Scleroderma

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scleroderma

[‚skler·ō′dər·mə]
(medicine)
An abnormal increase in collagenous connective tissue in the skin. Also known as chorionitis; dermatosclerosis; scleriasis.

Scleroderma

 

a disease of man, of the group of collagen diseases, characterized by thickening of the skin and underlying tissues; in systemic scleroderma, the internal organs are affected as well. Scleroderma may be circumscribed (localized), diffuse cutaneous, or systemic. Localized scleroderma, known as morphea, may occur in patches or linear lesions, or it may be superficial. The patches appear on any area of the skin, initially as pinkish red rounded or oval spots varying in size, with subsequent thickening in the center of the focus. The skin becomes waxy yellow and smooth, and loss of hair occurs. There are no subjective sensations. Several months or years later, atrophy of the skin develops at the site of thickening.

Linear scleroderma, marked by single or multiple linear lesions, occurs most frequently in children and is limited to the face or extremities. Guttate morphea (white-spot disease) is a manifestation of the superficial form of localized scleroderma and occurs mainly in women. It is marked by the formation of small, firm white spots with a nacreous gloss in the upper spinal region, the chest, and the genitals; superficial atrophy of the skin develops later at these sites.

Diffuse scleroderma affects the face, extremities, and torso. A compact edema is succeeded by thickening and then by atrophy, the face becomes masklike, and the fingers lose their capacity to move and remain in a half-flexed position, taking on the appearance of birds’ claws (sclerodactyly).

Systemic scleroderma (also called progressive systemic sclerosis) is marked by generalized sclerotic changes in the connective tissues and finer vessels. Factors inducing the disease include continuous hypothermia (cooling), physical and mental traumata, and intolerance to medication. Systemic scleroderma is characterized mainly by the disruption of microcirculation and of the functioning of the connective tissue as a whole, particularly of fibrogenesis. The disease affects principally middle-aged women. It develops gradually, with initial manifestations including spasms of the vessels of the extremities, impairment of the movement of joints, joint pains, and affection of the skin. Involvement of the interstitial tissue and of the vessels of internal organs leads to fibrosis of the lungs and heart (primarily the myocardium and system of valves) as well as the esophagus and other organs of the gastrointestinal tract; the functions of the affected organs are disrupted.

Scleroderma is treated by eliminating the disease’s causative factors and by administering corticosteroids, vasodilators, and agents acting on the permeability of vascular connective tissue barriers. Physiotherapy is also used to treat the disease. When scleroderma is chronic, treatment is carried out at sanatoriums and health resorts such as those located at Sochi, Piatigorsk, and Evpatoriia.

REFERENCES

Tareev, E. M. Kollagenozy. Moscow, 1965.
Nesterov, A. I., and Ia. A. Sigidin. Klinika kollagenovykh boleznei, 2nd ed. Moscow, 1966.
Gvseva, N. G. Sistemnaia sklerodermiia. Moscow, 1975.
La Sclérodermic. Paris, 1972.

V. A. NASONOVA and I. IA. SHAKHTMEISTER

References in periodicals archive ?
Key Words: Antiphospholipids, Systemic scleroderma, Complication, Senegal
Anti-centromere antibodies are seen in 50% (1) to 71% (4) of patients with CREST but in only 21% of patients with diffuse systemic scleroderma.
The most significant predictor of progression to systemic scleroderma in a patient with new-onset Raynaud's phenomenon is the presence of capillary abnormalities at the proximal nail fold, according to David H.
Although the sclerodermoid variant of porphyria cutanea tarda (PCT) is well recognized, the concurrence of systemic scleroderma and PCT has rarely been documented.
CREST syndrome, a relatively benign, slowly progressive variant of systemic scleroderma consists of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia.
arGentis Pharmaceuticals, LLC announced today that the European Medicines Agency (EMEA) accepted the company's product candidate ARG201 (native type 1 bovine collagen) for the treatment of diffuse systemic sclerosis, also known as systemic scleroderma (SSc) for designation as an orphan medicinal product in the European Union.
arGentis Pharmaceuticals, LLC announced today that the European Medicines Agency's (EMEA) Committee for Orphan Medicinal Products (COMP) adopted a positive opinion recommending the company's product candidate ARG201 (native type 1 bovine collagen) for the treatment of diffuse systemic sclerosis, also known as systemic scleroderma (SSc) for designation as an orphan medicinal product to the European Commission.