incontinentia pigmenti (ip) is an uncommon x-linked dominant genodermatosis characterized by four cutaneous stages and frequent association with dental (90%), central nervous system (33%) and ocular (35%) anomalies. the exact pathogenesis of this disorder remains unknown. herein, we report a newborn girl with inflammatory vesiculobullous and warty skin lesions and a positive family history of i...

Incontinentia Pigmenti (IP) is a rare X-linked dominant disorder with skin, eye, central nervous system (CNS) and tooth abnormalities. According to the reported cases, it is estimated that there have been nearly 900-1200 affected individuals. In this article, the literature is reviewed and a case of IP with characteristic skin lesions and optic atrophy is presented.

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 repor...

A case is described of incontinentia pigmenti in an infant with relatively normal retinae at seven days after birth who went on to total blindness by three months. This was due to excessive neovascularisation of retinae and vitreous, leading to bilateral pseudoglioma.

Bloch-Sulzberger syndrome (incontinentia pigmenti) is a rare genodermatosis that affects predominantly females, since it is generally lethal to male fetuses in utero. It is characterized principally by skin lesions, but may also involve dental, ophthalmological and neurological abnormalities. The skin lesions are present in four different phases: vesicular, verrucous, hyperpigmented and atrophi...