MR imaging in pseudoxanthoma elasticum.
نویسندگان
چکیده
Gri:inblad-Strandberg syndrome known as pseudoxanthoma elasticum (PXE) is a rare connective tissue disorder, the inheritance of which shows both autosomal and recessive characteristics [1]. The disease most commonly involves the skin , eyes , cardiovascular system, and gastrointestinal tract [2]. The vascular connective tissue supporting the retina, known as the Bruch membrane, tends to calcify [3] . Arterial calcifications may result in a variety of clinical manifestations including hypertension; angina; and neurologic deficits caused by ischemia , infarction, or hemorrhage [4 , 5].
منابع مشابه
Pseudoxanthoma Elasticum: A Report of Three Cases in a Family
Pseudoxanthoma Elasticum is an inherited disorder characterized by generalized fragmentation and progressive calcification of the elastic tissues in the dermis, blood vessels and Bruch’s membrane of the eye. Herein, we report 3 patients in a family with pseudoxanthoma elasticum: a father and his two sons.
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A 58-year-old, gravida 6, obese woman presented with a pruritic yellowish plaque around the umbilicus, which first appeared about 3 years ago. She also had flat yellow papules on the axilla and neck. After a burn from a heating device, a few perforating papules and a violaceous hue occurred on the plaque. Histological examination revealed pathological elastic fibers with a keratotic plug and pe...
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ورودعنوان ژورنال:
- AJNR. American journal of neuroradiology
دوره 10 5 Suppl شماره
صفحات -
تاریخ انتشار 1989