ichthyosis prematurity syndrome
Ichthyosis prematurity syndrome is 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.
MONDO:0002051
integumentary system disorder
is_a
MONDO:0002254
syndromic disease
is_a
MONDO:0003847
hereditary disease
is_a
MONDO:0043905
pneumonitis
is_a
IPS
exact
congenital ichthyosis type 4
exact
ichthyosis congenita IV
related
ichthyosis prematurity syndrome
exact
idiopathic pneumonia syndrome
exact