Two eyes of two siblings affected with incontinentia pigmenti having severe proliferative retinopathy are reported. Both cases showed complete resolution of new vessels after a single injection of intravitreal bevacizumab. At 7 months of follow-up, the first case revealed vascularization to the mid-periphery including a vascularized fovea (which was avascular before injection) but recurrence of...

Incontinentia pigmenti is rare X-linked dominant disorder. There is no consistent expression of Incontinetia pigmenti in female child, but in male child, they always lead to death in utero. Vesicular, verrucous, hyperpigmented, and atrophic stages are the four stages of Incontinetia Pigmenti and it is uncommon for all stages to be seen in a same case. It is a rare genodermatosis, with only very...

We report the case of an 8-days-old patient with linear rows of vesiculobullous lesions from birth. Along with these lesions, the neonate had eosinophilia, which made us arrive to the clinical diagnosis of Incontinentia Pigmenti. Incontinentia Pigmenti or Bloch Sulzberger disease is a multisystemic neuroectodermal disorder that affects mostly women, which includes as initial manifestation cutan...

Incontinentia pigmenti (IP, Bloch-Sulzberger syndrome) is a very rare genodermatosis characterized by typical skin lesions accompanied by dental, central nervous system, bone and ocular abnormalities. Incontinentia pigmenti is usually observed among women, as this X-linked dominantly inherited disorder is lethal in males. The hallmark feature of IP is cutaneous eruption along the lines of Blasc...

Sulzberger syndrome (incbntinentia pigmenti.) pigmenti associated with changes in the posterior Brit. J. Ophth. 39:276, 1955. chamber of the eye. Am. J. Ophth. 47 :321, 1959. 7. Kriimmel, H., and Rausch, L.: Anomalien 9. Reese, A. B.: Persistent hyperplastic primades Auges bei der sogenannten Incontinentia pigry vitreous. Am. J. Ophth. 40:317, 1955. menti. Ophthalmologica, 130:31, 1955. 10. Jon...

Incontinentia pigmenti is a rare genodermatosis in which the skin involvement occurs in all patients. Additionally, other ectodermal tissues may be affected such as the central nervous system, eyes, hair, nails and teeth. The disease has an X-linked dominant inheritance pattern. But in our case, there was a mutation in the body cells due to incontinentia pigmenti. The dermatological findings oc...

Incontinentia pigmenti (IP) is a complex genodermatosis inherited in an X-linked dominant pattern, associating multistadial cutaneous manifestations with an oculo-dento-cerebral syndrome, which affects only female newborns, as the disorder is lethal in males since intrauterine period. We report on a case of incontinentia pigmenti with an atypical debut consisting of tonic-clonic seizures which ...

Incontinentia pigmenti is a rare genodermatosis in which the skin involvement occurs in all patients. Additionally, other ectodermal tissues may be affected, such as the central nervous system, eyes, hair, nails and teeth. The disease has a X-linked dominant inheritance pattern and is usually lethal to male fetuses. The dermatological findings occur in four successive phases, following the line...

INTRODUCTION Incontinentia pigmenti is a rare X-linked dominant disease which affects the ectodermal tissues, usually lethal in males. MATERIALS AND METHODS A retrospective analysis of clinical data obtained from the photographic documentation and casenotes of patients diagnosed to have incontinentia pigmenti at the National Skin Centre. The study covered the period from January 1990 to Decem...