The main clinical manifestations of systemic scleroderma

Systemic scleroderma (progressive systemic sclerosis) is a disease that is not only unpleasant due to thickening of the skin and discoloration, but also dangerous by affecting the internal organs – damage to the joints, kidneys, cardiovascular system. Scleroderma can manifest itself in different forms, each of which has its own course and prognosis.

Systemic scleroderma (SSD) – a staging multiple organ disease with characteristic vasospastic vascular reactions, in which peculiar autoimmune disorders develop, accompanied by activation of fibrosis with excessive deposition of collagen and other components in the tissues.

What processes occur in the skin with systemic scleroderma?

Two main processes characterize changes in all affected organs in systemic scleroderma – development of obliterating microangiopathy and fibrosis. 

Pathological marker of scleroderma – excessive deposition of extracellular matrix in the dermis, which due to this becomes thickened and dense.

About causes of scleroderma read on estet-portal.com. In the early stages of the disease, cellular infiltration is detected at the border of the reticular dermis and subcutaneous tissue, mainly around the dermal vessels. Systemic scleroderma is characterized by vascular damage – proliferation with narrowing of the lumen of arteries and arterioles, desolation of capillaries. Microcirculation disorders are accompanied by an increase in vascular permeability and the release of the liquid part of the plasma into the tissues. Gradually develop atrophy of the skin appendages, thinning of the epidermis, disappearance of the papillae and atrophy of the dermis.

Symptoms and clinical signs of systemic scleroderma

The clinical picture of systemic scleroderma includes a wide range of manifestations. One of the first symptoms of the disease – Raynaud's syndrome (SR) – symmetrical paroxysmal spasm of arteries, arterioles, caused by cold, emotional stress, reports estet-portal.com. The syndrome has two or three phases, ie. successive changes in skin color: blanching, cyanosis and redness.

Episodes of vasospasm are accompanied by numbness, pain, are asymmetric and are often associated with recurrent ischemic skin lesions – digital scars or sores, less often – with dry necrosis or even gangrene. Staging is also characteristic of skin lesions.

One of the early signs of systemic scleroderma, which is of great diagnostic value, – dense swelling of the skin and underlying tissues. Because of the swelling of the fingers, the hand does not clench well into a fist, especially in the morning.

Sometimes diffuse hyperpigmentation develops early in the disease. The progression of systemic scleroderma is accompanied by skin induration – an increase in its density and a decrease in elasticity. The skin is difficult to fold and is much thicker than normal skin. At the stage of atrophy, the skin acquires a cyanotic brown color due to hyper– and dispigmentation, it becomes thinner, it has a characteristic luster, it becomes rough, dry, the hairline disappears.

Calcifications may appear in soft tissues – small subcutaneous and intradermal deposits of calcium salts, which open with the release of curdled mass and the formation of long-term non-healing ulcers. The typical manifestations of the advanced stage of the disease include the symptom of ”pouch”, when radial folds form around the mouth.

Main clinical manifestations of systemic scleroderma:

• Raynaud's syndrome;
• hyperpigmentation;
• swelling of fingers;
• skin induration;
• skin atrophy;
• subcutaneous calcifications;
• symptoms of "pouch";
• telangiectasias;
• synovitis.

What organs and systems can be affected in systemic scleroderma?

Late signs of the disease are telangiectasia – dilated capillaries and venules grouped into a bundle. The number of telangiectasias increases with time, they are localized on the face, hands, in the décolleté zone.

In systemic scleroderma, the musculoskeletal system is often affected. Polyarthritis with severe synovitis may develop (in the debut - often like rheumatoid arthritis), as well as tenosynovitis. Typical resorption of the end sections of the distal phalanges of the hands due to prolonged ischemia, which is manifested by a decrease in the volume of the soft tissues of the fingertips, shortening and deformity of the fingers. The frequency of involvement of internal organs and its severity in systemic scleroderma varies widely: the digestive organs, heart, lungs and kidneys are affected.

At the first signs of systemic scleroderma a comprehensive examination and specific treatment is required. The sooner the treatment of systemic scleroderma is started, the higher the patient's standard of living will be.