Systemic lupus erythematosus is a chronic systemic disease with the most pronounced manifestations on the skin; the etiology of lupus erythematosus is not known, but its pathogenesis is associated with a violation of autoimmune processes, as a result of which antibodies to healthy cells of the body are produced. Middle-aged women are more susceptible to the disease. The incidence of lupus erythematosus is not high – 2-3 cases per thousand people of the population. Treatment and diagnosis are carried out jointly by a rheumatologist and a dermatologist. The diagnosis of SLE is established on the basis of typical clinical signs, the results of laboratory tests.
Causes
The exact etiology of lupus erythematosus has not been established, but most patients have antibodies to the Epstein-Barr virus, which confirms the possible viral nature of the disease. The peculiarities of the body, as a result of which autoantibodies are produced, are also observed in almost all patients.
The hormonal nature of lupus erythematosus has not been confirmed, but hormonal disorders worsen the course of the disease, although they cannot provoke its occurrence. Women with diagnosed lupus erythematosus are not recommended to take oral contraceptives. In people with a genetic predisposition and identical twins, the incidence of lupus erythematosus is higher than in other groups.
The pathogenesis of systemic lupus erythematosus is based on a violation of immunoregulation, when the protein components of the cell act as autoantigens, primarily DNA, and as a result of adhesion, even those cells that were initially free of immune complexes become targets.
Systemic lupus erythematosus symptoms
In lupus erythematosus, connective tissue, skin and epithelium are affected. An important diagnostic sign is a symmetrical lesion of large joints, and if joint deformation occurs, it is due to the involvement of ligaments and tendons, and not due to erosive lesions. Myalgia, pleurisy, pneumonitis are observed.
But the most striking symptoms of lupus erythematosus are noted on the skin and it is for these manifestations that the diagnosis is made in the first place.
In the initial stages of the disease, lupus erythematosus is characterized by a continuous course with periodic remissions, but almost always passes into a systemic form. More often there is erythematous dermatitis on the face of the butterfly type – erythema on the cheeks, cheekbones and necessarily on the back of the nose.
Hypersensitivity to solar radiation appears – photodermatoses are usually rounded in shape, have a multiple character. In lupus erythematosus, a feature of photodermatoses is the presence of a hyperemic corolla, a site of atrophy in the center and depigmentation of the affected area. The bran-like scales that cover the surface of the erythema are tightly soldered to the skin and attempts to separate them are very painful. At the stage of atrophy of the affected skin, the formation of a smooth, delicate alabaster-white surface is observed, which gradually replaces the erythematous areas, starting from the middle and moving to the periphery.
In some patients with lupus erythematosus, lesions spread to the scalp, causing complete or partial alopecia. If the lesions affect the red border of the lips and the mucous membrane of the mouth, then the lesions are bluish-red dense plaques, sometimes with bran-like scales on top, their contours have clear boundaries, plaques are prone to ulceration and cause pain during eating.
Lupus erythematosus has a seasonal course, and in the autumn-summer periods the skin condition deteriorates sharply due to more intense exposure to solar radiation.
In the subacute course of lupus erythematosus, psoriasis-like foci are observed throughout the body, telangiectasia is pronounced, a reticulated livedio (tree-like pattern) appears on the skin of the lower extremities. Generalized or focal alopecia, urticaria and pruritus are observed in all patients with systemic lupus erythematosus.
In all organs where there is connective tissue, pathological changes begin over time. With lupus erythematosus, all the membranes of the heart, the pelvis of the kidneys, the gastrointestinal tract and the central nervous system are affected.
If, in addition to skin manifestations, patients suffer from periodic headaches, joint pains without connection with injuries and weather conditions, there are violations from the heart and kidneys, then already on the basis of the survey, it is possible to assume deeper and systemic disorders and examine the patient for the presence of lupus erythematosus. A sharp change of mood from a euphoric state to a state of aggression is also a characteristic manifestation of lupus erythematosus.
In elderly patients with lupus erythematosus, skin manifestations, renal and arthralgic syndromes are less pronounced, but Sjogren’s syndrome is more often observed – this is an autoimmune lesion of connective tissue, manifested by hyposecretion of the salivary glands, dryness and pain in the eyes, photophobia.
Children with neonatal lupus erythematosus, born from sick mothers, have erythematous rash and anemia already in infancy, therefore, a differential diagnosis with atopic dermatitis should be carried out.
Diagnostics
If systemic lupus erythematosus is suspected, the patient is referred for consultation by a rheumatologist and a dermatologist. Lupus erythematosus is diagnosed by the presence of manifestations in each symptomatic group. Criteria for diagnosis from the skin: butterfly-shaped erythema, photodermatitis, discoid rash; from the joints: symmetrical joint damage, arthralgia, “pearl bracelets” syndrome on the wrists due to deformation of the ligamentous apparatus; from the internal organs: serositis of various localization, persistent proteinuria and cylindruria in urine analysis; from the central nervous system: convulsions, chorea, psychosis and mood changes; from the hematopoietic function, lupus erythematosus is manifested by leukopenia, thrombocytopenia, lymphopenia.
Wasserman’s reaction can be false positive, as well as other serological studies, which sometimes leads to the appointment of inadequate treatment. With the development of pneumonia, a lung X-ray is performed, and if pleurisy is suspected, a pleural puncture is performed. To diagnose the condition of the heart – ECG and echocardiography.
Systemic lupus erythematosus treatment
As a rule, the initial treatment of lupus erythematosus is inadequate, as erroneous diagnoses of photodermatoses, eczema, seborrhea and syphilis are made. And only in the absence of the effectiveness of the prescribed therapy, additional examinations are carried out, during which lupus erythematosus is diagnosed. It is impossible to achieve a complete cure from this disease, but timely and correctly selected therapy makes it possible to improve the patient’s quality of life and avoid disability.
Patients with lupus erythematosus should avoid direct sunlight, wear clothes covering the whole body, and apply creams with a high protective filter from ultraviolet light to the exposed areas. Corticosteroid ointments are applied to the affected areas of the skin, since the use of non-hormonal drugs does not bring effect. Treatment should be carried out intermittently so that hormone-dependent dermatitis does not develop.
In uncomplicated forms of lupus erythematosus, nonsteroidal anti-inflammatory drugs are prescribed to eliminate pain in the muscles and joints, but aspirin should be taken with caution, since it slows down the process of blood clotting. It is mandatory to take glucocorticosteroids, while the doses of drugs are selected in such a way that, while minimizing side effects, protect the internal organs from damage.
The method when stem cells are taken from a patient, and then immunosuppressive therapy is performed, after which stem cells are reintroduced to restore the immune system, is effective even in severe and hopeless forms of lupus erythematosus. With such therapy, autoimmune aggression in most cases stops, and the condition of the patient with lupus erythematosus improves.
A healthy lifestyle, abstinence from alcohol and smoking, adequate physical activity, balanced nutrition and psychological comfort allow patients with lupus erythematosus to control their condition and prevent disability.
