What is it - scleroderma( dermatosclerosis) - the disease is a mystery, appears quite unexpectedly against the background of immunopathological processes.
Rapidly progressing, causes specific changes in the human body:
1) Inflammation and condensation of the skin;
2) Appearance of ring-shaped bruises on its surface;
3) Diseases of connective tissue;
4) Provokes sclerotic damage to the joints and internal organs.
Causes of scleroderma
The disease is more common in women on the background of functional nervous disorders and disorders of the endocrine nature - after labor, abortion or during menopause. But a purely female disease scleroderma can not be named, because its individual forms are manifested in children and the "strong" half of humanity.
Dermatosclerosis is a poorly understood disease, so there are no clear boundaries. Often in the lesions observed hyperhidrosis, with excessive secretion of sebum or sweating disorder. In some cases an infectious version is found.
The impetus for the development of the disease can be the introduction of serums and vaccines, dosage forms, physical and mental trauma, cooling and burns. But the most important role in the onset of the disease is attributed to autoimmune pathologies in consequence:
of the neuroendocrine disorder;
processes of systemic hyalinosis;
disorders of immune and metabolic homeostasis;
decrease in the indices of cellular and humoral immunity.
Such pathologies cause:
1) Violations of the functions of fibroblasts, expressed by accelerated biosynthesis and the formation of abnormal collagen.
2) Manifestation of urticaria vasculitis, marked by a sharp weakening of blood flow, violation of movement of blood and lymph by capillaries and arterioles.
3) Immunopathological pathologies with the formation of immune complexes in relation to the nuclei of cells, collagen, vessels, muscles and endothelium, leading to tissue destruction.
Stages of development of scleroderma
On the course of the disease:
1) The stage of edema - manifested by atrophy, dystrophy, dermal sebum.
2) Stage of sclerosis - seals, discoloration of the skin, formation of vascular patterns are noted.
3) The stage of atrophy is marked by the manifestation of skin tension and deformation of the joints on the fingers, limiting the joint mobility.
On the basis of:
1) In the initial stage - joint pain, tachycardia, frequent colds. Timely treatment gives positive results
2) In the subacute stage - the signs of the initial stage manifest themselves in the maximum, acute manifestation.
3) The pre-death stage-terminal - is marked by a sharp weight loss, a far-gone pathological process. Treatment does not give positive results.
Symptoms of scleroderma
Scleroderma is classified into several types and forms, depending on the symptoms and prevalence of the processes.
Focal type of scleroderma - in men the process develops on the skin of the phallus, in women it is manifested by small foci on the back, in the scapular region, the skin of the breast and genitals, hands and possibly spread all over the body in the form of spots and plaques.
Therefore, the focal scleroderma species is classified into plaque and small-focal form. Symptoms are manifested as:
skin edema with the formation of plaques;
seals causing skin stiffness;
reduced its sensitivity.
A distinctive feature is the transience of the period of spots and plaques and the appearance in their place of atrophy, expressed by white spots.
The plaque form of the focal scleroderma species, in its turn, has three forms of manifestation - limited, widespread and striped.
1) The limited form of scleroderma appears on any part of the body, except the head, is characterized by three stages of development - firstly, various purple spots are formed, not manifesting themselves as special sensations. Then, in the center of the spot, a seal is formed in the form of a plaque. On the contours of the spot is visible pale lilac aureole. His presence speaks of the progression of the disease. In the last stage, tissue atrophy occurs at the site of the plaque.
2) Common or diffuse form - the gradual development of scleroderma can occupy any areas on the body. It is noted:
tension of the skin
swelling of the face, hands and feet;
occurs skin scleroderma;
soldering with the underlying tissues causes limited joint movements;
fingers on the hands are half-bent, ulcerous suppuration forms on their tips.
in the process of skin stiffness, limited movements of the facial muscles, neck, chest, difficulty breathing
person slowly but surely turns into a living mummy.
3) A streaky or linear form of scleroderma is a rare form of the disease. It affects very young ladies. It is localized on the limbs. Atrophies are affected by deep layers of tissues, joints and bones, muscles. Limited mobility of affected areas. A scar in the form of a saber blow is formed during the transition of dermatosclerosis from the scalp to the forehead.
Symptoms of systemic scleroderma
Systemic, diffuse form of scleroderma - the formation of systemic dermatosclerosis is usually the result of stressful situations, injuries, colds, herpes or herpes zoster.
Non-specific signs in the interinzhubatsionnom period manifested:
pain in the head, muscles and joints;
chills and fever;
with insomnia and severe fatigue;
face, hands and feet become pale and cool.
The onset of systemic scleroderma is characterized by such symptoms:
vascular spasm;
cyanosis and sensation of cold;
numbness and limited mobility of the joints of the hands;
by the tightness and tightness of the skin on the fingers;
skin acquires a brown tint;
manifestations of onychriphosis are expressed by a decrease in sweating, dry and cracked skin. The fingers are fixed in a semi-bent state.
The defeat process begins with the face and hands, gradually spreading, reaching the limbs. The color of the skin acquires a waxy shade, the fleece hair on the body falls out. A person turns into a mummy, acquiring sharpened fingers tightened with thin skin.
As a result of dense soldering of the skin with muscles and tendons, joint movements are difficult. Developing flexion tendon and spastic contracture.
Sclerotic and atrophic processes that cause changes in the subcutaneous tissue in the facial and skin muscles are the cause of the dystrophic changes in the face, expressed as:
with pointed nose,
with a narrowing of the oral opening;
by cheek and thinning of the lips;
lack of facial expressions and bronze casting of the face;
is affected by the mucous membrane of the mouth and tongue, dystrophy of the gum base.
At the same time, processes that affect the system of blood vessels and heart, GIT system, endocrine glands, kidneys, muscles of joints and bones can develop.
The acrosclerotic form of the systemic species differs little from the above described symptoms. To the characteristic clinical manifestations of systemic scleroderma - sclerodactylia are added manifestations of calcinosis - the deposition of calcium salts in the affected skin and telangiectasia, an increase in the vascular capillaries, in the form of vascular "meshes and asterisks".
With this form of the disease progresses slowly, the process can go into a diffuse form. Possible pathological violations of internal organs, in particular narrowing of the esophagus.
A juvenile form of a systemic species is characteristic:
by sclerotic fibrotic skin changes;
with vasospasm;
disorders in the functions of the musculoskeletal system;
strong weight loss;
is accompanied by rapid fatigue, weakness, fever and high temperature
This form leads not only to cosmetic defects, but also to disability.
Treatment of scleroderma
The possibilities of medicine in the fight against dermatosclerosis are limited. The main direction in the treatment of scleroderma is the elimination or alleviation of symptoms.
An individual program of the complex of exercise exercises for the development of joint motility is being developed. For the same purpose, therapeutic massage and various methods of occupational therapy are prescribed. Recommended:
mud and paraffin applications;
on the affected limbs - electrophoresis and plasmapheresis procedures;
diadynamic currents and ultrasound.
As medicamentous therapy,
preparations are prescribed, which suppress fibro-formation on connective tissues;
enzymes that cleave already formed seals;
anti-inflammatory and restorative drugs;
vitamin therapy and vasoactive therapy;
antibiotics.
Self-medication with such a serious disease is inappropriate. If you do not want to turn into a mummy, ask for qualified help from a doctor. And the earlier, the better.
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