Because of the numerous combinations of potential
signs of the disorder, many clinical aspects are observed. Calcinosis
(subcutaneous calcareous concretions), telangiectasia, and scleroderma
(systemic sclerosis) are constant. Raynaud syndrome is very common.
Scleroderma often results in esophageal motility disorders (with permanent
strictures occasionally) and sclerodactyly. Calcinosis can involve many
joints (elbows, shoulders), periarticular areas (flank), and viscera
(heart). Clinically, the disorder is divided into two groups: (1) “pure” CREST
(calcinosis cutis, Raynaud phenomenon, esophageal motility disorder,
sclerodactyly, and telangiectasia), which occurs when patients have two or
more symptoms of CREST but do not meet the criteria for either limited or
diffuse scleroderma (no tight skin above their wrists, no pitting digital
ulcers, no lung fibrosis; and (2) “plus CREST,” when CREST symptoms appear
with another form of scleroderma (e.g., limited scleroderma plus CREST,
diffuse scleroderma plus CREST, any other autoimmune disease plus CREST).