Musculoskeletal ImagingScleroderma
also called progressive systemic sclerosis, a generalized disorder of connective tissue of unknown cause that affects principally the skin, lungs, gastrointestinal tract, heart, kidneys and musculoskeletal system. Another name for this disease is progressive systemic sclerosis. Severe
fibrosis and alterations of small blood vessels are among its pathologic features. Many of the diverse clinical manifestations in this disease are represented on radiographs as atrophy and
calcification of soft tissue and bone resorption. Frequently the abnormalities predominate in the phalanges of the hand, although
diffuse subcutaneous
calcification, widespread periarticular
calcification, and bone resorption are encountered at other sites, such as the mandible, the ribs and the clavicles. Joint alterations include erosive arthritis and intra-articular calcific collections.
Scleroderma has frequently been difficult to differentiate from other disorders associated with induration of the skin. Among the conditions with similar features are Raynauds phenomenon, acrosclerosis, diffuse systemic sclerosis, CRST syndrome, CREST syndrome, Thiebierge Weissenbach syndrome, and Shulman syndrome. In addition, some patients with scleroderma demonstrate clinical patterns that suggest the presence of more than one collagen vascular disease (collagen vascular overlap syndromes).
Intermittent pallor of the digits on exposure to cold (Raynauds phenomenon) is a common presenting feature of scleroderma.
On radiographs, hand alterations include soft tissue resorption of the fingertips, subcutaneous calcification and bone destruction. Erosion of the phalangeal tufts leads to pencilling, sometimes with destruction of much or all of the distal phalanx. Thickening of the periodontal membrane and mandibular resorption may result in loss of the lamina dura and loosening of the teeth. Erosions may also occur on the superior aspect of multiple ribs. In the spine, paraspinal calcification may be evident.
Joint involvement may be seen in the proximal and distal interphalangeal joints, the first carpometacarpal joints, the elbow, the inferior radioulnar joints of the wrist, metacarpophalangeal and metatarsophalangeal joints, knee and hip. A deforming nonerosive articular disease (Jaccouds arthropathy) identical to that occurring after rheumatic fever occasionally is observed in patients with scleroderma.
Arteriography is helpful in evaluating vascular abnormalities in patients with scleroderma, especially Raynaud's phenomenon.
Some patients may develop chemically induced scleroderma-like conditions from exposure to chemicals such as vinyl chloride, pentazocine, bleomycin, solvents, paraffin, silicone rubber prosthesis and cocaine. Furthermore, a toxic oil syndrome associated with scleroderma-like skin involvement has been associated with the consumption of an illegally marketed adulterated rapeseed oil sold as a cheap cooking oil.
Other conditions and syndromes associated with scleroderma are eosinophilic fasciitis (Shulman's syndrome) and graft versus host disease.
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