Ichthyosis-prematurity syndrome

Orpha code: 88621OMIM code: 608649

Definition

A rare, syndromic congenital ichthyosis characterized by premature birth (at gestational weeks 30-32, in general) in addition to thick, caseous and desquamating epidermis, neonatal respiratory asphyxia, and persistent eosinophilia. After the perinatal period, a spontaneous improvement in the health of affected patients is observed and skin features (vernix caseosa-like scale) evolve into a mild presentation of flat follicular hyperkeratosis with atopy.

Disease data
Classification

Disease

Synonyms
Congenital ichthyosis type 4
IPS
Wrodzona rybia łuska typu 4
IPS
ORPHA code
88621
OMIM code
608649
ICD10 code
-
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl