What is Scleroderma
Scleroderma - a chronic inflammatorydisease of the connective tissue, which affects the skin of classical cases, but can also capture the gastrointestinal tract, heart, lungs, kidneys and synovial tissue. Skin lesions that are the cardinal symptom of the disease, can be focal, or generalized linear, symmetric distribution of skin cells. The latter form is usually accompanied by systemic lesions (progressive systemic sclerosis) and the typical adult patients. Children scleroderma typically has a focal form; systemic lesions are rare.
Scleroderma - a rare disease of unknown etiology. It can occur in children of any age; more common in boys. Family history is not checked.
Symptoms of scleroderma
The first early symptoms of the disease arefocal lesions of the skin and subcutaneous tissue. These elements are often arranged in a linear manner, similarly to the course of the peripheral nerves, and can be localized predominantly on one side of the body. In the early stages of skin lesion sites have a slightly erythematous appearance (skin redness), swelling, or looking atrophic (thinned) and shiny.
The patient may complain of pain or feelingtingling. As the disease progresses the lesions undergo densification, their edges become purple color and sometimes lifted, and the central part becomes pale, waxy appearance. Skin elements are increased in the circumferential direction and may merge with each other, capturing the entire limb or extensive areas of the body. The areas marked lesions develop scarring and fibrosis, skin tissue tightly soldered to the underlying structures. This process is sometimes so pronounced that leads to limit the growth of the affected limb and appearance of disfiguring contractures. chronic defeat areas may be hyperpigmented and depigmented.
Active pathological process canstop after a few months or years, or sluggish stretch for years. In the absence of systemic lesions Weather favorable for life. Progressive systemic sclerosis - a poor prognosis. Cutaneous lesions are symmetrical. They are located on the hands, feet and distal extremities, sometimes on the trunk and face. As in the focal forms of the disease may be a seal, pigmentary changes and diseased skin tissue soldering. In some cases, there are Raynaud's phenomenon and skin ulcers. Synovitis, especially of hand joints, can resemble a picture of rheumatoid arthritis (differential diagnosis); sometimes observed tenosynovitis (inflammation of the synovial tendon sheath) and nodules in the course of the tendon sheath.
Pathological process can capturegastrointestinal tract, heart, lungs and kidneys. Systemic manifestations of the disease, especially kidney damage, heart and lungs, sometimes leading to death. esophageal dysfunction may be the cause of chronic aspiration pneumonia. In some cases there is severe hypertension.
Diagnosis of scleroderma
Specific laboratory tests are not sclerodermaexist. ESR is often not changed. As with focal, and in disseminated forms of the disease are found rheumatoid factor and antinuclear antibodies. With the help of X-ray examinations can be detected esophageal dysmotility and small intestine. Functional studies of lung, electrocardiography and chest X-rays reveal lesions of the heart and respiratory systems. When kidney disease are marked changes in the urine and renal dysfunction.
No specific treatment exists. Attempts to treat scleroderma variety of means including corticosteroids, salicylates, chelating agents, chloroquine, radiation, dimethylsulfoxide, para-aminobenzoic acid, penicillamine, and cytotoxic agents. How ever failed to achieve a noticeable effect. Surgical resection of the affected skin with focal scleroderma does not stop the pathological process. In severe systemic lesions can try to apply corticosteroids, penicillamine, or cytotoxic agents. For the treatment of focal lesions topically applied corticosteroids. To prevent or minimize obezdvizhuschih contractures at an early stage of focal scleroderma shows intensive physiotherapy.