ichthyosis prematurity syndrome

ichthyosis prematurity syndrome

A rare form of autosomal recessive congenital ichthyosis, most commonly seen in Scandinavia.

Clinical findings
Complicated mid-trimester of pregnancy and a premature birth of a child with thick caseous desquamating epidermis, respiratory complications and eosinophilia, who recovers with a lifelong non-scaly ichthyosis with atopic manifestations.

Molecular pathology
The gene responsible is located in chromosome 9q33.3–34.13.
References in periodicals archive ?
The exuberant vernix/cephalic hyperkeratosis phenotype generally appears in children with keratitis-ichthyosis-deafness syndrome (KID) and ichthyosis prematurity syndrome (IPS).
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