Author/Authors :
Basiri، behnaz نويسنده Assistant Professor of Neonatology, Hamedan University of Medical Sciences ,Hamedan, Iran , , Taghdiri ، Mohammad Mahdi نويسنده Associate Professor of Pediatric Neurology, Hamedan University of Medical Sciences,Hamedan, Iran ,
Abstract :
Objective
Incontinentia Pigmenti (IP) (Bloch_Sulzberg syndrome) is a rare neurocutaneous syndrome characterized by multisystemic involvement that is prenatally lethal in the majority of affected males and shows great clinical variability when expressed in women. The diagnosis of IP is performed based on clinical features and the family history with the support of histological findings.
We report a 10-day-old female neonate with typical skin lesions and frequent seizure. Skin biopsy showed second stage IP.
