MR imaging in pseudoxanthoma elasticum.

نویسندگان

  • M Hemmati
  • J Rosengarten
  • D B Hier
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].

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

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.

متن کامل

Two cases of pseudoxanthoma elasticum with renal involvement

  Abstract   Pseudoxanthoma elasticum is a rare, hereditary, multisystemic disease affecting   the skin, eye, and cardiovascular system. Renal involvement is uncommon.   We describe two cases of pseudoxanthoma elasticum (PXE) in two women with   distinctive skin lesions and nephrocalcinosis that renal ultrasonography showed a   characteristic pattern of dotted increased echogenicity in the cort...

متن کامل

Optical coherence tomography angiography findings of choroidal neovascularization in pseudoxanthoma elasticum

Angioid streaks (AS) are the most common ocular manifestation in patients with pseudoxanthoma elasticum (PXE). The major cause of severe visual loss in patients with AS is choroidal neovascularization (CNV). We report the optical coherence tomography angiography (OCTA) findings of CNV in a patient with PXE and angioid streaks. A 51-year-old man with PXE presented with visual disturbance in his ...

متن کامل

A Case of Pseudoxanthoma Elasticum with Microvascular Alterations: Possible Explanations and Causes

Pseudoxanthoma elasticum is a rare autosomal recessive disease, which is well-known for its affection of three major organ systems: skin, eye and cardiovascular systems, in particular large elastic arteries. This case is one of the first demonstrations of microvascular alterations in patients Pseudoxanthoma elasticum and offers hypotheses for their explanation.

متن کامل

Periumbilical perforating pseudoxanthoma elasticum.

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...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • AJNR. American journal of neuroradiology

دوره 10 5 Suppl  شماره 

صفحات  -

تاریخ انتشار 1989