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Related to Systemic sclerosis: progressive systemic sclerosis, CREST syndrome


An abnormal increase in collagenous connective tissue in the skin. Also known as chorionitis; dermatosclerosis; scleriasis.



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.


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.


References in periodicals archive ?
Nursing problems of patients with systemic sclerosis.
Unlike systemic sclerosis, the overlying skin in EF is flexible enough to be pinched because the pathological disturbance is subcutaneous.
High N-terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis.
The challenge of early systemic sclerosis for the EULAR Scleroderma Trial and Research Group (EUSTAR) community: it is time to cut the Gordian knot and develop a prevention or rescure strategy.
Preliminary criteria for the classification of systemic sclerosis (scleroderma).
The differential diagnosis for ISSc includes dSSc, pseudoscleroderma, and systemic sclerosis sine scleroderma (ssSSc) (table).
We conducted a systematic review of English language citation in PUBMED database referring to the keywords rheumatic disease OR autoimmune disease OR systemic sclerosis AND skin autofluorescence OR advanced glycation end products.
The diagnosis of scleroderma was established according to 2013 updated classification criteria for systemic sclerosis, proposed by The American College of Rheumatology/ European League Against Rheumatism (ACR/EULAR) [9].
Thyroid dysfunction has been reported in systemic sclerosis but it has been rarely reported in morphea.
As part of the transaction, NantCell also acquired VivaBioCell's diagnostic product, capable of identifying patients with systemic sclerosis and systemic lupus erythematosus.
AITDs have been reported to be commonly associated with other autoimmune diseases such as vitiligo or systemic sclerosis (scleroderma), but only rarely with CREST syndrome [19, 20].