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Disorder characterized by progressive systemic sclerosis (scleroderma), calcium deposits (calcinosis) usually in the fingers, Raynaud syndrome, loss of muscle control of the esophagus (difficult swallowing), osseous deformity of the fingers (sclerodactyly), and small red spots (telangiectasia) on the skin (fingers, face) or inside the mouth.

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Calcinosis.

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5:10,000,000 in adults; only 3% of total cases affect children.

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Unknown, but female-to-male ratio is 3:1. Dominant form described.

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Collagen (types I and III), fibronectin, and proteoglycans are deposited in the interstitium and the intima of small arteries. Fibrosis (involving several cytokines, especially interleukin-4) is found in clinically affected and unaffected tissue. Calcinosis is caused by deposits of carbonated apatite B. Autoimmune mechanism is suspected (increased chromosomal breakage rate is predominantly observed in linkage disequilibrium with HLA haplotype A1, Cw7, B8, C4AQ0B1, DR3, which is frequently observed in autoimmune diseases). A linkage between the fibrillin-1 gene locus and the scleroderma phenotype is suspected. Because of the similarity of scleroderma to chronic graft-versus-host disease, microchimerism is suspected in the pathogenesis of the disorder.

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Multisystemic disorder of connective tissue affecting the skin, which becomes hardened. Visceral involvement may be severe and affects the heart (myocardial fibrosis and secondary conduction defects, pericardial effusions), lung, kidneys (renal failure), intestinal tract (gastroesophageal reflux and strictures), and synovium. Whitish creamy discharge from the fingertips is observed occasionally. The deposit consists of carbonated apatite type B. The mineral substance seems to be less reactive and more stable than normal calcium pyrophosphate deposit in bone, explaining the total lack of effectiveness of calcium chelating agents.

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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).

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These patients are often treated with a systemic corticosteroid therapy; consequently, salt and water balance must be evaluated. Patients taking acetylsalicylic acid must be evaluated for bleeding. Evaluate mouth opening because of contracted perioral skin. Fiberoptic tracheal intubation should be available for induction of anesthesia. Pulmonary function tests prior to major procedures. Evaluation of cardiac, hepatic, and renal function is needed. Evaluation of venous access because of possible difficulties as a result of affected skin. Administration of antacid and cimetidine ...

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