Skin Changes in Scleroderma

Scleroderma (dermatosclerosis) is a disease from the group of collagenoses with a predominance of fibro-sclerotic and vascular disorders in the type of obliterating endarteritis with widespread vasospastic changes developing mainly in the skin and subcutaneous tissue.

[1], [2], [3], [4], [5], [6], [7]

Causes and pathogenesis of skin changes in scleroderma

The causes and pathogenesis of scleroderma are not fully understood. Trigger factors are viral, bacterial infections, stress, trauma, etc. Currently, many scientists scleroderma is considered as an autoimmune disease. Detection of autoantibodies to connective tissue components, a decrease in T-lymphocyte counts with an increase in B-lymphocyte count, an increase in the concentration of immunoglobulins of all classes indicate an autoimmune disease.

As a result of autoimmune processes, the progressive disorganization of connective tissue collagen develops: mucoid and fibrinoid swelling, fibrinoid necrosis, hyalinosis and sclerosis. Acceleration of biosynthesis and maturation of collagen in the skin of patients with both limited and systemic scleroderma have been noted. In addition, microcirculatory disorders and changes in blood properties play an important role. The presence of family cases of scleroderma, the association of focal scleroderma with antigens HLA-B18, B27, A1, and BW40 confirm the role of heredity in the pathogenesis of scleroderma. In the emergence of scleroderma, an important role is played by the state of the nervous, endocrine systems and other organs of the body.

[8], [9], [10], [11], [12], [13], [14]

Histopathology

Morphological changes of the skin with limited and systemic scleroderma are similar. In the edema stage edema and homogenization of the collagen of the dermis and the walls of the vessels with lymphocytic infiltration are noted.

In the consolidation stage, the atrophy of the epidermis and the papillary layer of the skin is visible. Characteristic is the fusion of collagen beams in the dermis with signs of hyalinosis. Sebaceous and sweat glands are absent or atrophic. Characteristic thickening and mucoid swelling of the vascular wall, a sharp narrowing of the lumen of the vessels due to hypertrophied endothelial cells, sclerosis of their walls. Cellular infiltration is poorly expressed and is represented by lymphocytic-histiocytic elements.

In the stage of atrophy, a pronounced atrophy of all layers of the skin and appendages is observed.

Symptoms of skin changes in scleroderma

In the clinical course of scleroderma there are 3 stages: edema, compaction, atrophy. Plaque scleroderma occurs in all regions of the world. Mostly women are ill, men and children are more rare. The lesions are located on the trunk, upper and lower extremities, neck. Scleroderma begins with the formation of single or multiple, slightly swollen round or oval spots of violet-generic color with a diameter of 5-15 cm or more. These changes correspond to the stage of edema. Over time, the center of the spots becomes denser, erythema becomes less pronounced or disappears and the focus becomes white with a yellowish hue. In this case, the peripheral zone remains cyanotic in the form of a lilac ring. Sometimes the compaction seizes subcutaneous fat and muscle and achieves a cartilaginous consistency. The surface of the hearth becomes smooth, the skin acquires the color of ivory, has a waxy shine, hair, sweat and salonism are absent. For several months or years, the lesion focus may increase in size due to peripheral growth. Subjective sensations are absent. Then comes the third stage of the disease - the stage of atrophy, in which the compaction gradually dissolves, the skin becomes thinner like a tissue paper, it is easily collected into a fold, it falls due to the atrophy of the underlying tissues.

Rare and atypical forms of plaque scleroderma include the knotty (with a pronounced compaction the foci swell), bullous-hemorrhagic (bubbles with hemorrhagic contents appear), necrotic (after the blisters there is ulceration of the tissues).

Linear scleroderma in its development passes through the same stages as plaque, differing only in the configuration of the foci. The lesions are located longitudinally along the sagittal line, to the forehead, passing to the back of the nose and resembling a deep scar after the impact of the saber. On foci may occur in other areas of the skin. At the same time atrophy is not limited to the skin, but extends to the underlying muscles and bones, deforming the face. Often, linear scleroderma is combined with the hemiatrophy of Romberg's face. This form is more common in children.

Superficial limited scleroderma - a disease of white spots (scleroatrophic leuchen) is usually found in women on the skin of the neck, upper chest or on the genitals. The disease begins with the appearance of small (up to 5 mm in diameter) spots of a snow-white color, often surrounded by a pinkish-lilac whisk, which subsequently become brown. The center of spots sticks, kamadons are often visible, atrophy develops later.

With limited scleroderma, the same patient may have a combination of different forms. Of the concomitant diseases, cardiovascular, nervous and endocrine systems and the musculoskeletal system can be affected.

Systemic scleroderma affects mainly women, much less often - men and children. Characterized by systemic disorganization of connective tissue of the skin and internal organs. The disease usually begins with a prodromal period (malaise, weakness, joint and muscle pain, headache, subfebrile temperature). Trigger factors are often stress, hypothermia, or trauma. There are acrosclerotic and diffuse forms of systemic scleroderma. The allocation of CREST-syndrome, according to many dermatologists, seems to be justified.

In the acrosclerotic form, the skin of the distal parts of the face, hands and / or feet is typically affected. In most patients, the first skin symptom is blanching or reddening of the distal phalanges of the fingers due to spasm of the vessels. The skin becomes taut, shiny, acquires a whitish or cyanotic-pink hue. Cyanotic coloration of the skin, numbness. When pressing for a long time, the pits are preserved (the stage of dense edema). Over time, an average of 1-2 months, develops a second stage of the disease - the stage of densification. The skin becomes cold, dry due to sweat and sebum, its color acquires a shade of old ivory, on the surface there are foci of telangioktasia and hypo- and hyperpigmentation. Movement of the fingers is limited, the contracture of the fingers may come.

In the third stage (the stage of atrophy), thinning of the skin and atrophy of the muscles of the hands are noted, as a result of which the fingers resemble the "fingers of the Madonna".) Some patients develop long-lasting non-healing trophic ulcers. When the face is affected, there is a mask-like face, a narrowing of the oral opening, a sharpening of the nose and a cyst-like fold around the mouth. Often the mucous membranes are affected, often the mouth. The edema stage is mocked by the compaction step. The tongue is fibrous and wrinkled, becomes rigid, which makes speech and swallowing difficult.

In the diffuse form of systemic scleroderma, generalization of the skin lesion and the involvement of internal organs in the pathological process are noted. In this case, the disease begins with the trunk, then spreads to the skin of the face and limbs.

When systemic scleroderma often develops CREST-sypdrom (calcinosis, Reyno-syndrome, esophagopathy, sclerodactyly, telangiectasia, musculoskeletal disorders (arthralgia, polyarthritis, joint deformities, contractures, osteoporosis, osteolysis), gastrointestinal tract (flatulence, vomiting, constipation or diarrhea, decreased secretory and motor function), neurasthenic and psychological disorders (neurocirculatory dystonia, hyperhidrosis of the palms and soles), trophic disorders (hair loss, nails).

Classification of scleroderma

There are two forms of scleroderma - limited and systemic, of which each has clinical varieties. Most dermatologists view these changes in the dermis as a manifestation of a single process. At the heart of these forms of the disease is a single or very similar pathological process, limited to individual parts of the skin in focal scleroderma, and generalized both in relation to the skin and other organs - in the systemic. In addition, there is a uniformity in the histological picture of the skin with limited and systemic scleroderma.

Limited scleroderma is divided into plaque, linear and spotted (white spot disease, or sclerotrophic leuchen).

[15], [16], [17], [18], [19], [20], [21], [22]

Differential diagnosis

Limited scleroderma should be distinguished from vitiligo, leprosy, atrophic form of red flat lichen, kraurosis vulva.

Systemic scleroderma should be differentiated from dermatomyositis, Raynaud's disease, sclerosis of adult Busca, sclera and scleraemia of newborns.

[23], [24], [25], [26], [27], [28], [29], [30], [31]

Treatment of skin changes in scleroderma

First, it is necessary to eliminate provoking factors and concomitant diseases. Antibiotics (penicillin), antifibrotic agents (lidase, ronidase), vasoactive drugs (xanthinal nicotinate, nifedipine, actovegin), vitamins (groups B, A, E, PP), antimalarial drugs (delagil, resichin) are often used to treat limited scleroderma. Physiotherapeutic procedures use low-intensity laser radiation, hyperbaric oxygenation, paraffin applications, phonophoresis with lidase. Outwardly recommend corticosteroids, drugs that improve trophic (actovegin, troxevasin). With the systemic form of patients hospitalized. Assign, in addition to the above drugs, systemic glucocorticosteroids, a-penicillamine, kurrenil, noted a good effect of neotigazone.