Fundus changes in incontinentia pigmenti (Bloch-Sulzberger syndrome): a case report.

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Fundus changes in incontinentia pigmenti (Bloch-Sulzberger syndrome).

A case of incontinentia pigmenti is reported with fundus changes in 1 eye. She had microaneurysms temporal to the macula, with an abnormal branch of inferior temporal vein. There was extensive retinitis proliferans in the upper temporal equatorial region, which showed leakage on fluorescein angiography.

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Fundus 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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[Incontinentia pigmenti (Bloch-Sulzberger syndrome)].

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...

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Bloch-sulzberger Syndrome (incontinentia Pigmenti): a Case Report

Incontinentia pigmenti (IP) is a X-linked dominant neuro cutaneous syndrome with cutaneous, neurologic, ophthalmologic and dental manifestations mainly in female neonates. Starting from neonatal period, IP passes through stages of vesicular (90%), verrucous (70%), hyper pigmented (98%) and hypopigmented lesions. Authors report a case of IP in 8 months old female child who presented with vesicul...

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Ocular changes in the Bloch-Sulzberger syndrome (Incontinentia pigmenti)

THE Bloch-Sulzberger syndrome is a familial condition consisting chiefly of ectodermal defects, of which changes in the skin, nails, hair, teeth, central nervous system, and eyes are the most common. The final dermal phase "incontinentia pigmenti" has received the most attention. This disease can be described as a rare and peculiar abnormality of development found almost exclusively in females ...

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ژورنال

عنوان ژورنال: British Journal of Ophthalmology

سال: 1978

ISSN: 0007-1161

DOI: 10.1136/bjo.62.9.622