Scleroderma


Scleroderma

Description disease

The term scleroderma includes several infrequent disease, for which the common basic principles, outlined in the very name of the disease (from the Greek skléros- hard dermis- skin): Sosclerotherapy or pour the connective tissue contained in the dermis and subcutaneous tissue, while thinning the outermost layer skin epidermis. Features of the disease is usually chronic and progressive, without the initiation of treatment gradually deteriorating. The exact reason for the outbreak is still not known, but most kinds of scleroderma develops from autoimmune, it is therefore an abnormal immune system response against the body’s own substances.

It is a rare disease in the Czech Republic are reported per year, 3 new cases per 100 000 population.It occurs about four times more frequent in females and globally, it is interesting that it seems that the disease prefers Americans. Localized forms are then more frequent in childhood, diffusion are rarer and put in adulthood, most are diagnosed between thirty and fifty years ago.

The mechanism of tissue damage begins at the arterioles, thus the small blood vessels in all organs.First dies lining these vessels consequently smooth muscle in the wall, and these dead cells are then replaced by collagen and other components of connective tissue. In the wall of blood vessels accumulate inflammatory cells and their secretion action and then further increase the damage.

Risk factors for scleroderma

Risk factors for scleroderma are mostly incidence of illness in the family by blood relatives. Family occurrence is fairly common; responsible gene, but so far still failed to appear.

Prevention

For a number of autoimmune diseases has been observed their outbreaks after a stress to which the organism is exposed, whether it is psychological or physical stress. Therefore, it must overload the body, do not downgrade common diseases. These guidelines should be followed to prevent any illness, especially if the disease has occurred in the family.

Signs and symptoms of scleroderma

There are several types of scleroderma and therefore the symptoms are different individual types.The two basic types are cirkumskriptní scleroderma or localized and diffuse scleroderma.

Cirkumskriptní slerodermie

Cirkumskriptní, or localized, further has several forms. One of them, called morphea, is characterized by a deep red lined mincovitých bearings ivory sized to human palm. These are often several years to completely heal up. Other times may appear pruhovitá bearing on the limbs and head. Sklerodermických stripes bearing on the feet in children may affect bone growth and lead todeformities of the feet or restrict their mobility. At the head of the chin, forehead and crown deposits resemble scar from a saber cut, which also gave this type its French name “en coup de saber”.

These localized forms are generally limited to the skin, even though it may occur pulmonary hypertension. Course usually lasts 2-5 years, after which the skin thickening gradually slows until ceases.

diffuse scleroderma

Much more serious, and fortunately rarer diffuse scleroderma, which is again divided into two basic forms.

Akrosklerodermie

The so-called akrosklerodermie primarily affects young women and there initially and later presentRaynaud’s phenomenon, which is a binge zbělávání fingers due to spasms of the small arteries accompanied by joint pain. Soon there is a solidification of the skin and subcutaneous tissue to the spreading from the fingertips upward. Rarely are affected leg. Fingers become drápovitého character, what we sklerodaktilie. On the hand may be present ulcers or abnormal discoloration of the skin,typically a strong pain. Due to the involvement of the esophagus leads to disorders of swallowing and an associated loss of weight of further muscle weakness and breathing problems relative to lung disease. This type is very often coexists other symptoms, and this combination is called CREST syndrome. Individual letters in the name of the syndrome represent just the symptoms. The first is calcinosis, therefore, deposition of calcium in the skin that outwardly manifested as a whitish area on the surface of the skin, most commonly in joints. Often these deposits alone fall, sometimes it is necessary to proceed to surgery and remove them. Another symptom is already mentionedRaynaud’s phenomenon, and then affection muscles of the lower two-thirds of the esophagus with difficulty swallowing as nullifying or heartburn. The last two symptoms are just Sclerodactylyand telangiectasia, which is an extension of the capillaries visible as red spots on the face and hands, which, when pressed, will fade. This type of scleroderma can often be associated with other connective tissue diseases such as polymyositis, arthritis, or systemic lupus erythematosus, and this complex is then called mixed connective tissue disease.

Generalized malignant scleroderma

The second type, called malignant generalized scleroderma, is a very rare type, which unfortunately ** rapidly deteriorates ** and has a very poor prognosis.

After initial rigid edema formation occurs rapidly to solidify the majority of the body including the internal organs such as kidney, lung, esophagus, and fever is present and within a few weeks to months person dies.

Examination and diagnosis

Diagnosis is based mainly on history and physical examination. The diagnosis can determine both a dermatologist and physician, rheumatologist, orthopedic surgeon or a pulmonary physician. The doctor investigates mainly the look and feel of the skin, the overall appearance of the hand, notes the possible calcium deposits under the skin or changes in the appearance of blood vessels.Then often they proceed to laboratory testing, which can be detected by anti-Scl-70 andanticentromerové antibodies present in up to ninety percent of affected.

Sometimes it may even be removed biopsy of the skin, which may confirm scleroderma, but can not distinguish between a localized and diffuse form.

Therapy

Given that the exact cause of scleroderma is the individual patient is still unknown, and also because of the diversity of species scleroderma, it is necessary to treat each patient individually and select specific medication primarily aimed at alleviating symptoms of the disease.

Since scleroderma develops from autoimmune, thus due to abnormal immune response against the body’s own substances, the treatment is focused on suppressing these reactions, namely the suppression of the entire immune system. This can be achieved pharmacologically, thus using drugs such as methotrexate, cyclophosphamide or azathiopin. For cirkumskriptní scleroderma affecting only the skin is sufficient to higher doses of vitamin E, by penicillin and histamine injections. It is also important to focus on treating the symptoms.

It is necessary to treat in particular high blood pressure, to prevent renal failure. They are often used medications such as captopril. Anti-inflammatory manifestations can use colchicine.Rayunaudův syndrome can be solved by carrying out the warm gloves as well as administration of aspirin, which dissolves the small blood clots in the veins of fingers, with moderate symptoms and drugs that open the artery, such as nifedipine, or topical application of nitroglycerin. To improve blood circulation, we can use some kind of antidepressants, a so-called serotonin-reuptake inhibitors such as fluoxetine.

In the most severe cases, it is necessary to resort to surgery and interrupt nerve that innervate the affected arterioles causing them to spasm.

Heartburn can relieve the administration of omeprazole and antacids, by omitting caffeine and cigarettes and adjusting sleep, it means propping head to prevent the backflow of stomach contents.Indigestion caused by bacteria solved with antibiotics, usually tetracycline and macrolide such as erythromycin and finish eating the increased intake of fiber.

On irritated skin use various ointments, telangiectasia are dealt with laser therapy and avoiding sunlight.

Since scleroderma may also occur problems with the lungs, patients often feel relieved after application of oxygen terapie- therefore inhalation of pure oxygen. This results in a better supply of oxygen to the tissues and prevent them from further damage, so is spared the heart that may not work so intensively and thus are also fixed problems with breathing and a feeling of breathlessness.

Children with localized, cirkumskriptní form of scleroderma have a very good chance to virtually complete recovery, which often affected skin areas after a certain time regains its original character and appearance, in other cases after Morpheus, leaving only the weak spots on the skin. In systemic scleroderma it is to cure virtually impossible, but with medication, it can maintain calm and prevent further damage and also improve the quality of life of the patient.


How can I help myself

Patients often alone can achieve little, perhaps at akrosklerodermie patient can improve their condition by wearing warm clothes, mainly gloves, and should be avoided injuries that may make the disease worse.

It is essential to timely seek medical care, which can help to significantly slow the progression of the disease, and subsequent adherence to the treatment plan.

Complication

Complications of scleroderma are fairly common and very diverse, with speeches in almost all organ systems. Up to seventy percent pacietntů may result in scleroderma lung involvement and related breathing problems such as shortness of breath, cough, alveolitis (sweeping the pulmonary alveoli), pneumonia (pneumonia) or even causes the cancer in the lungs. Renal vascular damage leads due to the importance in maintaining renal blood varnish to increase it with all its negative effects on heart such as cardiac arrhythmia, heart hypertrophy to heart failure, further may cause headache, syncope, seizures or stroke. The already mentioned disability esophagus leads to swallowing disorders, which in severe cases can lead to cachexia, thus malnutrition. It may occur heartburn, since the modified cap and gastric acid from the stomach back into the esophagus. In other parts of the digestive tract changes may cause problems with digestion and the associated diarrhea and weight loss. It can occur even muscle weakness or pain and joint mobility change.

Other names: hardening of the skin, skin thickening, skin sclerosis, scleroderma

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