The skin, or rather its epidermis, thins.. At the same time or sooner, T lymphocytes, monocytes and plasma cells accumulate in the skin.
Then, under the epidermis, a mass of collagen protein is formed.
The result is an internal growth of the upper layers of the skin in the fiber and subsequent welding with deeper tissues. Everything that lies between the layers atrophies.
Gastrointestinal tract loses thickness of the skin.. This process begins approximately from the middle third of the esophagus, but may start even lower.
The motor function of the affected areas is gradually lost, they begin to expand. The muscular layer thins.
Fibrosis of the lungs originates in the wall of the bronchi.. It extends even more to the alveoli of lung tissue.
Tissue compaction causes emphysema, loss of ventilation, interruption of the natural exchange of carbon dioxide with oxygen.
As a result of pulmonary fibrosis, the walls may break. Small cysts form.
Scleroderma in the joints leads to infiltration of the inner lining Articular bags of plasma cells and lymphocytes. The fibrin is deposited in the thickness of the bag.
The same process occurs in the tendons.
Since the disease is accompanied by a violation of the nutrition of the muscles, they are affected by atrophy. Another consequence is a breach of contractility.
Tissue compaction occurs as a result of the infiltration of collagen and lymphocytes.
Scleroderma causes changes in the heart, and the main ones refer to the myocardium. The replacement of myocytes by scar tissue and dystrophy occurs due to hypoxia.
Fibrous areas cause pathological changes in the conduction of nerve impulses. Disturbed formation of excitation foci.
The main manifestations of these changes:
- Frequent arrhythmias
- Atrioventricular block.
The necrosis can appear in the kidneys, as a result of changes in the structure of the capillary glomeruli..
Other manifestations include a reduction in urine leakage and minor heart attacks. It is necessary to clarify that changes in renal tissue are observed in approximately 50% of patients.
Constriction of the hand vessels.
Scleroderma code in ICD-10
Scleroderma manifests clinically in various forms.
Therefore, the international classification of ICD-10 diseases in the registration process in one way or another divides the disease into groups:
- Class L – skin related diseases
- Class M – Diseases related to connective tissue pathologies.
This practice has made it possible to include known forms of the disease in a group whose coding is denoted as L94 and M34. The name of the group is “Systemic sclerosis”, “Other localized changes in the connective tissue”.
The following classification has been adopted:
- Focal scleroderma (limited)
- Scleroderma on plate
- Scleroderma tear
- Linear scleroderma
- Buske’s disease
- Generalized scleroderma
- Eosinophilic fasciitis
- Induced scleroderma.
You should briefly describe each form of the disease. Limited scleroderma affects only the areas of the skin.
The changes do not affect the internal organs.
The shape of the plaque is a change in the structure of the skin tissue. Part of the skin is rounded. The lesion is quite large.
Also characteristic are the bluish rings around the plots.
If you see a tear shape, you may see small, slightly round spots. Its color is whitish with a pearly nuance.
The surface of the site sinks a little inside. Scleroderma drops are found in the upper part of the body, closer to the head.
The ring-shaped form is a specific ring-shaped rash. They appear on the skin of the legs.
Very often, one of the symptoms of this form is the disease of the elephant.
Linear scleroderma is somewhat different from other forms. Its manifestation is a strip of skin on the forehead, manifested as a result of a pathology.
Buske’s disease is also known as the edematous form of scleroderma. Other names are adult sclerema, benign aponeurotic sclerema.
Such scleroderma is a rapidly progressive edema. It will be dense to the touch.
There is swelling in the face and neck. Possible dissemination will occur on the shoulders and forearms.
The pathology does not develop in the internal organs. A distinctive feature of Bushke is the connection of the disease with acute streptococcal infections.
Generalized scleroderma is also known as progressive sclerosis, diffuse or systemic scleroderma.
This is one of the most severe forms of scleroderma.
In this case, the injury affects all the connective tissue. Here there is a gradual capture of the pathological processes of the skin, and then the organs as well.
Eosinophilic fasciitis involves skin manifestations, which are very similar to most forms of scleroderma.
However, the difference between fasciitis is the accumulation of eosinophils in the tissue. In the course of the veins formed a specific “orange peel”.
The furrows of the veins begin to fall.
Fasciitis also has contractures of joint flexion.
The form of scleroderma, known as induced, becomes the result of a reaction to low quality silicone breast prostheses for the bleomycin and pentazine preparations. Another condition for appearance – a long contact of silicone or vinyl chloride.
Scleroderma sometimes occurs in combination with the following types of connective tissue diseases:
- Systemic lupus erythematosus
- Rheumatoid arthritis.
In this case, special syndromes are distinguished in the ICD-10 classification. It is extremely important to separate scleroderma from a series of diseases and forms of diseases that also lead to fibrosis.
Therefore, in case of a disease, the precise diagnosis is extremely important, in which mandatory punctures of the affected skin tissue and plaques are used.
At the same time, punctures of the tissues of the internal organs are made.
Sometimes abnormalities develop with scleroderma. One of them is dermatosclerosis: diffuse sclerosis of the skin.
The skin tightens and gradually loses its elasticity.
Causes of scleroderma
The etiology of scleroderma is not fully understood. The true causes of the disease are separated.
Only a series of factors that cause scleroderma were studied and scientifically substantiated.
These factors include:
- Genetic predisposition
- Genetic mutations
Viral infections (cytomegalovirus is especially dangerous)
- Carbon or carbon powder
- Contact or work with organic solvents.
- Polyvinyl chloride
- Chemotherapy in the treatment of cancer.
- Vibration effects
- Endocrine disruption
- Blood transfusion
- Some drugs
Specialists who study the blood composition of patients with scleroderma, found in the blood a high content of enzymes that damage the walls of blood vessels from the inside.
Clotting factor VIII was also detected in a large volume. The diabetic form of scleroderma also has characteristics, which will be discussed later.
The clinical picture of scleroderma.
The symptoms of scleroderma will develop according to the nature of the disease. As an example, a plaque form can be given, when the changes relate only to the skin.
Or vice versa: when systemic scleroderma occurs, the skin lesions are accompanied by injuries to muscles and joints, as well as internal organs.
Raynaud’s syndrome – the earliest manifestation. This syndrome is caused by vascular spasms in the arms and legs.
Temporal narrowing of the arteries, as one of the clinical manifestations, and some other early signs may appear in a cold or stressful state.
In the case of narrowing of the arteries, a spasm occurs, which is expressed in the pallor of the hands, and then in the cyanosis. When the spasm passes, the bluish areas turn pink.
In adults, there are several types of changes in skin sensitivity. Patients begin to freeze, even when they are hot. Such attacks are accompanied by painful sensations at the tips of the fingers.
Such phenomena can occur on the lips, on the tip of the nose or on the ears.
The process of compaction and thickening of the skin initially affects the fingers. This is a common reaction for most patients.
Then the compaction process extends to other parts of the body.
There is a typical facial expression characteristic of patients with scleroderma:
- Nasolabial septum smoothed
- Softens frontal wrinkles
- Radial wrinkles around the lips.
For the face it is characterized by the absence of facial expressions, that is, the face becomes a mask.
To understand that swelling in the skin is a sign of scleroderma, you can push it.
On the skin there are no traces or depressions.
In 90% of cases, changes in the skin go through three stages:
Clinical manifestations of scleroderma.
In running situations, ulcers form on the fingers, and these ulcers will be quite painful. There are scars, shaped like a cured bite.
Some ulcers appear below the ankles, on the heels, elbows and knees.
In the first of these signs, do not panic, but you should immediately consult a doctor. The fact is that similar manifestations are characteristic of a much milder disease, xeroderma.
Joint changes are characterized by pain in the morning. The patient feels a clear stiffness in all joints.
The nails begin to collapse and gradually thin out.
The fingers become shorter and thicker. Therefore, the hand seems to have lost its shape.
Muscle changes are accompanied by fatigue and weakness.
The vast majority of people with scleroderma have digestive problems. A person can not swallow, after a meal, heartburn begins.
Another common symptom is a rape of the stool, pain and swelling.
Scleroderma clinic in children.
Elimination of vascular changes.
- The destruction of fibrosis in the skin and internal organs.
- Supporting the functionality of internal organs.
- Correction of immunity.
Day of routine and nutrition.
Treatment involves adherence to a particular regimen throughout life. Otherwise, the scleroderma will progress. Patients can not sunbathe, experience hypothermia or vibration.
It is important to completely give up bad habits and caffeine.
Dairy products, spicy, smoked foods can cause allergies. While vegetables, fruits, brown rice and algae are the healthiest food for the patient and their immunity.
The obligatory vitamins for scleroderma are A, C and E, which have a positive effect on immunity. The B vitamins increase the resistance to infections.
The use of all products containing vitamins is possible at home and does not require periodic visits to special establishments.
Conservative treatment of scleroderma.
Conservative or pharmacological treatment consists of taking medications, the dosage of which is prescribed only by the attending physician. It will also control the frequency of intake and how the drugs combine with each other.
- Vasodilator medications (verapamil, flunarizine, lacidipine, and other calcium channel blockers)
- Adrenoreceptor blockers A (nicergoline, terazosin)
- Ginkgo biloba extract medicines (Tanakan)
- Synthetic Prostaglandin (Alprostadil)
- Prostacyclins (iloprost)
All groups of medications are necessary to relieve symptoms. Thus, vasodilator drugs relieve spasms of blood vessels, antiplatelet agents and anticoagulants prevent the formation of blood clots.
Also named Artamin and Kuprenil, which act through the immune system and limit fibrosis. In addition, D-penicillamine, a drug that reduces collagen synthesis to zero, will inhibit fibrosis.
For joints that periodically experience inflammation, NSAIDs (diclofenac, ketoprofen) are required along with hormone therapy. If swallowing is altered, the patient will have to drink prokinetic – Meklozin.
If the intestines or lungs are affected, then special therapy is needed, including sanatorium treatment. To eliminate the manifestations of scleroderma on the face, you can apply cosmetics: Cleoderma face products and many others.
Traditional methods of treatment
Folk remedies therapy is not recommended by experts. The reason for this is the lack of knowledge of the pathology, as well as its mechanism.
In addition, the prognosis of the course of the disease is not always obvious.
Even an experienced specialist can not predict the positive or negative effect of “popular” procedures. If the patient decides to try one of the methods, he should consult a doctor.
Prognosis of the disease
The prognosis will depend directly on the form of the disease and the initial manifestations.
Adverse factors are the age of the patient with a diagnosis of more than forty-five years, pulmonary fibrosis, arrhythmia and kidney damage in the first three years of the disease.
A disease such as scleroderma has a specific etiology and an individual course. The nature of the disease is not fully understood.
And only the correct diagnosis, made as soon as possible, and the appropriate treatment can play a positive role in the patient’s survival factor.
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