Bloch-Sulzberger syndrome: a

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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: a case report

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...

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

The course and histologic skin signs are described at the stage of inflammation in 2 cases of incontinentia pigmenti (IP) or the Bloch-Sulzberger syndrome. In the authors' opinion, the pigmentary stage of skin lesion in IP does not replace the warty stage, but accompanies it or the inflammatory one as blebs disappear. In IP, the latter contain eosinophils; there is plasma and cell infiltration ...

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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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Incontinentia pigmenti (Bloch-Sulzberger syndrome) and retinal changes.

Incontinentia pigmenti is associated with various anomalies in 80% of cases. Among the most important are the ocular abnormalities and more particularly a retrolental mass with detachment of a dysplastic retina. At the basis of this manifestation are retinal vascular changes, characterised at first by ectatic tortuous veins and arteriovenous anastomoses as well as by aneurysmal-like dilatations.

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ژورنال

عنوان ژورنال: Advances in Dermatology and Allergology

سال: 2012

ISSN: 0860-6218

DOI: 10.5114/pdia.2012.31494