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.

Incontinentia pigmenti (Bloch-Sulzberger syndrome) is a genetic disease of the skin with generalised ectodermal and mesodermal dysplasia which may often involve the eyes (35% of the patients),' hair, teeth, and central nervous system. Skin lesions are Correspondence to Dr A Spallone. usually present at birth or shortly after in the form of erythematous eruptions with linear vesiculations. The f...

Incontinentia pigmenti (IP) is a rare disease with multisystemic anomalies, which commonly presents just after birth. Here, we report a rare case of IP patient with vitreous hemorrhage in school-age children. Therefore, physicians have to be alert and evaluate IP patients at all ages. Regular ophthalmic follow-up is necessary, and fluorescein angiography should be performed if peripheral ischem...

Incontinentia pigmenti (IP) is a rare X-linked dominant disease that affects the ectodermal tissues. It is associated with a whorled pattern of scarring alopecia, which is possibly underreported. This scarring alopecia could be used as a marker to identify adult women affected with IP as older patients may have minimal cutaneous manifestations. We report an unusual case of an 8-year-old girl wh...

Purpose To describe a patient with incontinentia pigmenti (IP) and Raynaud's phenomenon (RP). Observations A 5 year-old girl with history of IP was noted to have RP. Visual acuity was unaffected in both eyes, and fundus examination demonstrated regressed peripheral neovascularization. Photos of the patient's hands demonstrated pale discoloration associated with exposure to cold. Conclusions...

tological findings are different from our results. We had no evidence for other inflammatory skin diseases following Blaschko’s lines such as linear discoid lupus erythematosus or lichen striatus. Linear pigmentary disorders such as pigmentary mosaicism (naevoid hypermelanosis) or the pigmentary stage of incontinentia pigmenti were also excluded. The aetio-pathogeny of LAM remains unknown, but ...

Incontinentia pigmenti (IP) is an X-linked dominant disorder affecting the skin, teeth, eyes, and central nervous system. Ocular changes are common and may lead to severe vision loss. We report on the ocular manifestations in two young girls with IP, with emphasis on the asymmetry of this condition in both eyes and associated retinal problems. The outcomes of laser treatment of the ischemic per...

In 1993 diagnostic criteria for incontinentia pigmenti (IP), a genodermatosis in which skin changes are usually combined with anomalies of other organs, were established. Approximately a decade ago, IKBKG gene mutation was discovered as a cause for IP. This finding has not been included in IP diagnosis so far. In addition, literature data pointed out a few other clinical findings as possible IP...