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Lecture notes, cheat sheets
Dermatovenerology. Diffuse connective tissue diseases (most important)
Directory / Lecture notes, cheat sheets Table of contents (expand) LECTURE No. 6. Diffuse connective tissue diseases 1. Lupus erythematosus Lupus erythematosus is a group of connective tissue diseases that have an autoimmune pathogenesis and affect mainly exposed areas of the skin and internal organs. Etiology and pathogenesis. The leading role in the etiology of lupus erythematosus is hereditary predisposition and, apparently, viral infection, which contribute to the development of autoimmune processes. Patients with lupus have birth defects in complement genes. Decisive factors in pathogenesis are the acceleration of the synthesis of autoantibodies and increased lymphocyte activity. Most manifestations of the disease are associated with the formation of immune complexes. In cutaneous forms of lupus erythematosus, cellular reactions are of fundamental importance. The development of the disease is facilitated by increased estrogen production, ultraviolet radiation, chronic bacterial infection, and the action of certain medications. Clinic. There are mainly cutaneous and systemic forms of lupus erythematosus. Among the cutaneous forms, discoid, disseminated and deep forms of the disease are distinguished. Discoid lupus erythematosus affects women 3 times more often than men. The skin of the face is mainly affected: nose, cheeks (especially the zygomatic region), auricles and adjacent skin, forehead, chin, lips (usually lower), scalp. Single well-defined round or oval foci of infiltrated erythema are typical. The center of the focus sinks somewhat and is covered with scales tightly attached to the surface. Removal of scales is difficult due to follicular hyperkeratosis and is accompanied by soreness. On the reverse side of the scales there are spines. In the peripheral zone of the plaque, telangiectasias and dyschromia can be expressed. Plaques are characterized by pronounced infiltration, peripheral growth and the formation of gross atrophy in the center. After the resolution of the foci on the scalp, foci of cicatricial alopecia remain. The red border of the lips is often affected: erythematous foci appear with a tendency to erode and atrophy, extending beyond the immediate red border of the lips. It is characterized by a long relapsing course with exacerbations mainly in spring and summer. Signs of a systemic lesion are extremely rare. Disseminated lupus erythematosus is characterized by erythema, infiltration, follicular hyperkeratosis, and atrophy. However, with this variant of lupus erythematosus, the severity of these symptoms is much less. For the disseminated form, the defining feature is the formation of small, poorly infiltrated erythematous plaques with a slight tendency to peripheral growth, covered with densely set scales. When scraping, soreness is revealed. There is usually a significant number of lesions. Typical localization is the skin of the face, scalp, auricles, upper chest and back, rear of the hands. After resolution of the foci, mild atrophy usually remains. With disseminated lupus erythematosus, it is necessary to conduct examinations in dynamics to exclude systemic damage. In addition to disseminated lupus erythematosus, there is a special form - the so-called subacute cutaneous form of lupus erythematosus. This form is characterized by widespread annular lesions on the skin, which, when merged, form polycyclic scaly patches along the edges on the chest, back, and extremities with hypopigmentation and telangiectasias in the central part. This variant of lupus erythematosus occupies an intermediate place between cutaneous and systemic forms: there are signs of systemic damage, but they are moderately expressed (artalgia, changes in the kidneys, polyserositis, anemia, leukopenia, thrombocytopenia). Immunological changes are characteristic of systemic lupus erythematosus (LE cells, antinuclear factor, antibodies to DNA). However, unlike systemic lupus erythematosus, the prognosis of the disease is relatively favorable. The deep form of lupus erythematosus (lupus-panniculitis) is manifested by the formation of one or two asymmetrically located subcutaneous nodes. The nodes are dense, mobile, flat, little protrude above the surrounding skin and are better detected by palpation. The face (cheek, chin, forehead) is more often affected, but other localization of the rash is also possible (on the buttocks, thighs). The skin over the knot is smooth, cherry red or normal in color. Often in patients, manifestations of a deep form of lupus erythematosus are combined with skin changes typical of discoid or disseminated forms. Systemic lupus erythematosus can develop acutely, often without skin manifestations, or subacute and chronically, when there is skin involvement. In systemic lupus erythematosus, the following manifestations are most common: fever, skin rashes, arthralgia, arthritis, damage to the serous membranes (pleurisy, much less often pericarditis), lymphadenopathy, carditis, Raynaud's syndrome, nephropathy, psychosis, anemia, leukopenia, thrombocytopenia, detection of autoantibodies is characteristic to DNA. A typical skin lesion is an edematous erythema on the face, resembling a butterfly, gradually spreading to the neck and chest. On the skin of the body and extremities - polymorphic nonspecific rashes (erythematous and hemorrhagic spots, urticarial elements, papules, sometimes vesicles and blisters with hemorrhagic contents). A characteristic manifestation of the disease are capillaritis - bluish spots on the fingertips and paronychia. The oral mucosa is frequently affected. The most typical sign is whitish grouping papules on a bright erythematous background. Often, in patients with systemic lupus erythematosus, blisters with hemorrhagic contents form on the oral mucosa. Diagnostics. The basis for diagnosing cutaneous forms of lupus erythematosus is to identify the main clinical symptoms of skin damage: erythema, infiltration, follicular hyperkeratosis and skin atrophy. In difficult cases, histological examination is recommended. Suspicion of systemic lupus erythematosus arises already in the presence of a characteristic skin lesion, which is observed in 85% of patients. However, skin changes as the first signs of the disease appear in 40% of cases. In addition to the clinical picture, the results of laboratory tests are important for establishing the diagnosis. A significant increase in ESR, hypochromic anemia, leukopenia, thrombocytopenia, dysproteinemia, the presence of LE cells, the detection of antinuclear factor and antinuclear autoantibodies are characteristic. Treatment. For all forms of lupus erythematosus, protection from insolation and cold should be used. For cutaneous forms of the disease, aminoquinoline drugs (plaquenil, chloroquine diphosphate) are used in 5-10-day cycles with breaks of 3-5 days. The course dose is about 90 tablets. Topical steroids allow you to more quickly achieve a clinical effect and reduce the severity of cicatricial atrophy in the discoid form of lupus erythematosus. At the onset of the disease and to prevent exacerbations in early spring and autumn, courses of aminoquinoline derivatives, calcium supplements and nicotinic acid derivatives are administered. The use of photoprotective products is mandatory. All patients must be registered at the dispensary. For systemic lupus erythematosus, the main therapeutic agent is systemic glucocorticosteroids. Typically, treatment begins with a loading dose of prednisolone with a gradual reduction in the daily dose after achieving a clinical effect to a maintenance dose. 2. Scleroderma Scleroderma is a polysyndromic disease manifested by progressive sclerosis of the skin, internal organs, and vascular pathology. Currently, there are limited (skin) and systemic forms of the disease. In the first case, skin sclerosis is the only manifestation of the disease. Etiology and pathogenesis. Scleroderma is a multifactorial disease, which is based on dysregulation in the synthesis of connective tissue. Hereditary predisposition to the disease is not very clearly identified. The main pathogenetic link is a violation of the function of fibroblasts, which is genetically predetermined. In this disease, fibroblasts synthesize large amounts of immature collagen. The life span of such collagen is relatively short, but the rate of its synthesis exceeds the rate of decay. The action of cytotoxic factors (exogenous and endogenous) on the endothelium leads to damage to small vessels and impaired permeability, tissue hypoxia, thickening of the vascular wall with an infiltrate consisting mainly of macrophages, and pericapillary fibrosis. Scleroderma is also characterized by autoimmune pathogenetic factors. Antibodies to various nuclear components are detected - non-histone nuclear proteins associated with chromosomes, centromeres, fibroblasts, collagen. Factors of various origin act as provoking factors. In patients with scleroderma, the regulatory effect of estradiol on metabolic processes in the connective tissue is reduced. Often scleroderma develops after abortion, pregnancy, in menopause. There is evidence of a violation of the reception to some hormones and a decrease in the regulatory effect on the connective tissue of steroids and catecholamines. Classification. There are limited (cutaneous) and systemic forms of scleroderma. Limited scleroderma is subdivided into plaque (morphea), linear and small-focal scleroderma. Systemic scleroderma is divided into acrosclerotic form (Raynaud's phenomenon, calcification, esophageal lesion, sclerodactyly, telangiectasia) and diffuse (progressive systemic sclerosis). Clinic. Limited scleroderma occurs more often in women aged 40-60 years. Skin rashes can be either single or multiple. The most common is plaque scleroderma. Scleroderma plaque is formed from a lilac spot. Such a spot, often not noticed by the patient, may exist for several months, but later its central part thickens and becomes ivory. Along the periphery of the focus, a lilac corolla remains, indicating the activity of the skin process. In this state (or slowly increasing in size), the scleroderma plaque can exist for months or even years. In the future, atrophy is formed in this area (the skin becomes dry, smooth, there is no pattern), dyschromia, telangiectasia, and hair loss are possible. In places of resolved scleroderma plaques, especially in the lumbar region, the formation of atrophoderma (areas of skin retraction due to the death of subcutaneous tissue) is possible. Linear scleroderma is a variant of plaque scleroderma. With this form of the disease, the rashes are located linearly along the length of the limb, usually monolaterally, along the neurovascular bundle or in the form of a scar from a saber strike. This form of scleroderma is characterized by a fairly deep atrophy of the skin and underlying tissues. In this case, there is a violation of microcirculation in the affected limb, which over the years leads to its hypotrophy, stunting. Scleroderma of the "saber strike" type can be combined with facial hemiatrophy. Small-focal scleroderma is more common in women who are in menopausal and post-menopausal periods, who often have a history of indications of gynecological diseases or operations. In addition to skin lesions, lesions of the vulva and perianal region are often recorded in women. Men can also develop this clinical form of scleroderma. In this case, localization of rashes on the foreskin and glans penis is typical (may lead to cicatricial phimosis and urethral stricture). The small-focal form of the disease is characterized by the appearance of white or lilac papules, which quickly transform into foci of white skin atrophy. Moderate hyperkeratosis, horny plugs in the mouths of hair follicles are often noted. Sometimes closely spaced foci merge to form shiny dry plaques, on the surface of which manifestations of follicular keratosis are visible. A sharp and shallow disorganization of the connective tissue can lead to disruption of the connection between the epidermis and the dermis, resulting in the appearance of obvious and hidden blisters. Unlike other clinical forms of localized scleroderma, small focal scleroderma is often accompanied by itching or a burning sensation of the skin. With deep plaque scleroderma, fibrosis develops around the fatty lobules in the subcutaneous adipose tissue. Clinically, this form of the disease is manifested by dense, small-tuberous nodes located deep in the skin. The skin over these nodes is not changed. In one patient, it is possible to identify a combination of various clinical forms of scleroderma. Systemic scleroderma affects women about 10 times more often than men. Diffuse scleroderma is characterized by an acute or subacute course of the disease with rapidly progressive damage to internal organs. Skin lesions are diffuse and rapidly progressive. The rapid development of edema of all or almost all of the skin is characteristic. The edema is very dense, the fossa does not form when pressed. The color of the skin is gray, with a bluish tint. Gradually, the skin is soldered to the underlying tissues. Movement becomes difficult, then facial expressions disappear. Atrophy of the subcutaneous tissue and muscles develops. Raynaud's syndrome is often recorded. Internal organs are affected: lungs (pneumofibrosis), gastrointestinal tract (esophagus is first affected), heart, kidneys. In a laboratory study, acute phase indicators (ESR, sialic acids, CRP, fibrinogen) are increased, antinuclear factor and antinuclear antibodies are detected. The acrosclerotic form of the disease is characterized by a chronic course. The disease usually begins with Raynaud's phenomenon, which for a long time may be its only manifestation. Later, swelling of the hands and feet appears, and then sclerodactyly. At this stage, telangiectasias may appear. Even later, the facial skin is involved in the process: the patient's face becomes amimic, the skin is stretched, gleams, the nose sharpens, the mouth opening narrows, and purse-like folds form around it. Thickening of the tongue and shortening of its frenulum are possible. A large number of telangiectasias on the face is characteristic. Of the internal organs, the esophagus is most affected early. In the early stages of its defeat, a decrease in peristalsis is detected only with fluoroscopy in the supine position or with kymography. Later, patients begin to be disturbed by the difficulty in passing the food bolus through the esophagus, associated with sclerosis of its walls. In second place in terms of frequency of lesions are the lungs. In the early stages, their defeat is detected by fluoroscopy in the form of an increase in the pulmonary pattern. Then pneumofibrosis develops with the corresponding clinical and radiological picture. Laboratory parameters at the onset of the disease in a chronic course change little, characteristic shifts are detected mainly in the later stages of the disease. Treatment. For limited scleroderma, pathogenetic therapy is used. Typically, such patients do not need external therapy. When the disease occurs and worsens, a course of treatment with benzylpenicillin is used (500 units 000 times a day intramuscularly, 4 million units per course). Small doses of D-penicillamine are effective (28 mg per day, course duration 50-3 months). With small-focal scleroderma, courses of unithiol are used intramuscularly. Phonophoresis of topical steroids, physiotherapy with currents are also used. The basic tool in the treatment of acrosclerotic form of systemic scleroderma is D-penicillamine. In complex therapy, drugs that improve blood microcirculation, vitamins, and physiotherapy are used. Course treatment. All patients are subject to dispensary observation. 3. Dermatomyositis Dermatomyositis (synonyms - Wagner's disease, Wagner-Unferricht-Hepp syndrome) is a multifactorial disease characterized by widespread, predominantly erythematous skin rashes and severe striated muscle involvement. The defeat of the internal organs in dermatomyositis is usually mild. The disease is characterized by a chronically progressive course, leading to disability due to muscle damage. In cases of disease that occurs without a pronounced skin lesion, the term "polymyositis" is usually used. The onset of the disease is possible at any age. Etiology and pathogenesis. Hereditary predisposition plays an important role in the development of the disease. Women get sick 3 times more often than men. Provoking factors include insolation, hypothermia, surgery, menopause, pregnancy, childbirth, trauma, and emotional stress. In the development of dermatomyositis, the increased reaction of the sensitized organism to various antigens (tumor, infectious, parasitic, chemical) is of great importance. Unlike other diffuse connective tissue diseases, autoantibodies to muscle proteins and the absence of autoantibodies to DNA are characteristic. Clinic. Manifestations on the skin of dermatomyositis are highly diverse and have low specificity. The most common is persistent erythema (recurrent, bright red or purplish-purple). Typical localization is the face, upper chest. The development of erythroderma is possible. Subjectively, patients may note soreness of the skin in the area of the rash and slight itching. Periorbital edema accompanied by erythema is typical. Of the skin manifestations, the most specific for dermatomyositis are red-violet papules on the extensor surface of the interphalangeal joints. Violet spots on the back of the hands and on the extensor surface of the elbow and knee joints are also typical. The presence of micronecrosis in the area of the nail fold and the base of the nail is characteristic. In the area of the legs, there may be an accentuated venous network and a marbled skin pattern. In most cases, there is a pronounced swelling of the skin and subcutaneous fat, which often has a soft, doughy texture. The main places of localization are the face, hands, less often the feet, lower legs, torso. In addition, the skin may have urticarial elements, ecchymosis, roseola, petechiae, vesicles, and inflammatory papules. There may be scleroderma-like lesions. Simultaneously with the skin syndrome, mucous membranes are affected (conjunctivitis, rhinitis, pharyngitis). Muscle damage usually develops gradually with the involvement of new groups. In the debut of the disease, patients are concerned about increased fatigue, muscle weakness, and myalgia. The muscles of the shoulder girdle are most often affected first: for patients, all actions associated with raising the arms are difficult. Gradually, the muscles become denser, muscle contractures develop. Usually a little later, patients notice difficulty when climbing stairs. Panmyositis gradually develops. Of great importance is the defeat of the muscles of the epiglottis, pharynx, tongue, soft palate, as this leads to dysphagia and dysphonia. Possible damage to the respiratory muscles, eye muscles. Diagnostics. The following criteria are used to diagnose dermatomyositis: 1) weakness in the proximal muscle groups for at least 1 month; 2) myalgia for at least 1 month in the absence of sensory disturbances; 3) creatinuria more than 200 mg per day; 4) a significant increase in creatine phosphokinase and lactate dehydrogenase; 5) degenerative changes in muscle fibers; 6) characteristic skin changes. The presence of four or more of these signs indicates a high probability of a diagnosis of dermatomyositis. If dermatomyositis is suspected, in addition to confirming the diagnosis, it is necessary to exclude the paraneoplastic genesis of the disease. Treatment. In the treatment of dermatomyositis, glucocorticosteroid hormone preparations are used. Author: Sitkalieva E.V. << Back: Pustular skin diseases (Staphylococcal pyoderma. Streptococcal and streptostaphylococcal pyoderma. Atypical chronic pyoderma) >> Forward: Mycosis (Keratomycosis. Dermatomycosis. Candidiasis. Deep mycoses. Pseudomycosis)
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