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.