CREST Syndrome: Clinical Expression of the Disease

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Excerpt

A 54-year-old woman presented with a 2-year history of dyspepsia and intermittent dysphagia to solids and athralgias in hands and wrists. At physical examination, we found in the skin telangiectasia on the palate (Fig. A) and palms (Fig. B) and salt-and-pepper skin in ears, distal forearm, and lower limb (Fig. C) and sclerodactyly. The tests showed positive antinuclear antibody 1:1280 with centromeric pattern, antitopoisomerase antibody negative, and anticentromere antibody positive 1:10,240. According to the American College of Rheumatology classification criteria, patients with CREST syndrome must meet at least 3 of the following clinical features: calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.1
We show some cutaneous presentations of the CREST syndrome and the need to look for them intentionally. In the case of telangiectasias, whose etiology is not fully understood, it is known to occur more in limited sclerosis patients such as in this case. In early stages, they are located in the fingers, hands, face, and mucous membranes, and their presence has been considered as a clinical predictor of pulmonary vascular disease.
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