Matlike telangiectasia, especially the face, upper trunk, and hands; also in the entire GI tract is Crest syndrome. Calcinosis over bony prominences, finger tips, elbows, and trochanteric regions.
The term CREST is an acronym derived from the five most prominent features:
- C – Calcinosis – calcium deposits in the subcutaneous layer of the skin
- R – Raynaud’s phenomenon – extreme sensitivity to cold or stress
- E – Esophageal dysfunction – swallowing problems caused by scarring
- S – Sclerodactyly – tightening of the skin confined to fingers and toes
- T – Telangiectasia – red spots on the hands, palms, forearms, face and lips
Pathogenesis for Crest syndrome is unknown. Primary event might be endothelial cell injury in blood vessels, the cause of which is unknown. Early in course, target organ edema occurs, followed by fibrosis; cutaneous capillaries are reduced in number; remainder dilate and proliferate, becoming visible telangiectasia. Fibrosis due to overproduction of collagen by fibroblasts.
Crest syndrome is a subset of scleroderma, a disorder that leads to thickening, hardening and tightening of your skin and connective tissue. In localized scleroderma the damage is confined to your skin and the tissue just beneath it, but systemic scleroderma is more far-reaching, affecting blood vessels and internal organs as well.