Hospital pediatrics. Localized scleroderma (most important)

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Hospital pediatrics. Localized scleroderma (most important)

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26. Scleroderma localized

This is a chronic systemic disease of connective tissue, small vessels with widespread fibrous-sclerotic changes in the skin and stroma of internal organs and symptoms of obliterating endarteritis in the form of systemic Raynaud's syndrome.

Major criteria: scleroderma-like skin changes proximal to the major finger joints.

Small Criteria:

1) sclerodactyly;

2) dimpled scars or loss of soft tissue substance of the fingertips, hands and/or feet;

3) bilateral basal pulmonary fibrosis. Systemic scleroderma is a collagen disease that is associated with increased collagen formation and fibrosis, functionally defective fibroblasts and other collagen-forming cells. The predisposition is family genetic, women are ill 3 times more often than men.

Clinic. The clinical picture usually begins with Raynaud's syndrome (vasomotor disturbances), trophic disorders and persistent arthralgia, weight loss, increased body temperature, and asthenia.

Systemic scleroderma begins with a single symptom and quickly acquires the features of a multisyndromic disease. Skin lesions are a characteristic symptom of the disease.

The most common is dense edema, followed by thickening and atrophy of the skin. More often, changes are localized on the skin of the face, limbs, very rarely the skin of the entire body becomes dense.

Muscular syndrome manifests itself in the form of myalgia, characterized by progressive compaction, then muscle atrophy, and a decrease in muscle strength.

The damage to the joints is mainly associated with the pathological process of the periarticular tissue (skin, tendons, articular bags, muscles).

A special diagnostic sign is osteolysis of the terminal, as well as in severe cases, middle phalanges of the fingers, very rarely of the legs. Lung damage in the form of diffuse or focal pneumofibrosis, accompanied by emphysema and bronchiectasis. Complaints of shortness of breath, difficulty in deep breathing, hard breathing. Systemic scleroderma often has a chronic course, and the disease lasts for several decades with minimal activity of the process and the gradual spread of the lesion to various internal organs, the functions of which are not disturbed for a long time. These patients suffer mainly from diseases of the skin and joints with trophic disorders. The death of such patients occurs within 1-2 years from the onset of the disease. Forms of focal scleroderma.

1. Plaque form.

2. Keloid form.

3. Linear form.

4. Deep subcutaneous nodular form.

5. Atypical forms.

Treatment. The principles of treatment are as follows.

1. Antifibrotic agents.

2. The use of NSAIDs.

3. Treatment with immunosuppressive agents.

4. The use of antihypertensive drugs.

Author: Pavlova N.V.

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