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

Red lupus is a chronic disease, characterized mainly by exacerbation in the summer. It was first described in 1927 by 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 appropriate to call it erythematosis.

Lupus erythematosus is uncommon. It accounts for approximately 0.25-1% of skin diseases. Women are more often affected than men. The ratio of men to women with discoid lupus erythematosus is 1:15-1:3. This figure for systemic lupus erythematosus is 1:4-1:9. It is believed that it occurs more often in women because of their delicate skin. The frequent occurrence of lupus erythematosus in women is also associated with the activity of the endocrine glands, since relapses and its severe course are often observed before menstruation or after childbirth. Lupus erythematosus most often affects adults, and it usually occurs in people exposed to environmental factors (sun rays, wind, sudden changes in temperature).

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

Causes and pathogenesis of lupus erythematosus. The origin of lupus erythematosus is unknown, but it was previously believed that the occurrence of this disease is associated with tuberculosis (historical theory).

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

Electron microscopic studies have once again confirmed the viral concept. Microtubular particles have been found in the epithelial cells of the kidneys of patients with systemic lupus erythematosus. These particles are very similar to the ribonucleoproteins of paramyxoviruses. Such particles have been found not only on the affected skin of patients, but also on the healthy skin. Despite the in-depth studies, the literature still lacks sufficiently accurate information on the viruses that cause the disease isolated from the tissue in pure form. When studying the particles using cytochemical and autoradiographic methods, the existence of phospholipids and glycoproteins, rather than nucleoproteins, has been discovered in their composition.

It has now been proven that lupus erythematosus is an autoimmune disease. The immune system plays a major role in the development of the disease. Antibodies (autoantibodies) against nuclei and their components (DNA) have been found in the blood of lupus patients. These antibodies are directed not only against nucleoproteins, but also against nucleohistone and DNA (native and denatured). The immunofluorescence reaction always detects antinuclear factor in leukocytes, tissues and skin. If systemic lupus erythematosus is suspected, this reaction can be used. In 70-80% of patients, IgG and IgM were found at the border of the epidermis and dermis. In systemic lupus erythematosus, the presence of the above immunoglobulins was found on unchanged skin. The presence of antinuclear antibodies in the immune complexes circulating in the body and located in tissues has led to the idea that lupus is a disease of immune complexes.

Changes in the activity of the autonomic and central nervous systems, as well as neuroendocrine organs, are of great importance from a pathogenetic point of view. In the initial period of the disease, many patients experience an increase in the excitation process of the nervous system, which subsequently turns into inhibition. Sometimes systemic lupus erythematosus begins with changes in the nervous system (psychosis, epilepsy, chorea, lupus meningitis, etc.).

The patients were found to have a weakened hypothalamic-pituitary-adrenal system, disease progression during pregnancy, after abortion and childbirth, increased estrogen levels, decreased testosterone levels, hyperfunction or dysfunction of the thyroid gland, which indicates a greater significance of the endocrine system in the development of the disease.

There is an opinion that lupus erythematosus has a hereditary nature. Familial cases of the disease make up 1.1-1.3%. A case of the birth of 4 children with this disease in a sick woman suffering from the discoid form of lupus erythematosus is described. Some healthy relatives of patients with lupus erythematosus showed signs characteristic of this disease - hypergammaglobulinemia, an increase in the content of total and free oxyproline in the blood serum, the presence of an antinuclear factor.

As immunogenetic studies show, antigens A11, B8, B18, B53, DR2, DR3 are more common, and these indicators largely depend on age, sex, clinical signs, course of the disease and the population under study. Some scientists, having studied the HLA system in lupus erythematosus, expressed the opinion that from the pathogenetic point of view, the ring and systemic forms of this disease are a single process. A gene predisposing to cause lupus erythematosus (HLA BD/DR) was identified, which is located between the loci on the short arm of chromosome 6.

Lupus erythematosus also develops under the influence of infectious agents (streptococci and staphylococci), various medications (hydrolysin, antibiotics, sulfonamides, vaccines, serum), environmental factors (ultraviolet irradiation, infrared rays, radiation, etc.), pathologies of internal organs (hepatitis, gastritis, impaired amino acid and vitamin metabolism).

Lupus erythematosus is treated by dermatologists and therapists, but the specialists' attitudes toward this problem are different. While most dermatologists consider acute and chronic lupus erythematosus to be one disease that occurs in different forms, therapists consider them to be independent diseases that do not depend on each other.

According to some scientists, lupus annulare is a form of lupus erythematosus, with the pathological process limited to 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 practicing dermatologists distinguish between chronic (chronic erythematosis, forming a scar), acute, 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 manifests itself in the form of discoid (or ring-shaped), disseminated lupus erythematosus, centrifugal erythema of Biett and deep form of Kaposi-Irgang lupus erythematosus.

Symptoms of lupus erythematosus. At the beginning of the disease, subjective signs are almost not observed. Most often, lupus erythematosus manifests itself in the form of a chronic ring-shaped form, the rash can appear on various areas of the skin. The rash usually appears on the face in the form of pink-reddish spots growing along the edges and tending to merge with each other. At first, the surface of the spots does not peel, but later, shaving-like scales appear that firmly attach to the skin. The spots increase in size and turn into large spots, the inflammation increases somewhat and skin infiltration develops. Over time, the infiltrate in the center of the lesion is absorbed, atrophy appears in its place, a ridge covered with small scales is observed around the lesion. During this period, peeling is gradually rejected when scratched with a nail and protrusions are visible under the peeling. When scratching or removing scales, the patient feels a slight pain, so he throws his head back. This is called the "Besnier-Meshchersky" symptom. When the scales are rejected, protrusions are observed under them (the "female heel" symptom), and deep funnel-shaped forms are formed on the skin after the scales fall out. Thus, as the disease progresses, 3 zones of the lesion are noticeable: the central zone is the cicatricial atrophy zone, the middle zone is hyperkeratotic and the peripheral zone is erythema. At the same time, telangiectasia, de- and hyperpigmentation are found in the foci of the disease. At the initial stage of the disease, the lesion on the surface of the skin looks like a butterfly. In 80% of patients, the pathological process begins with damage to the skin of the nose. Erythema can also be found on other parts of the body - on the scalp, ears, neck, abdomen, limbs. If the rash is located on the scalp, hair loss (alopecia) is observed, and on the oral mucosa - leukoplakia, erosion and wounds. Edema and cracks appear on the lips. The more infiltration is developed in the pathological focus, the more cicatricial atrophy develops in its place. You can even see ugly deep scars. Cicatricial atrophy most often develops faster on the scalp. Hair falls out on atrophied skin and sometimes lupus erythematosus can recur in this place. Skin cancer can develop on old scars that appeared after lupus erythematosus.

Depending on the clinical signs, there are several clinical forms of lupus erythematosus. If brown spots appear around the pathological focus, this is the pigment form of lupus erythematosus. In the hyperkeratotic form, small scales crumble 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 wart-like tumor. If there are bluish edematous plaques, often located in the earlobe, this is a tumor form. In the seborrheic form, the pathological process is located on the seborrheic skin and hair follicles and its surface is covered with yellow-brown greasy scales. In the mutilating form, due to the highly developed atrophy on the nose and earlobe, tissue resorption is observed. Sometimes in the foci of lupus erythematosus, you can see the formation of bubbles and blisters - this is a pemphigoid form.

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

In lupus erythematosus, the lower lip is affected in 9% of patients, the upper lip in 4.8%, and the oral mucosa in 2.2%.

In annular lupus erythematosus, the eyes are affected very rarely. Lupus ectropion, choroiditis, keratitis, blepharoconjunctivitis, and iritis have been described in scientific literature.

The disseminated form of the disease accounts for 10% of all lupus erythematosus. In the disseminated form, the rash is widespread, located like clusters on the face, scalp and upper chest and resembles discoid lupus erythematosus. However, the border of the rash is clear and non-inflamed. In addition to erythema, infiltration, hyperkeratosis and atrophy are observed in the foci. On the legs and hands, joints of the hands, one can see erythematous spots with a bluish tint. Consequently, the rash in the disseminated form gradually becomes similar to the rash in the systemic form of lupus erythematosus. However, in this form, the general condition of the patient changes somewhat, the temperature is subfebrile, the erythrocyte sedimentation rate increases, leukopenia, anemia, pain in the joints and muscles are observed. Many patients have foci of chronic infection (chronic tonsillitis, sinusitis, dental caries, etc.)

Some scientists consider the disseminated form of the disease to be intermediate between the annular and systemic forms of lupus erythematosus. The boundary between these forms of the disease is not clearly defined and there is no clear boundary between the disseminated and systemic forms. Therefore, the disseminated form can turn into the systemic form. In this case, it is very important to detect LE cells, since the body of such patients undergoes the process of nucleosis, that is, immunological changes characteristic of systemic lupus erythematosus. The disease lasts for many years. It recurs in the autumn and spring months.

Centrifugal erythema, being a superficial form of lupus erythematosus, was described by Biett (1928). This form occurs in 5% of patients. The disease begins with the appearance of a small edema on the skin of the face, limited and centrifugal erythema of a pink-red or blue-red color. The erythema resembles a butterfly and can be observed on both cheeks or only on the nose ("butterfly without wings"). There are no signs of hyperkeratosis and cicatricial atrophy on the rash or they cannot be seen due to weak development. Centrifugal erythema differs from the annular form in its clinical course. In the treatment of centrifugal erythema, a good effect is achieved by conventional methods. Sometimes, during the period of absence of sunny days, this form disappears without any treatment. However, in 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 in a short time spreads to the entire skin of the face.

Also, dilation of blood vessels is observed. Apparently, this is why some authors have identified rosacea-like and telangiectatic forms of lupus erythematosus. Clinical forms are a variant of the course of centrifugal erythema. With centrifugal erythema, rashes on the face become similar to erythematous elements in lupus erythematosus. Erythema in acute lupus erythematosus is very prominent, but its boundaries are not sharp and clear. Such a clinical picture is observed when this form of lupus erythematosus is severe and recurring.

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

According to some authors, clinical and hematological changes in patients with the 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 transitions to the systemic form, prolonged exposure to insolation, frequent sore throats, pregnancy and other factors are important. The gradual transition of the disease from one form to another (disappearance of centrifugal erythema and the onset of the systemic form) cannot be noticed. As can be seen from the above data, centrifugal erythema is considered a potentially dangerous disease, it cannot be compared with annular lupus erythematosus, and such patients should be left under long-term dispensary observation for more in-depth clinical and laboratory testing.

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

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

Depending on the clinical course, acute, subacute, and chronic forms of the disease are distinguished. The acute form of the disease is most often found among women aged 20-40. The temperature rises (39-40°C), there is pain in the joints, swelling, redness, and changes in the configuration of the finger joints. There are various rashes on the skin, which can be found all over the body and on the mucous membranes. At first, 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 significant area. Blisters and crusts appear on the reddened skin, patients are bothered by itching or burning. Sometimes the rash resembles erythema multiforme exudative or toxic-allergic dermatitis. The lips of patients are swollen, covered with bloody-purulent crusts. In some cases, the rash on the body of patients may be absent or limited. Approximately 5-10% of patients with acute systemic lupus erythematosus do not have skin rashes. During an exacerbation of the disease, the patient's health worsens, the temperature rises, joint pain, insomnia, anorexia, and nausea are observed. In severe cases of lupus erythematosus, the patient lies in bed, cannot get up, loses weight, becomes weak and exhausted. During this period, LE cells are detected in the blood during laboratory testing, which is very important when making a diagnosis. Subacute systemic lupus erythematosus is less common; it can develop independently or after annular chronic lupus erythematosus. There are foci of the disease on closed areas of the body, a change in the general condition, joint pain, and an increase in temperature. The rash on the skin resembles erysipelas. Along with limited edema, hyperemia and peeling of the skin are observed. Erythematous-papular rashes remain on the skin for a long time, and subsequently the liver and other internal organs may be involved in the process. Depending on the organ and system in which the pathological process manifests itself, there are skin-articular, renal, pulmonary, neurological, cardiovascular, gastrointestinal, hepatic and hematological forms of lupus erythematosus.

In the knee-joint form of lupus erythematosus, in addition to skin rashes, joint damage is also observed, occurring in the form of arthralgia and arthritis. Sometimes, signs of joint damage are noted before the development of skin symptoms of the disease. Small joints are affected first, then large ones. Deformation of blood vessels is observed in 10% of patients. Muscle damage is observed in 25-50% of patients. Muscle damage in lupus erythematosus is difficult to distinguish from myalgia and myositis in dermatomyositis.

In systemic lupus erythematosus, kidney damage (lupus nephritis) is observed. 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 treatment or activation, protein, erythrocytes, leukocytes and cylinders appear in the urine. Lupus nephritis most often manifests itself as focal glomerulonephritis, nephrosis, nephrosonephritis, focal interstitial nephritis and does not differ in clinical picture from renal pathology caused by other factors. In severe cases of the disease, such signs as hypertension, general edema, uremia and renal failure, etc. appear.

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

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 ESR. Sometimes the spleen and lymph nodes enlarge, hair falls out, the skin becomes dry, and the nails become brittle.

Lupus erythematosus, occurring with rashes similar to erythema multiforme exudative, has been described. The combination of these diseases was first noted in 1963 by Rovel (Rovel's syndrome). While some dermatologists consider Rovel's syndrome to be one of the forms of lupus erythematosus, others classify it as two diseases developing simultaneously.

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

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

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

Treatment of lupus erythematosus. Treatment is established depending on the form of the disease. In case of annular lupus erythematosus, antipyretic drugs (resochin, hingamin, plaquenil, delagil) are prescribed at 0.25 g 2 times a day for 5-10 days. Then a break of 3-5 days is taken. These drugs accelerate the work of the adrenal glands, affect the metabolism in the connective tissue, resulting in photodesensitization. Taking presocil, containing 0.04 g of resohip, 0.00075 g of prednisolop and 0.22 aspirin, 6 times a day, will give a good effect. Conducting vitamin therapy (group B, ascorbic, nicotinic acids, etc.) increases the effectiveness of treatment.

In systemic lupus erythematosus, systemic glucocorticoids are prescribed together with antipyretic 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, treatment with vitamins (B1, B9, B6, B12, B15, PP, C) is advisable, since they increase the effect of steroid hormones and antipyretic drugs. A good effect is observed with the use of aromatic retinoids (acitretin at a dose of 1 mg / kg).

Corticosteroid creams and ointments are used externally.

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