Bloch-Sulzberger Syndrome (incontinentia pigmenti).
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چکیده
منابع مشابه
[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...
متن کامل[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 ...
متن کاملIncontinentia 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.
متن کاملFundus 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.
متن کاملOcular 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: Journal of Medical Genetics
سال: 1966
ISSN: 1468-6244
DOI: 10.1136/jmg.3.2.148