Incontinentia pigmenti: the development of pseudoglioma.
نویسنده
چکیده
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.
منابع مشابه
Incontinentia Pigmenti
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 IP...
متن کاملA Case Report of Incontinentia Pigmenti in a Newborn with Positive Family History Extending Over Three Generations
Background: Incontinentia pigmenti (IP), also known as Bloch-Sulzberger syndrome, is a rare X-linked dominant genodermatosis that presents at the time of birth or soon after birth with cutaneous manifestation. This disorder may also affect the ectodermal tissues, such as the central nervous system, skeletal system, eyes, hair, nails, and teeth. The dermatological findings occur in four successi...
متن کاملگزارش یک مورد Incontinentia pigmenti
سابقه و هدف: (IP) Incontinentia pigmenti یا سندرم Bloch-Sulzberger اولین بار توسط Garrod در سال 1906 معرفی گردید. سپس، در سال 1926 توسط Bloch و در سال 1927 توسط Sulzberger گزارش شد. این بیماری یک بیماری ژنتیکی پوستی است که از نظر توارث وابسته به جنس غالب می باشد. ماکول های نامنظم، شیر قهوه ای یا خاکستری یا ضایعات آتروفیک، فرورفته و دپیگمانته از علایم پوستی این بیماری می باشند. هدف مقاله حاضر مع...
متن کاملA Case Study of Hypomelanosis of Ito
Among nevoid causes of hypopigmentations; incontinentia pigmenti achromians (Hypomelanosis of Ito) is a neurocutaneous syndromes characterized by distinctive macular, linear or irregular whorls or swirls of Hypopigmentations. Unrelated to incontinentia pigmenti, the loss of pigment begins spontaneously during infancy or early childhood and is of Particular importance because of the falt that m...
متن کاملFundus changes in incontinentia pigmenti ( Bloch - Sulzberger syndrome ) : a case report
The ocular and systemic changes in incontinentia pigmenti are reviewed here and a case is reported with fundus changes which have not been described previously. Incontinentia pigmenti (IP) is characterised by ectodermal defects, with occasional associated mesodermal abnormalities. The typical skin lesions are blue-grey to chocolate-brown arranged along the naevus lines of Blaschko. These occur ...
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ورودعنوان ژورنال:
- The British journal of ophthalmology
دوره 72 6 شماره
صفحات -
تاریخ انتشار 1988