Skin rash in lung sarcoidosis. Sarcoidosis - which doctor treats? Why is sarcoidosis of the skin dangerous?

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What is sarcoidosis?

This disease often affects young and adults (up to 40 years old) people. In the elderly and children, sarcoidosis practically does not occur. Women get sick more often than men. The disease affects non-smokers more often than smokers.

Most scientists believe that sarcoidosis results from a combination of causes, which may include immunological, environmental, and genetic factors. This view is supported by the existence of familial cases this disease.

Classification of sarcoidosis according to the ICD

Stages of the disease

• Stage 0 - on the radiograph chest no changes are noted.
• Stage I - an increase in intrathoracic lymph nodes. Lung tissue is not changed.
• Stage II - enlarged lymph nodes in the roots of the lungs and in the mediastinum. There are changes (granulomas) in the lung tissue.
• Stage III - changes in lung tissue without an increase lymph nodes.
• Stage IV - pulmonary fibrosis (lung tissue is replaced by compacted connective tissue, respiratory function is irreversibly impaired).

Symptoms

• morning (the patient has not yet got out of bed, but already feels tired);
• daytime (you have to take frequent breaks in work for rest);
• evening (intensifies in the 2nd half of the day);

In addition to fatigue, patients may experience decreased appetite, lethargy, and lethargy.
With the further development of the disease, the following symptoms are noted:

• a slight rise in temperature;
• dry cough;
• muscle and joint pain;
• chest pain;
• dyspnea.

If the disease does not heal spontaneously, but progresses, pulmonary fibrosis develops with impaired respiratory function.

In the later stages of the disease, the eyes, joints, skin, heart, liver, kidneys, and brain may be affected.

Localization of sarcoidosis

Lungs and VLU

If left untreated, a patient with sarcoidosis of the eye can go blind.

Diagnostics

• Blood chemistry .
• Chest X-ray.
• Mantoux test (to exclude tuberculosis).
• Spirometry is a test of lung function using a special device.
• Analysis of fluid from the bronchi, taken with a bronchoscope - a tube inserted into the bronchi.
• If necessary, a lung biopsy is performed - removing a small amount of lung tissue for examination under a microscope. A piece of tissue necessary for analysis is removed using a special (puncture) needle or bronchoscope.

Where is sarcoidosis treated?

At the moment, patients with sarcoidosis can receive qualified assistance in the following medical institutions:

• Moscow Research Institute of Phthisiopulmonology.
• Central Research Institute of Tuberculosis Russian Academy Medical Sciences.
• St. Petersburg Research Institute of Pulmonology named after. Academician Pavlov.
• St. Petersburg Center for Intensive Pulmonology and Thoracic Surgery at City Hospital No. 2.
• Department of Phthisiopulmonology, Kazan State medical university. (A. Wiesel, the chief pulmonologist of Tatarstan, deals with the problem of sarcoidosis there).
• Tomsk Regional Clinical Diagnostic Polyclinic.

Treatment

Fatal outcome in sarcoidosis is extremely rare (in the case of a generalized form with total absence treatment).

Prevention

What are the symptoms of sarcoidosis and what are the causes of this disease? We will talk about how the diagnosis is made and what methods of treatment are possible for use in the treatment of affected organs: lungs, skin, bones, heart.

What is sarcoidosis - definition of pathology

Sarcoidosis- this is idiopathic disease(that is, the cause is unknown) and systemic, since can cover the whole body.

it inflammatory granulomatous benign pathology; its main characteristic is the formation of granulomas in various organs and tissues.

It affects mainly females (although this has not been confirmed by a sufficient number of studies), aged 20 to 40 years; although rare cases have been reported in children, over 50 years of age and in the elderly.

Areas and organs affected by sarcoidosis

As we have said, sarcoidosis is a multisystem disease that affects various organs and systems; it should, however, be pointed out that it can often begin in one organ and then spread to other anatomical structures. In addition, some sites of the disease are very common, while others are much more rare.

Based on the affected areas, the following can be distinguished types of sarcoidosis:

• Lungs and bronchi. This is a rare form of sarcoidosis in which granulomas are localized at the level of the lungs and bronchial tree. Symptoms are variable: there may be asymptomatic forms and forms of respiratory failure.
• upper respiratory tract. In this case (one of the rare forms of the disease), granulomas form at the level of the nose and larynx.
• Bones. All granulomas are localized mainly in small bones or at the level of the jaws. It occurs not very often - in 3-9% of patients.
• Skin. In 10-40% of patients suffering from sarcoidosis, skin lesions are noted as the only symptom, where they can sometimes coincide with bone injuries.
• The lymph nodes. It is a very common form of sarcoidosis that occurs in 70-90% of patients.
• heart contractions. Tumor-like formations at the level of the heart, localized mainly in the left ventricle, are rare (only 5-10% of patients), but always have an unfavorable prognosis and death; destruction of the parenchyma of the heart leads to heart failure.
• Liver. The liver is affected in two-thirds of people suffering from sarcoidosis, and sometimes (in 20% of cases) is accompanied by spleen sorcaidosis.
• Nervous system . In this case, they speak of neurosarcoidosis and it manifests itself only in 5% of cases. Mostly the cranial nerves are damaged, but the brain, brain stem, and spinal cord can also be affected.
• Eyes. This form affects one percent of patients. Granulomas can be localized in eyeball, and at the level of the eyelids and lacrimal glands, less often - at the level of the retina and conjunctiva.
• kidneys. This form is found in about 25% of cases, involves the entire kidney parenchyma, and leads to progressive loss of kidney function.
• Other forms. Sarcoidosis can also affect the salivary glands, mammary glands, pancreas, pituitary and hypothalamus, gastrointestinal tract, and reproductive system.

Causes of Sarcoidosis

The causes of sarcoidosis are currently unknown. It is assumed that the main role is played by autoimmune processes, but the genetic component is not excluded.

General and local symptoms of sarcoidosis

Sarcoidosis produces general and local symptoms related to the affected anatomical structure. Sometimes asymptomatic development of the disease can be observed.

Common symptoms include:

• fever, general malaise, fatigue, joint pain, lack of appetite and weight loss.
• Change in calcium metabolism caused by macrophages that form granulomas and produce a large number of hydroxycholecalciferol (active form vitamin D). This causes hypercalcemia and hypercalciuria. it general symptoms but extremely important.

Specific symptoms of sarcoidosis:

• Dermal. Sarcoidosis of the skin is characterized, first of all, by the appearance of very painful purple nodules on the skin, which are localized mainly at the level lower extremities. Other symptoms of skin lesions: maculo-papillary rash, ulcers, wounds, cicatricial injuries. You can also observe hair loss.
• cardiac. Symptoms of heart involvement include heart failure, cardiomyopathy, abnormal heartbeat (bradycardia or tachycardia). Even sudden cardiac arrest, leading to the death of the patient, is possible.
• Bone. In this case, the typical symptom is bone and joint pain. There may also be manifestations rheumatic diseases such as arthritis and acute polyarthritis.
• Nervous system. Depending on the affected area, hematochemical changes may occur, such as leukopenia, thrombocytopenia and anemia, neuropathies, hearing and vision disorders (vision and hearing loss, headaches, meningitis, paralysis or weakness in the face), convulsive seizures, dizziness, loss of smell, psychiatric disorders, epilepsy.
• Pulmonary. Pulmonary sarcoidosis may be completely asymptomatic or present with difficulty breathing, coughing with or without blood, shortness of breath, chest pain, and inability to exercise.
• Liver and spleen. In this case, enlargement of the liver (hepatomegaly) and spleen (splenomegaly) can be observed, as well as changes in hematochemical values ​​(most often an increase in the level of alkaline phosphatase).
• Ophthalmic. Most common symptom- uveitis, but burning and itching in the eyes, visual disturbances, damage to the conjunctiva, glaucoma and cataracts can also occur.
• kidneys. The most common symptom is kidney failure; glomerulonephritis, interstitial nephritis, glomerular lesions, kidney stones, and nephrocalcinosis also appear.
• The lymph nodes. There is an increase in lymph nodes, a change in elasticity (they become hard to the touch).
• Upper Airways . Symptoms include nasal polyps and granulomatosis, hoarseness, and dysphonia.
• salivary glands . In this case, xerostomia, also known as dry mouth, is found.
• Hypothalamus and pituitary gland. Metabolic disturbances occur.

Tests to Diagnose Sarcoidosis

Diagnosis of sarcoidosis carried out through various types exams in which general state patient, referring Special attention on skin lesions, the regularity of the heartbeat and breathing, the presence of pain points and the shape of the lymph nodes.

Only after that it is possible to carry out additional diagnostic studies:

• Blood analysis. Allows you to assess the levels of blood cells, thus, to identify possible leukopenia and thrombocytopenia, to evaluate the performance of organ functions. For example, in the case of liver damage, the level of alkaline phosphatase increases, while elevated creatinine indicates kidney damage.
• Radiography. Particularly well suited for detecting granulomas in the lungs.
• Magnetic resonance imaging. Particularly useful in the diagnosis of nerve sarcoidosis and possible muscle and heart involvement. Also useful in identifying granulomas in abdominal cavity: liver, spleen and pancreas.
• Six minute test. This test is done to test the patient's ability to exert effort by observing changes in the heartbeat.
• Breath test. Produced to assess the respiratory capacity of the subject.
• Hemogasanalysis. Serves to assess the ability of the lungs to regulate the level of carbon dioxide and oxygen in the blood. It is performed with the help of arterial blood sampling.
• Biopsy. It consists in obtaining a piece of tissue for subsequent examination under a microscope. It can be performed in case of sarcoidosis of the lungs, bronchi, skin, conjunctiva and lymph nodes.

Therapy, risk factors and chances of cure

Sarcoidosis is a disease that can regress spontaneously, but in some cases (about 10-15% of individuals) can become chronic.

Drug therapy for sarcoidosis

In the case of diagnosed sarcoidosis, one should contact a specialized center that is able to provide treatment in accordance with the form and aggressiveness of the disease. Therapy is usually based on corticosteroids and lasts for several months. If corticosteroids do not give an effect, then they switch to more aggressive drugs:

• Cytotoxic drugs, that is, capable of killing cells. Usually, chemotherapeutic agents are used for the treatment of autoimmune diseases, such as rheumatoid arthritis. In the case of sarcoidosis, methotrexate is used, azathioprine and cyclophosphamide.
• Immunomodulators. It has a direct effect on the body's immune response. Such drugs are usually used after transplantation or for the treatment of chronic autoimmune diseases. In the case of sarcoidosis, use pentoxifylline and infliximab.
• Antimalarial. Corticosteroids (such as chloroquine and hydroxychloroquine), which are used, in particular, in patients with high calcium levels, in sarcoidosis of the skin.

The dosage and mode of administration are determined by the doctor depending on the case.

Sarcoidosis Risk Factors and Prognosis: What You Need to Know

Risk factors for developing sarcoidosis include:

• Some categories of persons such as firefighters, foresters and workers who work in contact with silicon, aluminium, beryllium and zirconium powder are at risk due to dust inhalation. In such cases, we speak of an occupational disease.
• Living in Scandinavian countries, where, due to the climate and high humidity, numerous cases of sarcoidosis appear to be recorded.
• Cases in the family especially if one of the parents suffers from the disease.

Sarcoidosis can have an individual development, but in general we can state that:

• in 1/3 of patients, after three years from the moment of diagnosis, the disease goes into pseudo-remission, that is, it does not disappear completely, but is asymptomatic.
• in 2/3 of patients it goes into remission 10 years after the diagnosis of the first symptoms.
• In about 5% of cases, after a year of remission, the disease becomes active again.
• In 10-15% of patients with sarcoidosis, the disease can become chronic.

• Weakness
• Elevated temperature
• convulsions
• Dyspnea
• Tinnitus
• Dry cough
• Chest pain
• Loss of consciousness
• Labored breathing
• Hearing loss
• Decreased vision
• Lack of air
• The appearance of ulcers
• The appearance of nodules on the skin
• Changing the tone of the voice
• Edema of the lower extremities
• feeling hot
• blurred vision
• Skin roughness
• Purple plaques with a white center

Sarcoidosis is a disease in which some internal organs of a person are affected, as well as lymph nodes, but the lungs are most often affected by this disease. The disease is characterized by the appearance on the organs of specific granulomas, which contain healthy and modified cells. Patients with this disease are characterized by severe fatigue, fever and pain in the chest.

• Etiology
• Varieties
• Symptoms
• Complications
• Diagnostics
• Treatment
• Prevention

Diseases affect people of both sexes equally, at the age of twenty to thirty-five years. The main reasons are hereditary predisposition and viral infections. The most common, occurring in almost 90% of cases of the total incidence, is sarcoidosis of the lungs, in which neoplasms appear on the lymph nodes. The second most common is sarcoidosis of the skin and eyes. The disease is non-infectious, as a result of which it is not transmitted from an infected person to a healthy one, although this has not been fully proven in medicine.

The granulomas that appear in sarcoidosis merge and form many foci of various sizes. If you do not start treating them in time, there are only two possible outcomes - either the neoplasms will resolve on their own, or there will be a change in the structure of the organ that has undergone the disease, which can lead to irreversible health consequences. Treatment of sarcoidosis occurs only in a complex and by many means - from pharmaceuticals different groups up to folk methods and diets.

The real causes of such a disease in the human body are unknown to physicians, there is only a theory about predisposing factors that contribute to the manifestation of sarcoidosis:

• predisposition due to genetics;
• impaired immunity due to exposure to various microorganisms, bacteria, viruses;
• air pollution;
• smoking can cause disease in humans adolescence. This factor complicates the treatment of sarcoidosis;
• tuberculosis. People who have had an infection have an increased chance of being diagnosed with the disease;
• working conditions under which a person comes into contact with toxic substances;
• side effects of some medicines that a person takes long time;
• HIV infection.

The causes of sarcoidosis in the human body, however, it has been found that one or more of the above factors can lead to the formation of the disease.

Varieties

According to the place of occurrence, they distinguish:

• sarcoidosis of the lungs;
• sarcoidosis of the skin and eyes;
• sarcoidosis of the heart;
• lymph nodes;
• mixed sarcoidosis of the lungs and lymph nodes;
• sarcoidosis of the liver;
• other possible combinations of lesions internal organs.

Sarcoidosis of the lungs

Sarcoidosis of the lungs can occur in various stages:

• no changes in the organs on the x-ray;
• proliferation of lymph nodes inside the chest, but there are no pathologies in the tissues of the lungs;
• an increase in the volume of lymph nodes with a change in the structure of the lung tissue;
• deviations in the structure of lung tissue without damage to the lymph nodes;
• irreversible tissue damage and disruption of the organ.

According to the course of the disease, sarcoidosis is divided into several degrees:

• the first is the intensive occurrence and growth of granulomas. The first signs of the disease appear;
• the second - the neoplasms do not increase in number for some time, and their growth slows down, the granulomas are fully formed, the symptoms are also expressed, but the patient does not get worse;
• the third degree is characterized by a slight increase in granulomas. The first signs of dysfunction of the affected organ appear.

According to the rate of spread, the disease can be:

• chronic form - proceeds slowly and for a long time;
• delayed - treatable;
• progressive - prevention is carried out by folk remedies;
• refractory - does not lend itself to any treatment methods.

According to the expression of the disease, patients are divided into several groups:

• people who have active sarcoidosis;
• patients diagnosed for the first time;
• patients with exacerbations and remissions of symptoms;
• those who have an inactive form.

Symptoms

Since there is a wide variety of types and manifestations of sarcoidosis, its symptoms will depend on which organ has been affected by the disease.

Signs of sarcoidosis of the lungs:

• discomfort and pain in the chest area, which are exacerbated by inhalation and exhalation;
• labored breathing;
• constant lack of air, causing shortness of breath;
• strong dry cough.

Symptoms of sarcoidosis of the skin:

• the appearance on the skin of small dense nodes - at first red. After a few days, they acquire a yellowish-green hue, slightly rise above healthy skin. May go away on their own, even without treatment;
• sudden redness and intense soreness of old scars or scars;
• the appearance on the body, limbs and face of specific purple-colored plaques with a white center. They do not pass without proper treatment;
• discoloration of the skin of the face, ears and fingers in purple or red. Practically not amenable to therapy;
• violation of the structure of the skin - ulcers and roughness appear.

Manifestations of sarcoidosis of the eye:

• decreased visual acuity. If treatment is not started on time, the patient may completely lose the ability to see;
• hazy and blurry picture before the eyes.

Signs of sarcoidosis of the heart:

• often changes the rhythm of the heartbeat;
• pain and discomfort in the area of ​​the heart;
• the appearance of shortness of breath not only when performing physical activities, but also at rest;
• increase in body temperature;
• severe weakness of the body;
• swelling of the lower extremities;
• loss of consciousness.

Damage to the larynx and hearing organs:

• change in voice timbre;
• occasional lack of voice;
• labored breathing;
• hearing loss;
• persistent ringing in the ears of varying intensity.

Disorder of the nervous system:

• disturbed perception of the surrounding world by all senses, the sensation of tastes, smells, etc. disappears;
• paralysis of some nerves or complete immobilization of one half of the face;
• the appearance of seizures;
• loss of sensation in the fingertips, constant feeling of goosebumps or tingling;
• muscle weakness and pain;
• constant sleepiness;
• severe dizziness;
• migraine;
• fever (occurs rarely).

Sarcoidosis of the skin

With the defeat of other internal organs, a violation of their functioning is observed. During a pathological effect on the lymph nodes, their compaction occurs. Often the nodes of the neck, elbows, collarbone, groin and armpits are affected. Damage to bones is fraught with frequent fractures for a person.

Complications

Sarcoidosis, as an independent disease, in very rare cases becomes the reason for the death of a person or serious health problems. The danger lies in the possible development of the consequences of the disease, the most common of which are:

• mental problems;
• pulmonary fibrosis;
• complete loss of vision or its critical decrease;
• violation of the rhythm of the heartbeat;
• the formation of kidney stones;
• chronic pneumonia;
• hemorrhages;
• lung collapse.

Diagnostics

To approve an accurate diagnosis, a pulmonologist must have enough information about the disease that he receives from:

• the patient, based on complaints, the timing of the appearance of the intensity of the expression of symptoms;
• palpation of the lymph nodes;
• examination of the patient, to detect characteristic rashes;
• blood test, general and biochemical;
• Mantoux tests - carried out to determine the body's susceptibility to tuberculosis infection;
• radiography of the affected organ, lymph nodes or eyes (depending on what worries the patient);
• CT scan of internal organs;
• studying the passage of air through the lungs;
• biopsies;
• Ultrasound of the organs involved in the pathological process;
• consultation with an ophthalmologist (after an eye examination);
• endoscopy.

Treatment of the disease

Sarcoidosis is treated in several ways:

• medicines - does not apply to people who have not previously suffered from such a disease, and did not experience symptoms. The basis is glucocorticosteroids, which can be administered in the form of tablets or intravenous injections. The appointment of steroids, anti-inflammatory drugs, and drugs that strengthen the immune system is shown. In addition, special ointments are used to eliminate rashes and the use of therapeutic eye drops;
• surgical intervention - this method of therapy is addressed for sarcoidosis of the lungs or other internal organs. It is based on the removal of an infected part of an organ, a complete transplant, stopping bleeding in the gastrointestinal tract, or removing an organ. Not applicable for sarcoidosis of the eyes and lymph nodes;
• irradiation is an experimental method that is used only if other methods of therapy have not helped the patient. Only the problematic organ is irradiated;
• folk remedies;
• following a special diet.

Drugs for the treatment of sarcoidosis

Treatment of sarcoidosis with folk remedies includes the preparation of tinctures from:

• herbal collection - sage, marshmallow root, calendula flowers, plantain leaves;
• nettle, mint, chamomile, St. John's wort, calendula, celandine, coltsfoot and string;
• ginseng and pink rhodiola;
• propolis;
• lilac;
• ginger.

It is important to remember that folk remedies it is forbidden to use as the only way to treat sarcoidosis and use them without first consulting a doctor.

An important component of the treatment of sarcoidosis is assigned to a special diet, which provides for minimal use, and in some cases a complete exclusion of:

• flour products;
• Sahara;
• sweet carbonated drinks;
• salty and fried foods;
• hot spices and sauces;
• dairy and sour-milk products;
• all foods high in carbohydrates and calcium.

The diet for sarcoidosis involves eating in any quantities:

• lean meat and fish, steamed;
• porridge with butter;
• legumes;
• fruits and vegetables;
• freshly squeezed juices, compotes and fruit drinks.

Prevention

Main preventive actions for sarcoidosis include:

• limit contact with carriers of infectious diseases;
• healthy lifestyle - completely give up nicotine;
• timely treatment of diseases that cause a weakening of the immune system;
• rational diet and adherence to a non-strict diet. Eat small meals five or six times a day;
• if possible, change the environment of residence, move to an area with cleaner and non-polluted air;
• undergo a full medical examination at the clinic several times a year.

What to do?

If you think that you have Sarcoidosis and symptoms characteristic of this disease, then doctors can help you: therapist, pulmonologist.

Sarcoidosis (synonyms: Benye-Beck-Schaumann disease, benign sarcoidosis, Beck's disease) is a systemic disease of unknown etiology that affects a variety of organs and tissues, the pathomorphological basis of which is an epithelial-cell granuloma without signs of caseous necrosis.

The disease sarcoidosis was first described by the Norwegian dermatologist Beck (1899).

What causes sarcoidosis?

The causes and pathogenesis of sarcoidosis have not been elucidated. For several decades, the theory of the tuberculous origin of sarcoidosis dominated, i.e., it was believed that sarcoidosis was a special form of tuberculosis infection. In the development of the disease, the role of genetic factors is important, as indicated by the greater concordance of monozygotic twins for this disease compared to dizygotic twins, the unequal association of sarcoidosis with certain tissue compatibility antigens in populations (for example, HLA-B8, DR3), and racial differences in incidence.

The presence of family cases, the defeat of mono- and heterozygous twins confirms the genetic predisposition to granulomatous inflammation. Some authors believe that an imbalance in the immune system plays an important role in the development of sarcoidosis.

Summarizing all the views on the etiology and pathogenesis of sarcoidosis, we can conclude that it is a polyetiological syndrome.

Histopathology of Sarcoidosis

In all forms of sarcoidosis, the same type of changes are noted. In the middle and deep parts of the dermis, granulomas are found, consisting of epithelioid histiocytes with an admixture of lymphocytes, single giant cells of the Langhans type or foreign bodies. Unlike tuberculosis, caseous necrosis is usually absent. In the stage of resolution, the replacement of granulomatous islets with connective tissue is noted.

Pathomorphology of sarcoidosis

Typically, the presence of numerous similarly constructed sharply limited granulomas, consisting mainly of histiocytic elements. Necrosis is not typical. In the center of individual granulomas, giant cells of the Pirogov-Langhans type can be seen, and cells of foreign bodies are also found. In the cytoplasm of these cells, crystalloid inclusions and Schaumann asteroid bodies are often found, which, however, are not specific for sarcoidosis. The peripheral rim of lymphoid elements at this stage is small or completely absent. Characteristic of these granulomas is the presence around them of concentrically arranged collagen fibers, stained red with picrofuchsin and giving a weak PAS-positive reaction. Fugue's silver nitrate impregnation reveals reticulin fibers both around and within the granuloma. In the stage of fibrotic changes, granuloma cells are mixed with fibroblastic elements, reticulin fibers turn into collagen ones.

In Beck-Schaumann sarcoid, epithedioid islets are localized in the upper third of the dermis, closer to the epidermis, in Darier-Roussy sarcoid, mainly in the subcutaneous fat layer. Lupus pernio differs from Beck-Schaumann's sarcoid only in the presence of sharply dilated capillaries in the upper dermis. In the erythrodermic form, the infiltrate consists of small foci of epithelioid cells and a number of histiocytes and lymphocytes located around the superficial capillaries.

Sarcoidosis must be differentiated from lupus erythematosus, in which tubercles of the epithelioid structure are also observed. In the presence of caseosis and a significant number of lymphoid elements in the granulomas, it is very difficult to distinguish sarcoidosis from tuberculosis. However, in tuberculosis, the granulomatous infiltrate adheres closely to the epidermis, often destroying it, while in sarcoidosis the infiltrate is separated from the epidermis by a strip of unchanged collagen. In sarcoidosis, the granulomas typically have very few lymphoid cells, absent or very mild necrosis, and normal or atrophic epidermis. in tuberculous lupus, acanthosis is often noted, sometimes ulceration with phenomena of pseudoepitheliomatous hyperplasia. Importance has a bacteriological study. It is difficult to differentiate sarcoidosis from the tuberculoid type of leprosy, since mycobacterium leprosy peaks in only 7% of cases. However, granulomas in leprosy are located mainly around and along the cutaneous nerves. as a result they have irregular shape, in their center necrosis is often visible.

Histogenesis has not been elucidated. Currently, sarcoidosis is considered as a polyetiological disease, mainly of immunopathological origin. There is a decrease in the number of T-lymphocytes, an imbalance in their main populations; decreased T cell response to mitogens; weakened delayed-type hypersensitivity reactions; an increase in the number and hyperactivation of B-lymphocytes with nonspecific polyclonal hypergammaglobulinemia, increased level circulating antibodies, especially in the presence of erythema nodosum. At the stage of granuloma formation, T-helpers dominate in them with a relative increase in the number of circulating T-suppressors.

In the development of sarcoid granuloma of the skin, K.A. Makarova and N.A. Shapiro (1973) distinguish three stages: hyperplastic, granulomatous, and the stage of fibrous-hyalinous changes. In the hyperplastic stage, proliferation of cells of the mononuclear-macrophage system is observed, among which epithelioid elements then appear. Forming granulomas, as a rule, are still not clearly defined. multinucleated cells are usually absent at this stage of the process. It is believed that the hyperplastic and granulomatous stages are an expression of an increase in tension cellular immunity, and fibrous-hyalinous changes - morphological feature the onset of the phase of immunological exhaustion. An electron microscopic examination showed that round cells located on the periphery of the granuloma, which are considered lymphocytes, have lysosomes containing acid phosphatase and other lysosomal enzymes. They are blood monocytes, from which epithelioid cells are then formed. There is no evidence for the presence of bacterial fragments in epithelioid cells, although they contain electron dense and electron clear lysosomes, several autophagic vacuoles, and a residual body complex. Giant cells are formed from epithelioid, Schaumann bodies are formed from residual gels of lysosomes. Asteroid bodies are composed of collagen clumps with a typical (64 to 70 nm) periodicity. This is due to the fact that collagen is between the epithelioid cells at the time of the formation of giant cells from them. An immunomorphological study in some cases shows the deposition of IgM in the area of ​​the dermo-epidermal border and in the walls of blood vessels, as well as IgG in the granulomas themselves and in the surrounding dermis.

Symptoms of Sarcoidosis

Skin lesions are observed in less than 50% of patients, it can be polymorphic (such as erythema nodosum, macular erythematous foci), but more often there are tuberculous elements of various sizes, the originality of which is the basis for the allocation of such clinical options such as Beck's cutaneous sarcoid, Brock-Potrier's angiolupoid, Besnier-Tenneseon's pernicious lupus, Darier-Roussy's subcutaneous sarcoids. Beck's sarcoid may present as small tubercles, including lichenoid, coarse nodular, and diffuse plaque rashes. In rare cases, the process can occupy the entire skin (erythrodermic form of sarcoidosis). The color of the tubercles is characteristic: cyanotic, yellowish-brown, yellowish-brown spots appear during diascopy. In Besnier-Tennesson's pernicious lupus, changes are observed mainly on the skin of the nose and the cheeks adjacent to it in the form of diffuse plaque foci of a bluish-red color; with subcutaneous Darier-Ruesi sarcoid, hypodermal nodes are found, the skin over which becomes pinkish-bluish. There are rare (atypical) variants of sarcoidosis: erythematous (spotted), erythrodermic, lichenoid (clinically similar to lichen planus), prurigo-like, verrucous-papillomatous, ring-shaped, figured (circinary), erosive-ulcerative. ulcerative gangrenous, papulonecritic, scleroderma-like, post-traumatic (scarring), elephantiatic, clinically similar to the tuberculoid form of leprosy, lupoid necrobiosis, erythematous-squamous (ichthyosis- and psoriasis-like), atrophic, angiomatous, etc., which may resemble a wide variety of dermatoses, in including such peculiar clinical manifestations like eczema.

Sarcoidosis is more common in women and is manifested by a large clinical polymorphism. Skin rashes may be non-specific or specific. The latter are found at histological examination affected skin.

Depending on the manifestation of dermatosis, typical (small-nodular, large-nodular, diffuse-infiltrative, nodular sarcoid, Besnier-Tenesson's lupus pernio) and atypical forms of sarcoidosis are distinguished.

The small-nodular form is the most common and is characterized by a rash of numerous pink-red spots, which eventually turn into nodules of a densely elastic consistency of a brownish-bluish color, ranging in size from a pinhead to a pea, hemispherical in shape, with clear boundaries and a smooth surface, rising above the surrounding skin. Elements are often localized on the face and upper limbs. Diascopy reveals the smallest yellowish-brown spots (“dust particles”, or dustiness phenomenon). With regression, hyperpigmentation or superficial atrophy, telangiectasia remain in place of the nodules.

The large-nodular form of sarcoidosis is manifested by single or multiple, sharply delimited and clearly protruding above the level of the surrounding skin, hemispherical flat nodes of violet-brown or bluish-brown color, ranging in size from 10 to 20 kopeck coins or more. The elements have a dense texture, a smooth surface, sometimes covered with telangiectasias, large nodes can ulcerate. At a diascopy the phenomenon of dustiness is observed.

Diffuse-infiltrative sarcoid is more often localized on the face, rarely on the neck, scalp and is manifested by the development of plaques of a dense consistency of brownish or brownish-blue color, with a smooth surface, not sharply demarcated and slightly protruding above the skin level. The surface may be covered with a network of capillaries. Diascopy reveals a yellowish-brown color (symptom of "mote").

Angiolupoid Broca-Potry usually develops on the nose, cheeks and, extremely rarely, on other parts of the body. At the beginning of the disease, there are round, painless spots the size of a bean or a coin, of medium size, red or purple-red. Gradually, they transform into slightly protruding rounded plaques with clear boundaries, acquiring a brown or rusty tint, with a smooth surface. Rarely, on palpation, you can feel a slight induration of the spot, and with diascopy, you can see brownish dots (a symptom of "apple jelly"). Sometimes telangiectasias are visible on the surface of the elements. Plaques persist for a long time.

Pernicious lupus of Besnier-Tenneson. Small erythematous infiltrated lesions appear on the skin of the cheeks, nose, chin, dorsal surface of the fingers and forehead. As a result of the growth and fusion of elements, purple-red nodes or plaques are formed, which have fairly clear boundaries. There are dilated holes sebaceous glands and hair follicles. The deterioration of the process is noted in the cold season. In some patients, lesions of the lungs, bones, joints, enlarged lymph nodes are found. Diascopy reveals yellowish dots.

Knotty sarcoid (Derje-Roussy subcutaneous sarcoid) is clinically characterized by the formation on the skin of the trunk, thighs and lower abdomen of subcutaneous nodes ranging in size from 1 to 3 cm in diameter. They are usually few, painless, movable on palpation, and when confluent form large plaque infiltrates resembling orange peel. The skin over the nodes has a normal or slightly cyanotic color, covering the foci - dull pink.

Atypical forms of sarcoidosis. AT clinical practice meets several atypical forms: ulcerative, maculopapular, psoriasiform, ichthyosiform, scleroderma-like, scofulodermic, paillomatous, angiomatous, similar to basalioma, lupus erythematosus, etc.

In patients with sarcoidosis, damage to various organs and systems is noted. In this regard, patients are advised to do X-ray and tomography of the chest, bones and conduct an ophthalmological examination.

The general sarcoidal lesion of the body proceeds in several ways. Sarcoidosis of the skin in this case, it is manifested by the formation of tuberculous seals on the skin. The disease is often accompanied by the formation of granulomas in the lung tissues and regional lymph nodes.

What are the reasons for development?

The exact etiology of the disease has not been elucidated. In the recent past, doctors have associated sarcoidosis with manifestations of tuberculosis. To date, scientists have established that these are two completely different diseases.

Many modern studies have identified the key role of hereditary DNA mutations in the occurrence of sarcoidosis of the epidermis. In addition, to contribute to the development of pathology skin may include the following risk factors:

1. Chronic injury to the skin.
2. Ultraviolet irradiation of the epidermis.
3. Constant contact of a person with toxic substances that can be in everyday life and at work.
4. Abuse of strong alcoholic beverages and smoking.

Disease pathogenesis

The disease begins with the formation of small nodes of atypical compaction of the epithelial tissue. Subsequently, in the growth zone of the neoplasm, redness and itching of the skin is noted, which ends with the formation of an inflammatory granuloma.

Sarcoidosis of the skin is characterized by a gradual fusion of the primary elements of the lesion. At the same time, multiple sarcoidomas gradually increase in size and cause significant discomfort to a person.

Clinical picture and early signs

In medical practice, it is customary to divide the disease into two forms:

1. Small-nodular pathology - such a nodular growth of the epidermis is limited to red or cyanotic areas of the skin. The size of the neoplasm is 5-6 mm. The surface of the granuloma is smooth.
2. Coarse-grained pathology - in such cases, the seal grows to 1-2 cm in diameter. The granuloma has a reddish or brown surface.

The disease for a long time may not cause any subjective sensations in the patient. In this case, the skin completely covers the formation, which is accompanied by increased peeling of the skin and its itching.

Why is sarcoidosis of the skin dangerous?

The disease does not pose a direct danger to the patient's life. Negative consequences disease can be considered the spread of pathology to the lungs, spleen and regional lymph nodes.

Granulomatous lesions of the skin, in addition to aesthetic discomfort, can provoke chronic injury to the epidermis.

What is included in the diagnosis?

The cutaneous form of the disease is quite rare. In this regard, the diagnosis should be carried out by a qualified specialist. The procedure for examining a patient consists of the following procedures:

1. Collecting an anamnesis of the disease and clarifying family predisposition.
2. Visual inspection and palpation of the atypical area. At this stage, the doctor determines the size, consistency and degree of mobility of the granuloma.
3. General and biochemical analysis blood.
4. Mantoux test, which allows you to exclude the development of tuberculosis.
5. X-ray examination of the respiratory system and abdominal organs.
6. Biopsy. In doubtful clinical cases, doctors resort to histological and cytological examination of a small part of the pathological area.

Modern treatment

Treatment skin manifestations this disease begins with drug therapy as:

1. Taking a course of non-steroidal anti-inflammatory drugs.
2. Vitamin therapy. Doctors pay special attention to vitamins A and E.

Such therapy is symptomatic. In some cases, the patient needs to switch to an appointment hormonal drugs such as Hydrocortisone and Prednisolone.

Gain therapeutic effect medications is achieved by methods of physiotherapy. The most effective methods are:

1. Laser therapy - laser radiation stimulates the restoration of damaged epidermal tissues and enhances local microcirculation. As a result, there is a decrease in formations.
2. Electrophoresis with hormonal drugs. Doctors with the help of microcurrents carry out a point injection of hydrocortisone into the pathological area of ​​the skin.
3. Iontophoresis of anti-inflammatory drugs. Deep penetration of therapeutic agents several times enhances the effectiveness of therapy.

Folk remedies

Sarcoidosis of the skin is considered serious illness which should be treated by an experienced specialist. A positive result of treatment can be observed only if the patient stays in a medical institution.

Funds traditional medicine in such cases are used as additional method stimulation of the immune system. Activation of the protective abilities of the body is carried out with the help of tinctures and decoctions of marshmallow root, wild rose, viburnum, currant and calendula.

Complications that can result from skin sarcoidosis

The negative consequences of sarcoidosis lesions of the skin are observed in the following forms:

1. Spontaneous bleeding. Enlargement of the skin granuloma can cause traumatic injury to a nearby blood vessel, which is clinically manifested by bleeding. In most cases, this complication does not require specialized medical care.
2. The spread of the pathological process to the lungs, which can result in fibrosis. This state of the body is characterized by the replacement of lung tissue with fibrous tissue.
3. In a certain category of patients, the disease is accompanied by the formation of kidney stones due to the increased content of calcium in the blood.

Sarcoidosis of the skin turns into cancer?

Sarcoidosis of the skin is not a precancerous condition and, accordingly, cannot be the cause of the formation of a cancerous tumor.

Forecast

The cutaneous form of the disease usually has a favorable prognosis. The danger to the life of the patient is sarcoidosis of the lungs and of cardio-vascular system. Patients should be aware that most epidermal granulomas are accompanied by the spread of the pathological process to the internal organs.

The causes of deaths can be:

• spontaneous internal bleeding;
• development of acute respiratory and heart failure;
• urolithiasis disease.

In medical practice, it is customary to distinguish between such risk factors that aggravate the course of the disease:

1. Extremely slow progress of pathology when a patient is diagnosed chronic course systemic granulomatosis.
2. Involvement in the pathological process of lung, cardiovascular and nervous tissues.
3. X-ray examination states the late stage of the disease.
4. Increasing symptoms of respiratory and heart failure.

Sarcoidosis of the skin can be warned in a healthy way life, the passage of annual preventive examinations, regular exercise physical education, smoking cessation, timely diagnosis disease and treatment of symptoms of granulomatosis.