Scleroderma - a disease of the connective tissue characterized by a noticeable seal.The disease can be attributed to the unusual form of arthritis, which in some areas of the skin, and in rare cases, the skin is deposited on the entire scar tissue.
Scleroderma skin was known long ago, but to this day, this poorly understood disease.The disease is surprising for its manifestations, and for unpredictability.Currently, there are not enough specialists in this field.
Scleroderma Skin belongs to a group of collagen diseases, and is a disease of adults as well as children.Often the sick women, aged 30-50 years.
main causes of the disease scleroderma is hypothermia, endocrine disorders, skin injury, vaccinations, blood transfusions.Some cases have a genetic predisposition.The most important mechanism in the development of the disease is a disturbance of microcirculation and function of connective tissue.In order to early treatment of scleroderma, it is necessary
External factors that can lead to the development of scleroderma: retroviruses, organic solvents, quartz and coal dust, vinyl chloride, drugs (Bleomycin), used for chemotherapy.
Modern medicine distinguishes focal (limited), diffuse cutaneous and systemic scleroderma.
Alopecia (limited) form is patchy, and the strip surface.
Alopecia, strip (linear) form peculiar to children with places of localization on the face and extremities.
localized scleroderma manifested in the formation of purple-pink spots at various parts of the body.Often subject to attack your skin and face.Sometimes other areas are also subject to defeat.The spots formed on the skin of the patient, can be round, oval or oblong.Their sizes have the ability to vary.Some time later, the patient observes that the central part of the spot turns into a light, and then compacted.Further changes in the spot light yellow thick plaque having luster.For the safety of the characteristic plaques on the body up to several years.When treating localized scleroderma untimely fall hair disappear sweat and sebaceous glands, which leads to complete atrophy of tissue.
Diffuse scleroderma attacking the face, trunk, limbs, one after another until a tight seal swelling and the appearance of atrophy.A person takes a mask-like appearance, and frozen, half-bent fingers (sclerodactyly) visually similar to the bird's paws.
Systemic scleroderma skin is manifested in the deterioration of the general health of the sick.All muscles ache, fever, shivering patient, there is fatigue.In this case, all the skin is exposed to the disease.Initially the spots appear, then there is a transformation in the plaque.As a result, the skin turns into diseased ivory.Its seal acquires a degree that a person loses the ability to move.
Defeat all exposed skin face, and this in turn leads to the disappearance of facial expressions.His face turned to stone.But that's not all changes.May suffer nails, muscles, bones, tendons.Lose the ability wiggling fingers.System Form (progressive systemic sclerosis) is marked by extensive sclerotic changes in the small vessels, and connective tissue.The disease develops gradually, starting with vascular spasm of limbs, general stiffness, skin lesions, pain in the joints.With involvement in the pathology of internal organs develop pulmonary fibrosis, esophagus, heart (myocardium) is completely broken all the functions of the affected organs.
Systemic scleroderma skin also attacks the veins and capillaries.Because of the pathological processes that occur in the veins and capillaries develops Raynaud's syndrome.As a result, the fingers are cold sick, disturbed by pain.Cardio - vascular system and internal organs also respond to changes in the lung failure, urogenital system, kidneys and digestive tract.Another sign of systemic scleroderma appears dramatic weight loss.
Scleroderma leads to the formation of muscle weakness, the defeat of the gastrointestinal tract, which manifests itself in violation of swallowing, persistent heartburn, abdominal pain, flatulence, and may develop diarrhea, constipation.Lung damage occurs in 70% of patients, and manifests itself in the increasing shortness of breath and persistent cough.The following common manifestation of scleroderma - is Sjogren's syndrome, as well as the defeat of the thyroid gland (thyroiditis).
Scleroderma in children
Child age carries a lot of mysteries and hassles associated with scleroderma.Scleroderma, being a very rare disease, and children are not spared.Children disease called juvenile scleroderma.The disease is rare, it suffers from the from 2 to 12 children per million.
Juvenile scleroderma in children is the inflammatory nature of the connective tissue, and often affects the skin.
Scleroderma in children becomes a linear or patchy form.Both cases are characterized by rapid development.Patchy form occurs in girls and boys linear.There are distinctive features in the course of the disease.Inflammation spreading on the skin and subcutaneous tissue does not affect internal organs.But this does not mean that parents should remain calm and not to see a doctor.
Symptoms of scleroderma in children are manifested in the form of oval or strip-spots, different in magnitude.Affected areas with reddish swelling.Gradually, the affected areas become more dense, acquiring a shade of ivory.Next comes their atrophy.
Juvenile scleroderma diagnosis lends itself quite difficult becauseSymptoms of this disease are very similar to others.
How to treat scleroderma in children?
Any form of scleroderma is very difficult to treat.In this case, disease prevention is important, is to avoid hypothermia, exposure to chemicals, vibration, appearance microtrauma.Gold salts aid in the treatment of scleroderma.Treatment of the disease involves Auranofin, as well as vitamins A, B, PP, E, E. widely used methods of physical therapy: warming of the skin electrical current, ultrasound, laser and magnetic therapy, electrophoresis, ultrasound with drugs potassium iodide Lydasum therapy ozokerites, healing mud.
external therapy includes the use of creams and ointments with protivoliticheskimi substances (chymotrypsin, trypsin), with Dimiksidom, hormonal agents and vasoactive drugs.
After examination the doctor prescribes a full examination.
Analyses in scleroderma include (complete blood count, chest X-rays, blood chemistry, X-rays of the affected joints, ECG).Approximately half of the cases shows increased ESR 20 mm / h.In 20% of cases detected anemia due to iron deficiency, and vitamin B12, or kidney disease.The significance of determining the specific diseases autoantibodies.Important instrumental methods capillaroscopy scleroderma is the nail bed.
Before treatment it is important to eliminate the triggers.After touring the doctor prescribes a full examination.
Scleroderma is treated with corticosteroids, vasodilators, including physical therapy, as well as means of influencing the permeability of vascular connective tissue barriers.
therapy prescribed individually, it depends on the flow, forms, as well as the extent of damage the disease.The most effective are the vasodilators, calcium channel blockers (gallopamil, Verapamil, Nifedipine, Nicardipine, Amlodipine, Isradipine, nimodipine, lacidipine, Valium, nitrendipine, and others).
effectiveness of the treatment of vascular symptoms of scleroderma increases by connecting means which contribute to the fluidity of blood - antiplatelet agents (Ginkgo biloba, acetylsalicylic acid, dipyridamole, pentoxifylline, ticlopidine) and anticoagulants (Enoxaparin Sodium, acenocoumarol, Heparin Sodium, Warfarin, Nadroparin calcium, dalteparinSodium Ethyl Biskumatsetat).
antifibrotic therapy is used in the treatment of diffuse forms of scleroderma.For this purpose, connect the drug D-penicillamine, which inhibits the development of fibrosis, disrupting the synthesis of collagen.
patients is necessary to protect your face, hands and ears from the cold, and avoid over-active air-conditioning.An important consideration in the treatment gets a balanced diet, refusal of all alcohol and smoking, support normal weight, avoiding fatigue, vibration, stress.Focal infection is treated with antibiotics.The chronic course of illness in need of spa treatment in Pyatigorsk, Sochi, Yalta.