Skin changes with lupus erythematosus: causes, symptoms, diagnosis, treatment

Red lupus is a chronic disease characterized mainly by exacerbation in the summer. For the first time in 1927 he described P. Raycr under the name "Flux scbacc". A Cazenava (1951) called this disease "red lupus." However, according to many dermatologists, this name does not reflect the essence of the disease and it is expedient to call it erythematosis.

Red lupus occurs infrequently. It is approximately 0.25-1% of skin diseases. Women are sick more often than men. The ratio of men and women with discoid lupus erythematosus is 1: 15-1: 3. This indicator in systemic lupus erythematosus is 1: 4-1: 9. There is an opinion that because of the delicate skin of women, she meets them more often. The frequent occurrence of lupus erythematosus in women is also associated with the activity of endocrine glands, since relapses and severe course of it are often observed before menstruation or after childbirth. Lupus erythematosus often affects adults, and it, as a rule, occurs in people exposed to environmental factors (sunlight, wind, sudden temperature changes).

The disease can occur on all continents, but is more common in countries with high humidity (Scandinavia, England, northern Germany, Greece, Japan, etc.). Red lupus, despite increased insolation, in tropical countries (Brazil, Egypt, Syria) is rare. White people in comparison with blacks are sick several times more often.

Causes and pathogenesis of lupus erythematosus. The origin of lupus erythematosus is not known, but previously believed that the onset of this disease is associated with tuberculosis (historical theory).

The detection of circulating antibodies against Epstein-Barr and herpes, oncovirus in leukocytes and liver confirm the viral origin of the disease.

Electron microscopic studies again confirmed the viral concept. In the epithelial cells of the kidneys of patients with systemic lupus erythematosus, microtubular particles were detected. These particles are very similar to the ribonucleoproteins of paramyxoviruses. Also, such particles are found not only on the affected, but also on healthy skin of patients. Despite of the carried out deep researches, in the literature till now there is no enough exact information about the viruses which have caused a disease, isolated from a tissue in the pure state. In the study of particles by cytochemical and autoradiographic methods, the existence of non-nucleoproteins and phospholipids and glycoproteins was observed in their composition.

It has now been proven that lupus erythematosus is an autoimmune disease. In the emergence of the disease, the immune system is of great importance. In the blood of patients with lupus erythematosus, antibodies (auto-antibodies) were detected against nuclei and its constituents (DNA). These antibodies are directed not only against nucleoproteins, but also against nucleogiston and DNA (native and denatured ones). Through the immunofluorescence reaction in leukocytes, tissues and skin, an antinuclear factor is always found. If there is a suspicion of systemic lupus erythematosus, this reaction can be used. In 70 80% of patients at the border of the epidermis and dermis, IgG and IgM were detected. With systemic lupus erythematosus on unchanged skin, the presence of the above immunoglobulins was detected. The presence of antinuclear antibodies in the immune complexes circulating in the body and in tissues made it possible to express the idea that lupus erythematosus is a disease of immune complexes.

Changes occurring in the activity of the autonomic and central nervous system, as well as neuroendocrinal organs, are of great importance from the pathogenetic point of view. In the initial period of the disease in many patients, the process of excitation of the nervous system is intensified, and in the subsequent it goes into inhibition. Sometimes systemic lupus erythematosus begins with changes in the nervous system (psychosis, epilepsy, chorea, lupus meningitis, etc.).

In patients, weakened the activity of the hypothalamic-pituitary-adrenal system, the progression of the disease during pregnancy, after abortion and childbirth, increased estrogen content, decreased testosterone, hyperfunction or thyroid dysfunction, which indicates the greater importance of the endocrine system in the onset of the disease.

There is an opinion that lupus erythematosus is hereditary in nature. Family cases of the disease are 1.1-1.3%. A case of birth in a sick woman with a discoid form of lupus erythematosus, 4 children with this disease is described. In some healthy relatives of patients with lupus erythematosus, signs characteristic of this disease - hypergammaglobulinemia, elevation in the serum of the content of total and free hydroxyproline, the presence of antinuclear factor.

Immunogenetic studies show that the antigens A11, B8, B18, B53, DR2, DR3 occur more frequently, and these indicators largely depend on the age, gender, clinical signs, incidence and the studied population. Some scientists, having studied the HLA system in case of lupus erythematosus, expressed the opinion that, from the first pathogenetic point of view, the ring-shaped and systemic forms of this disease are a single process. A gene has been identified, predisposed to cause red lupus erythematosus (HLA BD / DR), which is located between the loci on the short arm of the 6th chromosome.

Lupus erythematosus also develops under the influence of infectious agents (streptococci and staphylococci), various drugs (hydrolysine, antibiotics, sulfonamides, vaccine, serum), environmental factors (ultraviolet irradiation, infrared rays, radiation, etc.), pathology of internal organs (hepatitis, gastritis, impaired metabolism of amino acids and vitamins).

Dermatologists and therapists are engaged in lupus erythematosus, but the attitude of experts to this problem is different. If the majority of dermatologists consider acute and chronic lupus erythematosus one disease, proceeding in different forms, then therapists consider them as independent diseases that do not depend on each other.

According to some scientists, ring-shaped lupus is one of the forms of lupus erythematosus, while the pathological process is limited to the location in the skin. Systemic lupus is also a form of lupus erythematosus.

However, the pathological process that begins in the skin gradually spreads to the internal organs and the musculoskeletal system.

Classification of lupus erythematosus. There is no generally accepted classification of lupus erythematosus. Most practical dermatologists distinguish between chronic (chronic erythematosis, forming a scar), osseous, or systemic (acute erythematosis), and subacute forms of lupus erythematosus.

In the systemic form of the disease, internal organs are damaged along with the skin. The clinical picture of the chronic form is manifested in the form of discoid (or annular), disseminated red lupus erythema, centripetal erythema and deep form of lupus erythematosus Kaposhi-Irganga.

Symptoms of lupus erythematosus. At the beginning of the disease, subjective symptoms are almost not observed. Most often, lupus erythematosus appears as a chronic annular form, the rash may appear on different parts of the skin. The rash usually appears on the face in the form of spots of pink-reddish color, growing at the edges and prone to fusion with each other. At first, the surface of the spots does not peel off, but subsequently there are chip-shaped scales that firmly attach to the skin. The spots increase and turn into large spots, the inflammation increases somewhat and skin infiltration develops. Over time, the infiltrate in the center of the focus dissolves, in its place appears atrophy, around the hearth there is a roller, covered with small scales. During this period, the scaling gradually, when scratching with a fingernail, is rejected and under the scaling visible protrusions. When scratching or removing scales, the patient feels a slight pain, so he throws his head back. This is called the Bénye-Meshchersky symptom. When the scales are rejected, protrusions (a symptom of the "female heel") are observed under them, and deep funnel forms are formed on the skin after the scales fall out. Thus, with the progression of the disease, three zones of the lesion are evident: the central zone is the zone of cicatricial atrophy, the middle is the hyperkeratotic zone and the peripheral one is the erythema. At the same time, telangiectasia, de- and hyperpigmentation occur in the foci of the disease. In the initial stage of the disease on the surface of the skin, the lesion focuses like a butterfly. In 80% of patients, the pathological process begins with nasal skin damage. Erythema can also occur on other parts of the body - on the scalp, ears, neck, abdomen, extremities. If the rash is located on the scalp, then hair loss (alopecia), and on the mucous membrane of the mouth - leukoplakia, erosion and wounds. On the lips there are swelling and cracks. The more infiltration is developed in the pathological focus, the more atrophic atrophy develops in this place. You can even watch ugly deep scars. Cicatricial atrophy most often develops faster on the scalp. On the atrophied skin hair falls out and sometimes on this place the lupus erythematosus can recur. Possible development of skin cancer on old scars that appeared after lupus erythematosus.

Depending on the clinical features, there are several clinical forms of lupus erythematosus. If spots of brown, brown color appear around the pathological focus, this is the pigmented form of lupus erythematosus. When hyperkeratotic form small scales are deposited, like lime and hyperkeratosis is observed. As a result of the growth of the papillary layer of the dermis and the development of hyperkeratosis, the pathological process resembles a warty tumor. If there are bluish edematous plaques, often located in the earlobe, is a tumor form. With seborrheic form, the pathological process is located on seborrheic skin and hair follicles and its surface is covered with yellow-brown fatty scales. With the mutating form due to strongly developed atrophy on the nose and earlobe, tissue resorption is observed. Sometimes in the foci of red lupus you can see the formation of bubbles and bubbles - this is a pemphigoid firm.

Incorrect and irrational treatment of lupus erythematosus can lead to the development of lupus carcinoma.

With red lupus the lower lip is affected in 9% of patients, the upper lip - in 4.8% and the mucous membrane of the mouth - in 2.2%.

With ring-shaped red lupus, the eyes are very rarely affected. The scientific literature describes the lupus ectopion, choroiditis, keratitis, blepharoconjunctivitis, iritis.

The disseminated form of the disease is 10% of the total lupus erythematosus. With disseminated form, the rash, which is spread multitudinously, is located like a cluster on the face, the scalp and upper chest and resembles discoid lupus erythematosus. However, the rash boundary is clear and non-inflamed. In the foci, in addition to erythema, infiltration, hyperkeratosis and atrophy are observed. On the legs and hands, the joints of the hands you can see having a bluish shade of erythematous spots. Consequently, the rash in disseminated form gradually becomes like a rash in the systemic form of lupus erythematosus. However, with this form the general condition of the patient changes somewhat, the temperature is subfebrile, the erythrocyte sedimentation rate increases, leukopenia, anemia, joint and muscle pains are observed. In many patients, foci of chronic infection are identified (chronic tonsillitis, sinusitis, dental caries, etc.)

Some scientists consider the disseminated form of the disease intermediate between the ring-shaped and systemic forms of lupus erythematosus. The border between these forms of the disease is not clearly expressed and there is no clear boundary between disseminated and systemic forms. Therefore, the disseminated form can pass into the systemic form. In this case, it is very important to detect LE-cells, since in the body of such patients the nucleoside process proceeds, that is, the immunological changes inherent in systemic lupus erythematosus. The disease lasts for many years. It recovers in the autumn and spring months.

Centrifugal erythema, being a superficial form of lupus erythematosus, is described by Biett (1928). This form occurs in 5% of patients. The disease begins with the appearance on the skin of the face of a small edema, limited and centrifugal erythema pink-red or blue-red. Erythema resembles a butterfly, can be observed on both cheeks or only on the nose ("butterfly without wings"). On the rashes there are no signs of hyperkeratosis and cicatricial atrophy or because of poor development they can not be seen. In its clinical course, the centrifugal erythema differs from the annular form. In the treatment of centrifugal erythema, a good effect is achieved by conventional methods. Sometimes in the absence of sunny days this form disappears without any treatment. However, in the autumn and winter, under the influence of cold, wind, and in summer and spring, under the influence of the sun, it very quickly recurs and spreads over the entire skin of the face in a short time.

The expansion of blood vessels is also observed. Apparently, therefore, some authors identified rosace-like and telangiectatic forms of lupus erythematosus. Clinical forms are a variant of the flow of centrifugal erythema. With centrifugal erythema, the eruptions on the face become similar to the erythematous elements in case of lupus erythematosus. Erythema in acute lupus erythematosus very strongly protrude, but their boundaries are blurred and indistinct. This clinical picture is observed when this form of lupus erythematosus is severe and recurs.

In subacute and chronic forms of systemic lupus erythema symmetrically located almost does not differ from centrifugal erythema. Therefore, the question arises whether centrifugal erythema is a sign of systemic lupus erythematosus, which proceeds chronically. However, the systemic course of the disease is not determined by rashes on the skin, but by damage to internal organs, blood and other systems.

According to some authors, the clinical and hematological changes in patients with disseminated form were very similar to those in patients with systemic lupus erythematosus. However, these changes in centrifugal erythema are less pronounced. When the disease is transferred to the systemic form, it is important to stay for a long time under insolation, frequent angina, pregnancy and other factors. Gradual transition of the disease from one form to another (the disappearance of centrifugal erythema and the beginning of the systemic form) can not be seen. As can be seen from the above data, centrifugal erythema is considered a potentially dangerous disease, it can not be compared with ring-shaped lupus erythematosus, and such patients should be left under long-term follow-up for a deeper clinical and laboratory study.

With the deep form of Kaposi-Irgang red lupus in the subcutaneous tissue, deeply located nodes appear, their deepened center is atrophied, they are most often found on the head, shoulders and hands. Sometimes after the nodes appear ulcers. With this form of the disease, in addition to nodes, pathological foci characteristic of lupus erythematosus are also observed. Of the subjective signs, the most troubling is the itching. Histopathology. In the chronic form of lupus erythematosus in the epidermis follicular hyperkeratosis, atrophy of basal layer cells, in the dermis - proliferation of plasma cells, lymphocytes, histiocytes, edema.

Systemic lupus erythematosus occurs suddenly or as a result of the progression of chronic erythematosis, it is difficult. Under the influence of various stress conditions, infections, ultraviolet rays, chronic or disseminated lupus erythematosus can be transformed into a systemic form.

Depending on the clinical course distinguish acute, subacute, chronic form of the disease. The acute form of the disease is most often found among women 20-40 years old. The temperature rises (39-40 ° C), pains in the joints, edema, redness, changes in the configuration of the joints of the fingers. On the skin there are various eruptions that can occur throughout the body and on the mucous membranes. Initially, the surface of the erythematous rash is covered with scales, they gradually spread to other parts of the body or, merging with each other, occupy a considerable area. On the reddened skin appear blisters and crusts, patients are concerned about itching or burning. Sometimes the rash resembles the multiform ekssidative erythema or toxic-allergic dermatitis. The lips of the patients are swollen, covered with blood-purulent crusts. In some cases, the patient's body may be absent or restricted. Approximately 5-10% of patients with acute systemic lupus erythematosus do not have rashes on the skin. When the illness worsens, the state of health worsens, the temperature rises, joint pains, insomnia, anorexia, nausea. In severe lupus erythematosus, the patient lies in bed, he can not get up, lose weight, becomes impotent and exhausted. During this period, in the laboratory study, LE cells are found in the blood, which is very important when making a diagnosis. Subacute form of systemic lupus erythematosus is less common, it can develop independently or after annular chronic lupus erythematosus. The appearance of foci of the disease is observed in the closed parts of the body, changes in the general condition, the appearance of pain in the joints, and a rise in temperature. The rash on the skin resembles a face. Together with limited edema, there is hyperemia and peeling of the skin. On the skin, erythematous-papular eruptions persist for a long time, and later the liver and other internal organs may be involved in the process. Depending on the organ and system in which the pathological process manifests, the skin-articular, renal, pulmonary, neurological, cardiovascular, gastrointestinal, hepatic and hematological forms of lupus erythematosus are distinguished.

In the knee-joint form of lupus erythematosus, in addition to skin rashes, damage to the joints is noted, taking place in the form of arthralgia and arthritis. Sometimes, before the development of skin symptoms of the disease, there are signs of joint damage. First small, then large joints are affected. Vascular deformities are observed in 10% of patients. In 25-50% of patients, muscles are damaged. The defeat of muscles with lupus erythematosus is difficult to distinguish oi myalgia and myositis with dermatomyositis.

In systemic lupus erythematosus, kidney damage is observed (lupus-nephritis). Clinical signs of lupus-nephritis depend on the degree of activity of the pathological process. In the initial period of the disease, the kidneys are usually not involved in the pathological process. Subsequently, without carrying out therapeutic procedures or activating, protein appears in the urine, red blood cells, leukocytes and cylinders. Lupus nephritis often manifests itself in the form of focal glomerulonephritis, nephrosis, nephrosis nephritis, focal interstitial nephritis and does not differ in clinical picture from renal pathology caused by other factors. In severe illness, such symptoms as hypertension, general edema, uremia and kidney function deficiency, etc.,

In systemic lupus erythematosus, the cardiovascular system is often involved in the pathological process. Endocarditis, pericarditis, myocarditis are observed, and in severe cases, signs of pancarditis. Some patients develop Limball-Sachs disease (or Limball-Sachs endocarditis). In this case, along with endocarditis, there are clinical signs such as myocarditis, polyserositis, hepatitis, splenomegaly and neuritis. Due to changes in the walls of the vessels appears Raynaud's syndrome.

There are also changes in the central nervous system (polyneuritis, myeloradiculoneuritis, encephalitis, myelitis, encephaloradiculitis, severe leptomeningitis, acute cerebral edema), lungs (interstitial pneumonia, pleurisy), gastrointestinal tract (abdominal syndrome), liver (lupus-hepatitis), leukopenia, thrombocytopenia, hemolytic anemia, lymphopenia, increased ROE. Sometimes the spleen and lymph nodes increase, hair falls out, the skin becomes dry, the nails are brittle.

Lupus erythematosus is described, which proceeds with eruptions similar to multiforme exudative erythema. For the first time the combination of these diseases was noted in 1963 by Rovel (Rowel's syndrome). If some dermatologists consider Rowel's syndrome to be one of the forms of lupus erythematosus, others treat it as two diseases developing simultaneously.

Identification of the signs of the disease (erythema, follicular hyperkeratosis, cicatricial atrophy), changes in blood (leukopenia, lymphopenia, anemia, thrombocytopenia, gammaglobulinemia, increased ROE), antibodies opposite to LE cells and nucleus is of great importance in determining the diagnosis.

Histopathology. With systemic lupus erythematosus in collagen fibers of the skin and internal organs, fibrinous degeneration is observed, and in the dermis - an infiltrate consisting of leukocytes.

Differential diagnosis. In the initial period of ring-shaped or disseminated lupus erythematosus, it should be distinguished from psoriasis, rosacea, tuberculosis, lupus erythematosus, sarcoidosis, erythematous form of pemphigus and other diseases.

Treatment of lupus erythematosus. Treatment is established depending on the form of the disease. With annular lupus erythematosus, antiplatelet agents (resorchin, hingamin, plakvenil, delagil) are administered 0.25 g twice daily for 5-10 days. Why is a break 3-5 days. These drugs accelerate the work of the adrenal glands, affect the metabolism in the connective tissue, resulting in photosensitization. The intake of presocil containing 0.04 g of a resichip, 0.00075 g of prednisol and 0.22 aspirin, 6 times a day, will give a good effect. Carrying out vitamin therapy (group B, ascorbic, nicotinic acid, etc.) increases the effectiveness of treatment.

With systemic lupus erythematosus, systemic glucocorticoids are prescribed together with antiplatelet drugs, which gives a good effect. The dose of steroid drugs is prescribed depending on the clinical course of the disease and the patient's condition (on average 60-70 mg of prednisolone is recommended). At the same time, it is advisable to treat vitamins (B1, B9, B6, B12, B15, PP, C), as they increase the effect of steroid hormones and antiplatelet agents. A good effect is observed when using aromatic retinoids (acitretin in a dose of 1 mg / kg).

External apply corticosteroid creams and ointments.

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