Causes of the disease are unknown. It is believed that scleroderma develops under the influence of some external factors in people with certain genetic disorders. The external factors that can trigger the development of scleroderma include retroviruses (especially cytomegalovirus), quartz and coal dust, organic solvents, vinyl chloride, some drugs (bleomycin, and a number of other drugs used for chemotherapy).
What are the symptoms of scleroderma
As a systemic disease characterized by the simultaneous defeat of scleroderma skin, vascular, musculoskeletal system and internal organs, including the heart, lungs, kidneys and gastrointestinal tract.
A typical early symptom of sclerodermaRaynaud's syndrome is a - transient episodes of spasm of the vessels in the skin of limbs under the influence of cold or emotional stress. Raynaud's syndrome is manifested clinically clearly defined areas of discoloration of fingers. At the beginning of an attack of vasospasm of fingers become pale coloration, which in a few minutes changes to a bluish-purple hue. After allowing spasm and restore blood flow occurs reddening of the skin and the skin becomes intensely pink. In some patients, a vascular spasm attacks are accompanied by a feeling of freezing hands, numbness, or sensory disturbances. In the phase of redness, patients may feel pain in fingers. Vascular spasms may also be exposed to blood vessels of the skin and other areas. In these cases, there are characteristic changes color tip of the nose, lips and ears, above the knee.
The most specific sign of sclerodermais a skin lesion as its thickening and seals, which are observed in the vast majority of patients with scleroderma. The severity and prevalence of skin seals vary in individual patients, but the seal skin in scleroderma always starts with a brush of fingers, and in the future may extend further out on a limb and torso. Simultaneously with the fingers is often observed skin lesion, a result of which smooth out the nasolabial folds and frontal, thinning red border of the lips, around which there are radial lines. Long-term follow celebrated staging skin lesions: edema, induration, thinning. Seal skin has a tendency to progression in the first 3-5 years of the disease. In later stages of the disease the skin becomes less dense, and the seal is only fingers.
Often a sign of scleroderma is intensecoloration of the skin, limited or widespread, with areas of hypo- or depigmentation ( "salt and pepper"). A characteristic feature are ulcers on the fingers, which can be dramatically painful. Ulcerative skin lesions observed in other areas exposed to mechanical stress: over the elbow and knee joints in the ankles and heels. As a result of circulatory disorders appear scars on the fingers, dot thinning areas of the skin ( "rat-bite"). Hem on the fingers may also occur after ulcer healing. Due to the death of hair follicles, sweat and sebaceous glands of the skin in places seal becomes dry and rough, it loses hair.
Joint pain and morning stiffness are common manifestations of scleroderma, especially in the early stages of the disease.
The result is the destruction of circulatory disorders of nail phalanges, manifested shortening and deformity of the fingers.
The defeat of the muscles can lead to muscle weakness.
Involvement of the gastrointestinal tract develops90% of patients with scleroderma. The defeat of the esophagus manifested impaired swallowing, persistent heartburn, which increases after a meal. The defeat of the stomach and duodenum is manifested by abdominal pain, flatulence. The defeat of the small intestine often asymptomatic, but expressed changes develop a syndrome of impaired intestinal absorption of diarrhea, flatulence and weight loss. The consequence of the defeat of the colon are constipation.
Pulmonary disease develops more than 70% of scleroderma patients and manifests increasing shortness of breath and persistent cough.
Other common manifestationsscleroderma include Sjogren's syndrome (20%) and thyroid disease (Hashimoto's thyroiditis or thyroiditis De Quervain), leading to a decrease in thyroid function.
How to diagnose scleroderma
Approximately half of the cases observedincreased erythrocyte sedimentation rate 20 mm / h. With the same frequency revealed signs of inflammatory activity in scleroderma: elevated levels of fibrinogen and seromucoid; less experienced higher rates of C-reactive protein.
In 10-20% of patients with anemia is detected, the causewhich may be of iron and vitamin B12 deficiency, renal failure or bone marrow itself. Of great importance is the identification of specific autoantibodies for scleroderma.
Among the many instrumental methodsResearch plays an important role Capillaroscopy nail bed. Methods of study of microcirculation, such as laser-Doppler flowmetry, plethysmography, and others, are of secondary importance in the diagnosis of scleroderma because of significant variability in results.