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[Prognosis and treatment of scleroderma].

The prognosis and treatment of scleroderma depend on the clinicopathological form of the disease: superficial, localised scleroderma is only a minor cosmetic problem; circumscribed, deep forms of scleroderma may cause troublesome symptoms which justify steroid therapy. Circumscribed and diffuse scleroderma are similar to systemic scleroderma for which prognostic criteria have been identified. There is a poor prognosis in men, in extensive skin involvement and in intestinal, cardiac or renal involvement. Limited skin disease and anti-centromere antibodies are associated with a good prognosis. The absence of an animal model and the uncertainty as to the underlying cause, explain the therapeutic problems. The authors discuss the respective value of a number of "specific" drugs, including D-penicillamine which seems to be the most hopeful line of treatment. Continuous follow-up of this chronic condition remains essential for the diagnosis and symptomatic management of visceral complications.

Adrenal Cortex Hormones↗

Differential diagnosis of sclerodermoid skin changes.

Sclerotic changes in the skin are most frequently caused by scleroderma. In those cases in which scleroderma is not implicated, careful historical, physical, histologic, and laboratory evaluation will allow an appropriate diagnosis to be made. In this way, appropriate therapy and prognostication can be given.

Amyloidosis↗

[Distribution of fibronectin in the skin of patients with scleroderma].

The distribution of fibronectin (FN), a major glycoproteic component of extra-cellular matrix, has been studies by an indirect immunofluorescence technique in the skin of 50 normal controls and 19 sclerodermic patients. In the normal skin, FN was present mainly in the papillary dermis, as thin strips and less abundant in reticular dermis, bound to collagen bundles. In scleroderma skins, FN was increased in the deep dermis of extensive and evolutive lesions (11 cases). In an other hand, the distribution of FN was not modified in stabilized lesions (8 cases). We conclude that the detection of FN in the scleroderma skin is an useful marker of the activity of the systemic sclerosis process and we discuss the possible role of FN as a primary matrix for organization of the collagenous connective tissue during the sclerosing process.

Extracellular Space↗