A chronic disorder of unknown cause characterized by progressive fibrosis of skin and multiple organs, and by vascular insufficiency. Oral structures become indurated and stiff; radiographically, the periodontal ligament spaces may be thickened.
A form of chronic autoimmune disease involving the skin and connective tissue of the internal organs. Progressive systemic sclerosis (PSS) is a relatively rare disease that is a form of scleroderma. It is also known as systemic sclerosis (SS) and the crest syndrome and results in inflammation of the connective tissue surrounding the capillaries (tiny blood vessels). Internal scarring occurs as the inflammation heals; this shrinks and hardens the tissue. The disease may involve body organs and systems, including the esophagus, which tends to stiffen, as well as the intestines, lungs, heart, and kidneys. Gastrointestinal bleeding can occur. PSS can occur in both men and women, but usually affects women in their 30s and 40s. Individual incidents of disease are different from one another, and severity may vary widely from one person to another.
A chronic disease of unknown etiology that occurs four times as frequently in women as in men. It causes sclerosis of the skin and certain organs including the gastrointestinal tract, lungs, heart, and kidneys. The skin is taut, firm, and edematous and is firmly bound to subcutaneous tissue, which often causes limitation of the range of motion; it feels tough and leathery, may itch, and later becomes hyperpigmented. The skin changes usually precede the development of signs of visceral involvement. A limited variant, called the CREST syndrome, includes only the following findings: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia.