Cutaneous changes in scleroderma

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

[ 1 ], [ 2 ], [ 3 ]

Causes and pathogenesis of skin changes in scleroderma

The causes and pathogenesis of scleroderma are not fully understood. Trigger factors include viral and bacterial infections, stress, trauma, etc. Currently, many scientists consider scleroderma to be an autoimmune disease. The detection of autoantibodies to connective tissue components, a decrease in the content of T-lymphocytes with an increase in the level of B-lymphocytes, and an increase in the concentration of immunoglobulins of all classes indicate an autoimmune genesis of the disease.

As a result of autoimmune processes, 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 has been noted. In addition, microcirculatory disorders and changes in blood properties are of no small importance. The presence of familial 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. The state of the nervous, endocrine systems and other organs of the body plays an important role in the development of scleroderma.

[ 4 ], [ 5 ], [ 6 ], [ 7 ]

Histopathology

Morphological changes in the skin in limited and systemic scleroderma are similar. In the edema stage, edema and homogenization of the collagen of the dermis and vessel walls with lymphocytic infiltration are observed.

In the compaction stage, atrophy of the epidermis and papillary layer of the skin is visible. The fusion of collagen bundles in the dermis with signs of hyalinosis is characteristic. Sebaceous and sweat glands are absent or atrophic. 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 are characteristic. Cellular infiltration is weakly expressed and is represented by lymphocytic-histiocytic elements.

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

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. It mainly affects women, less often - men and children. The lesions are located on the trunk, upper and lower limbs, neck. Scleroderma begins with the formation of single or multiple, slightly edematous round or oval spots of a purple-birth 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 thickens, erythema becomes poorly expressed or disappears and the lesion acquires a white with a yellowish tint color. At the same time, the peripheral zone remains cyanotic in the form of a lilac ring. Sometimes the compaction captures the subcutaneous tissue and muscles and reaches a cartilaginous consistency. The surface of the lesion becomes smooth, the skin acquires the color of ivory, has a waxy shine, hair, sweat and sebum secretion are absent. Over the course of several months or years, the lesion may increase in size due to peripheral growth. Subjective sensations are absent. Then comes the third stage of the disease - the atrophy stage, in which the compaction gradually dissolves, the skin becomes thinner like tissue paper, easily gathers into a fold, and sinks due to atrophy of the underlying tissues.

Rare and atypical forms of plaque scleroderma include nodular (with pronounced compaction, the lesions bulge), bullous-hemorrhagic (blisters with hemorrhagic contents appear), and necrotic (tissue ulceration remains after the blisters).

Linear scleroderma goes through the same stages in its development as plaque scleroderma, differing only in the configuration of the lesions. The lesions are located longitudinally along the sagittal line, on the forehead, passing to the bridge of the nose and resembling a deep scar after a sabre blow. But lesions can also be found on other areas of the skin. In this case, atrophy is not limited to the skin, but spreads to the underlying muscles and bones, deforming the face. Linear scleroderma is often combined with Romberg's hemiatrophy of the face. This form is more common in children.

Superficial limited scleroderma - white spot disease (lichen sclerosus) is usually found in women on the skin of the neck, upper chest or genitals. The disease begins with the appearance of small (up to 5 mm in diameter) snow-white spots, often surrounded by a pinkish-lilac halo, which later turn brown. The center of the spots sinks, comedones are often visible, and atrophy develops later.

In limited scleroderma, a combination of different forms may be observed in the same patient. Associated diseases may include damage to the cardiovascular, nervous, endocrine systems, and musculoskeletal system.

Systemic scleroderma affects mainly women, much less often - men and children. It is characterized by systemic disorganization of connective tissue of the skin and internal organs. The disease usually begins with a prodromal period (malaise, weakness, pain in the joints and muscles, headache, subfebrile temperature). Trigger factors are often stress, hypothermia or trauma. A distinction is made between acrosclerotic and diffuse forms of systemic scleroderma. The allocation of CREST syndrome, according to many dermatologists, is apparently considered 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 pallor or redness of the distal phalanges of the fingers due to vascular spasm. The skin becomes tense, shiny, and acquires a whitish or bluish-pink tint. Cyanotic coloration of the skin and numbness are noted. When pressed, a pit remains for a long time (stage of dense edema). Over time, on average after 1-2 months, the second stage of the disease develops - the stage of compaction. The skin becomes cold, dry due to sweat and sebum secretion, its color acquires the shade of old ivory, there are foci of telangioctasia and hypo- and hyperpigmentation on the surface. Finger movement is limited, contracture of the fingers may occur.

In the third stage (the atrophy stage), thinning of the skin and atrophy of the hand muscles are observed, as a result of which the fingers resemble "Madonna's fingers.") Some patients develop long-term non-healing trophic ulcers. When the face is affected, a mask-like face, narrowing of the oral opening, a pointed nose and purse-string folds around the mouth are observed. Mucous membranes, often of the mouth, are often affected. The edema stage is followed by the compaction stage. The tongue becomes fibrotic and wrinkled, becomes rigid, which makes speech and swallowing difficult.

In the diffuse form of systemic scleroderma, generalization of skin lesions and involvement of internal organs in the pathological process are observed. In this case, the disease begins with the body, then spreads to the skin of the face and extremities.

Systemic scleroderma often causes CREST syndrome (calcinosis, Raynaud's syndrome, esophagopathy, sclerodactyly, telangiectasia, lesions of the musculoskeletal system (arthralgia, polyarthritis, joint deformation, 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).

Classifications of scleroderma

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

Limited scleroderma is divided into plaque, linear and spotty (white spot disease, or lichen sclerosus).

[ 8 ], [ 9 ], [ 10 ], [ 11 ]

Differential diagnosis

Limited scleroderma should be distinguished from vitiligo, leprosy, atrophic lichen planus, and vulvar kraurosis.

Systemic scleroderma should be differentiated from dermatomyositis, Raynaud's disease, Buschke's scleredema of adults, scleroderma and scleredema of newborns.

[ 12 ], [ 13 ], [ 14 ], [ 15 ], [ 16 ], [ 17 ], [ 18 ]

Treatment of skin changes in scleroderma

First, it is necessary to eliminate the provoking factors and concomitant diseases. In the treatment of limited scleroderma, antibiotics (penicillin), antifibrotic agents (lidase, ronidase), vasoactive agents (xanthinol nicotinate, nifedipine, actovegin), vitamins (groups B, A, E, PP), antimalarial drugs (delagyl, resoquin) are often used. Physiotherapeutic procedures include low-intensity laser radiation, hyperbaric oxygenation, paraffin applications, phonophoresis with lidase. Corticosteroids and drugs that improve trophism (actovegin, troxevasin) are recommended for external use. In the systemic form, patients are hospitalized. In addition to the above drugs, systemic glucocorticosteroids, a-penicillamine, cuprenil are prescribed. A good effect from the use of neotigazone has been noted.