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Sclerodactyly is often preceded by months or even years by Raynaud's phenomenon when it is part of systemic scleroderma. [citation needed] The term "sclerodactyly" comes from Greek skleros 'hard' and daktylos 'digit'. It is generally associated with systemic scleroderma and mixed connective tissue disease, and auto-immune disorders.
CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder.The acronym "CREST" refers to the five main features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.
Most patients (over 80%) have vascular symptoms and Raynaud's phenomenon, which leads to attacks of discoloration of the hands and feet in response to cold. Raynaud's normally affects the fingers and toes. Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes, which are known as digital ulcers.
Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. [ 2 ] [ 6 ] [ 8 ] The disease can be either localized to the skin or involve other organs, as well. [ 2 ]
Common symptoms include photosensitivity and malar rashes, similar to those seen with SLE. [17] Discoid lesions are also occasionally seen. [10] Some patients with MCTD may have scleroderma-like symptoms such as squared telangiectasia on the hands and face, periungual telangiectasia, sclerodactyly, and calcinosis cutis. [18] [10]
Telangiectasias, aside from presenting in many other conditions, are one of the features of the acronymically named CREST syndrome, a form of systemic scleroderma. The syndrome recognises the significantly co-presenting symptoms of calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia.
Scleroderma - diverse collection of autoimmune fibrosing conditions. [21] Dermatomyositis and polymyositis - autoimmune myopathies that are clinically characterized by extramuscular symptoms, muscle inflammation, proximal muscle weakening, and oftentimes the detection of autoantibodies. [22] Vasculitis - disease that results in blood vessel ...
Atrophoderma of Pasini and Pierini (dyschromic and atrophic variation of scleroderma, morphea plana atrophica, sclérodermie atrophique d'emblée) Calcinosis–Raynaud phenomenon–esophageal dysmotility–sclerodactyly–telangiectasia syndrome (CREST syndrome) Chilblain lupus erythematosus (chilblain lupus erythematosus of Hutchinson)