, eclabium, ectropion and intellectual disability. Although similar to Sjögren-Larsson syndrome, this syndrome lacks the presence of neurologic or macular changes. There have been no further descriptions in the literature since 1987. Disease data Classification Disease Synonyms Jagell-Holmgren-Hofer syndrome Zespół Jagella, Holmgrena i Hofera ORPHA code 2269 OMIM code 242510 ICD10 code Q82.4 ICD11 code LD27.0Y *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl