Nailfold scleroderma-like capillary abnormalities in Werner syndrome (adult progeria)
نویسندگان
چکیده
منابع مشابه
Adult Progeria: Werner Syndrome.
Werner's syndrome is an adult premature aging syndrome of autosomal recessive inheritance affecting connective tissues throughout the body.1 The exact etiology remains obscure even though biochemical and connective tissue abnormalities have been postulated.2 The disease involves multiple systems of the body and may be associated with internal malignancy.3 We report a case of a 35 year old man w...
متن کاملExcess of rare cancers in Werner syndrome (adult progeria).
The association between genetic disorders and diverse cancers has provided clues for laboratory research into carcinogenesis. Such an opportunity now arises from studies of cancer in Werner syndrome (WRN). Soft-tissue sarcoma (STS) and benign meningioma have been associated with WRN, an autosomal recessive disorder characterized by premature aging, more commonly reported in Japan than elsewhere...
متن کاملDecreased nailfold capillary density in limited scleroderma with pulmonary hypertension.
Approximately 20% of patients with the limited form of scleroderma will develop pulmonary hypertension which is generally a late stage fatal complication. Why pulmonary hypertension occurs in this subset of patients is unknown and it has not been possible to predict which patients are at risk. Nailfold capillary dilatation, distortion and drop occurs universally in patients with scleroderma and...
متن کاملSequential nailfold capillary microscopy in scleroderma and related disorders.
Sequential nailfold capillary microscopy was carried out monthly for seven months in seven patients (four with scleroderma, one with dermatomyositis, one with mixed connective tissue disease, and one with limited connective tissue disease). Progressive enlargement of some capillary loops was observed, with a number becoming obliterated, leaving avascular areas. Extravasation from capillaries us...
متن کاملMicrovascular abnormalities in Sjögren's syndrome: nailfold capillaroscopy.
OBJECTIVE To describe microvascular abnormalities by nailfold capillaroscopy in patients with primary Sjögren's syndrome (SS) with or without Raynaud's phenomenon (RP) and those with anticentromere antibodies (ACA). METHODS Forty patients with SS (14 without RP, 16 with RP, 10 with ACA), 20 patients with scleroderma (SSc) (10 with limited and 10 with diffuse disease) (disease control group) a...
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ژورنال
عنوان ژورنال: Vascular Medicine
سال: 2017
ISSN: 1358-863X,1477-0377
DOI: 10.1177/1358863x17692667