about the project
Medical news
For authors
Licensed books on medicine
<< Previous Next >>

Topic number 6. Diffuse diseases of the connective tissue. Bubble Dermatoses

Lupus erythematosus is a serious disease that affects connective tissue and blood vessels. Among the factors that provoke the appearance or exacerbation of lupus erythematosus, first of all, hypersensitivity to insolation and meteorological influences is noted. In some cases, in women, the disease occurs after pregnancy and childbirth. Distinguish between chronic (limited and disseminated) and systemic lupus erythematosus. Systemic lupus erythematosus is an autoimmune disease in which various autoantibodies and immune complexes are formed in the patient’s body, which are deposited in the walls of small vessels and under the basement membrane of the epidermis.

Chronic lupus erythematosus. Etiopathogenesis. An important role in the formation of the disease is played by chronic focal infection, the intake of certain medications (antibiotics, sulfonamides, serums, vaccines), insolation, burns, frostbite. Distinguish between discoid and disseminated forms.

Discoid lupus erythematosus is characterized by three objective signs: erythema with mild infiltration, follicular hyperkeratosis, and cicatricial atrophy. Mostly the skin of open areas of the body is affected: face, scalp, chest, rear of the hands. At the height of its development, the focus has the form of a disk consisting of three zones: in the center, skin atrophy, surrounded by a zone of follicular hyperkeratosis, and on the periphery, an erythematous corolla. A typical form of the focus is a “butterfly”, localized on the back of the nose and skin of the cheeks. Sometimes the mucous membranes of the cheeks, gums, palate, lips can be involved. Discoid lupus erythematosus is characterized by the Benje-Meshchersky symptom (pain when removing scales in the area of ​​the outbreak) and the symptom of “ladies' heel” - a spike on the surface of the removed scale. Varieties of discoid lupus erythematosus are papillomatous, telangiectatic, pigmented, tumor-like forms.

Disseminated lupus erythematosus is characterized by multiple erythematous-squamous foci. Some of them are identical to the foci of discoid lupus erythematosus, others without infiltration and hyperkeratosis. The foci do not tend to peripheral growth, reaching the size of a 5-kopek coin stop growing. Localization of elements - face, scalp, auricles, trunk, limbs. On the scalp, foci are represented by infiltrated spots with hyperkeratosis, after which cicatricial atrophy and persistent baldness remain. On the red border of the lips weakly infiltrated red-violet spots are formed, covered with hard to remove flakes. Later, atrophy forms in the central part. On the mucous membrane of the cheeks and lips erosion is possible. Disseminated lupus erythematosus is usually accompanied by common symptoms: subfebrile condition, weakness, and malaise.

In addition, there are superficial (centrifugal erythema Bietta) and deep (Kaposi-Irgang form) forms of lupus erythematosus. With a superficial form, there is no hyperkeratosis and skin atrophy. The foci are single or small, located asymmetrically on the face. They are represented by a round spot with clear boundaries, slightly swollen, erythematous with a bluish tinge. Can transform into systemic lupus erythematosus. With a deep form, deep nodes or flat infiltrates form on their own or along with typical rashes. The skin color above them is not first changed, then it turns cyanotic red. As the foci resolve, deep impressions remain.

The differential diagnosis is carried out with dermatomyositis, psoriasis, seborrheic dermatitis, smooth skin dermatophytosis, lupus erythematosus tuberculosis, tubercular syphilis, photodermatitis. The deep form is differentiated with erythema nodosum, scleroderma.

To make a diagnosis of lupus erythematosus, a general blood test, a study on LE cells, immunological studies, serological reactions for the presence of antinuclear autoantibodies, and sometimes histological and immunomorphological studies, are mandatory.


1. Remediation of foci of chronic infection, normalization of disorders of various organs and systems.

2. Antimalarials: hydroxychloroquine (6.5 mg / kg / day), mepacrine 100 mg 3 times a day, delagil, plaquenil, hingamine, rezokhin, chloroquine, pivacine 0.25 2-3 times a day with ten-day courses with 3 5-day breaks or 5 days with 5-day breaks to avoid serious complications (pathology of the organs of vision, changes in blood count, liver damage, insomnia, dizziness, heart pain. A total of 25 g.

3. Glucocorticosteroids: in the acute stage, large doses are prescribed (40-100 mg of prednisone per day or other glucocorticosteroids in appropriate dosages). Upon reaching clinical recovery (persistent remission), hormone doses are gradually reduced to the minimum maintenance dose, which usually ranges from 10-15 mg of prednisolone.

4. Combined treatment with glucocorticosteroids and antimalarials (presocyl - delagil, prednisone, acetylsalicylic acid), low doses of glucocorticosteroids (prednisolone 10-15 mg) and antimalarials.

5. Aromatic retinoids: etretinate 1 mg / kg / day.

6. Vitamin therapy: nicotinic acid, nicotinamide, cyanocobalamin, retinol, pyridoxine, calcium pantothenate.

7. Salicylates: acetylsalicylic acid, aminosalicylic acid.

8. External treatment includes the use of glucocorticosteroid creams and ointments (on the face - non-fluorinated, on the skin of the trunk and extremities - fluorinated and difluorinated).

9. Local injection into foci of glucocorticosteroids.

10. For the purposes of prevention - mandatory photo protection. The use of sun protection with a protection factor of more than 30.


A serious disease, which is based on inflammation. Sclerosis and vascular disorders. The etiology is not established. There are infectious, neurogenic, endocrine, genetic theories of etiopathogenesis. Currently, scleroderma is referred to as rheumatic diseases with immune disorders. The pathological basis of the disease is a progressive collagen disorganization: mucoid and fibrinoid swelling, fibrinoid necrosis, hyalinosis, and sclerosis. Scleroderma can occur at any age, more common in women 20-50 years old.

Clinical forms: systemic (generalized) and limited (focal). With systemic scleroderma, the skin and internal organs are affected. With the diffuse form of systemic scleroderma, the disease begins rapidly, accompanied by generalized damage to the skin of the trunk and extremities, arthritis, tendovaginitis and rapid involvement of internal organs in the process. With the acrosclerotic form of systemic scleroderma, the skin of the hands, feet, face, and forearms is affected. The disease develops slowly, the internal organs are involved in a few years. One of the options for acrosclerosis is CREST syndrome: skin calcification, Raynaud's syndrome, esophageal dysfunction, sclerodactyly, telangiectasia.

Rashes on the skin with scleroderma go through 3 stages: edema, compaction, atrophy. In the edematous stage, the skin is red-violet or pink with a purple tint. In the compaction stage, erythema becomes less pronounced with the exception of the growth zone, where it remains in the form of a lilac ring around the compaction site, which can reach the consistency of cartilage. The skin is not creased. It acquires the color of the old ivory, it becomes smooth, without hair, fat and sweating are reduced. The purple ring disappears. Sometimes telangiectasias and pigmentation appear. Sensitivity in the focus is reduced, paresthesia is possible. In the stage of atrophy, the compaction gradually resolves. The skin is thinning like tissue paper, easy to fold, sinks.

In systemic scleroderma, changes in the skin of the face and hands are especially characteristic. In the stage of atrophy, the face becomes masked, amimic, without wrinkles. Nose, ears are thinned, lips are thin, do not always close. The mouth gap is narrowed by a purse-shaped folds around. The fingers are thinned, shortened due to osteolysis of the terminal phalanges, and sometimes flexion contractures (sclerosis) form. There may be cracks and trophic ulcers. On any affected area, calcification is possible, which can be opened with the release of a white crumbly mass.

Limited scleroderma. There are plaque, linear forms, white spot disease, idiopathic atrophy of Pasini-Pirini.

Plaque scleroderma occurs most often. Mostly women aged 30-50 years are ill. Rashes characteristic of scleroderma pass through 3 stages in their development: edema, compaction and atrophy. The diameter of the foci is 1-20 cm, calcification is sometimes possible, rarely damage to other organs and systems.

Linear scleroderma usually develops in children. The process goes through 3 stages, differing in the configuration of the outbreak. The lesion vertically crosses the forehead, descending from the scalp to the back of the nose or below, resembling a deep scar as after a saber strike. Skin atrophy spreads to the underlying muscles and bone, deforming the face. It is often combined with Romberg face hematrophy: foci of scleroderma are asymmetrically and characterized by unilateral atrophy of the facial skin, and with it atrophy of muscles, cartilage and bones. Atrophy of Romberg's face is conditionally attributed to varieties of scleroderma. With this disease, patients often suffer from headaches and sensory disturbances. Possible eyebrow, eyelash loss. Linear scleroderma can also be located on the limbs along the neurovascular bundles, along the intercostal nerves.

White spot disease. More often women are ill. Typical localization is the neck. Upper body, genital area. Small (0.5 cm) spots of snow-white color appear, often bordered by a pinkish-lilac corolla. The center of the spot sinks, then atrophy is formed. Sometimes follicular spines (gums) appear on the elements.

Idiopathic atrophy of Pasini-Perini. Mostly women (10-20 years old) are ill.
Localization - the skin of the back, rarely the skin of the chest, abdomen. There are pink-cyanotic or brown spots, there is no seal. Then atrophy is formed in the center of the focus.

Differential diagnosis. Systemic scleroderma is differentiated with dermatomyositis. Limited forms of scleroderma - with vitiligo, leprosy, atrophic lichen planus, vulvar kraurosis.


1. Penicillin therapy (20-30 million per course).

2. Vasoactive drugs: theoniol, nicotinic acid, no-spa, pentoxifylline.

3. Absorbable agents: subcutaneous and intramuscular lidase, lidase electrophoresis, hyaluronidase, vitreous body, ronidase, trypsin, collitin.

4. Lubrication of foci with a 30-50% solution of dimexide in combination with lidase.

5. D-penicillamine (cuprenyl)

6. EDTA, unitiol (especially with calcification).

7. Glucocorticosteroids in medium doses (prednisone 40-80 mg / day).

8. Anti-malarial drugs (although their effectiveness is lower than with lupus erythematosus).

9. Vitamin therapy: vitamins A, E, B15.

10. ATP.

11. Physiotherapy: magnetotherapy, electrophoresis with absorbable drugs, helium-neon laser, ultrasound, paraffin and ozokerite applications, mud therapy, hydrogen sulfide and rhodon baths, massage.

Dermatomyositis. A serious disease in which the skin and striated muscles are affected, as well as internal organs. Belongs to rheumatic diseases with immune disorders. The etiology of the disease is unknown. In pathogenesis, infectious diseases, vaccinations, drug allergies, stress, climatic factors, and malignant neoplasms are of particular importance. Idiopathic (primary) and paraneoplastic (secondary) forms are distinguished. The disease is observed at any age, more often in women.

During the disease, 3 periods are distinguished: prodromal, manifest and dystrophic. The disease can begin with fever, weakness, pain in the muscles and joints. Muscle weakness develops: it is difficult for a patient to get out of bed, climb stairs, put his hand behind his head, and turn in bed. The patient is not able to serve himself: hold a spoon, comb, shave, etc. These symptoms are accompanied by myalgia.

Skin lesions: periorbital edema and bright, “blazing” erythema with a purple tint (dermatomyositis), spots, papules, erosion, ulcers, peeling. Lesions of the nail rollers: telangiectasia, erythema. Less commonly recorded are urticarial, lichenoid, nodular, vesicular, bullous rashes, predominant localization of the rash - places of compression of the skin, open areas of the skin (face, chest, hands).

The diagnosis of dermatomyositis is confirmed by a clinical blood test, a biochemical blood test, a study of the activity of transaminases, creatine phosphokinase, lactate dehydrogenase, aldolase, urine biochemical analysis (creatinuria), electromyography, and, if necessary, a skin and muscle biopsy.

Dermatomyositis should be differentiated with lupus erythematosus, systemic scleroderma, erysipelas, Quincke's edema, toxicoderma, trichinosis.


1. Glucocorticosteroids in average doses (40-80 mg of prednisone per day). Dangerous development of steroid myopathy, especially triamcinolone.

2. Cytostatics: methotrexate, mercaptopurine, cyclophosphamide, azathioprine.

3. The combination of glucocorticosteroids and cytostatics.

4. Preparations of the quinolone series: hingamine, etc.

5. Anabolic steroids - nerobol, retabolil.

6. Vitamins: vitamin A, cocarboxylase, vitamin B5, ATP.

7. With calcification - complexones (EDTA).

Pemphigus acantholytic - severe dermatosis with a chronic undulating course. The basis is an autoimmune lesion of the skin and mucous membranes, which is manifested by acantolysis and the formation of blisters.

The etiology and pathogenesis are not clear. There are various theories of the occurrence of the disease: viral, neurogenic, endocrine, metabolic, the leading one is autoimmune. As for the pathogenesis of the disease, the mandatory presence of acantholysis is generally accepted. The destruction of bonds between epidermal cells is mediated by autoantibodies (Ig G). They bind to glycoproteins of cell membranes and cause acantholysis.

Pemphigus vulgaris more often occurs in the age range of 40-60 years, men and women get sick equally often. Pemphigus vulgaris usually begins with damage to the oral mucosa, and a rash on the skin appears after a few months. At first, the lesion is local in nature, and after 6-12 months, the rash becomes generalized. There is no itching, with the appearance of erosion - burning, pain. On the apparently unchanged skin, intraepidermal flabby blisters appear, which, due to peripheral growth, increase in size. The arrangement of the elements is random. The bubble of the bladder easily bursts and red, painful, weeping erosions are formed without a tendency to heal. Nikolsky’s positive symptom is characteristic, which is determined both directly in the lesion focus and on apparently healthy skin. Often, rashes are located on the mucous membrane of the mouth, nose, larynx, pharynx, and vagina. The bubbles here open so quickly that they are very difficult to see. In place of the blisters, painful erosion is formed. As a rule, the general condition is violated: weakness, malaise, weight loss, hoarseness of the voice, dysphagia, septic fever, complications from the internal organs are possible.

Other clinical forms of pemphigus.

Vegetative pemphigus affects the skin folds, near the mouth, neck and scalp. Characterized by the appearance of erosion covered with vegetation with purulent discharge. Transition of ordinary pemphigus into vegetative and vice versa is possible.

Pemphigus foliage is characterized by the appearance of bubbles on a slightly hyperemic background, which, when opened, form erosion, covered with leaf-shaped, multilayer crusts. In most cases, the bubbles are not clearly expressed as they open immediately after formation. Favorite localization is the face, scalp, upper chest, abdomen, but lesions of the entire skin are possible - exfoliative erythroderma.

Erythematous (seborrheic) pemphigus is observed more often in people 40-50 years old. It can be transformed into ordinary or leaf-shaped. Initially, erythema-squamous rashes appear, resembling seborrhea or discoid lupus erythematosus, localized on the face and scalp. Rashes spread to the skin of the chest and interscapular region. Bubbles may appear, which are covered with grayish-brown crusts.

Diagnostics. Positive symptoms of Nikolsky (detachment of the epidermis when pressure is applied to the skin near the bladder) and Asbo-Hansen (pressure on the lid of the bladder increases its area).

Detection of Tzanka cells in smear smears from the surface of erosion (round acantholytic cells). Skin pathomorphology: histological examination (acantholysis, intraepidermal bladder), immunofluorescence examination (Ig G deposition in the intercellular substance of the epidermis).

Дифференциальный диагноз проводят с дерматитом Дюринга, другими буллезными дерматозами, афтозным стоматитом, синдромом Лайела и тяжелыми формами токсикодермии. Вегетирующую пузырчатку дифференцируют с широкими кондиломами, вегетирующей пиодермией, йодо- и бромодермой, себорейную пузырчатку – с себорейной экземой и дискоидной красной волчанкой.

Treatment. Общее лечение заключается в назначении глюкокортикостероидов вначале в ударных дозах (60-80 мг преднизолона), затем снижая до терапевтической, а после достижения клинического выздоровления на длительное время назначают поддерживающую дозу.

Цитостатики (метотрексат, азатиоприн) назначают в виде монотерапии или в сочетании с глюкокортикостероидами, что позволяет снизить дозу последних.

Местно используют глюкокортикостероиды в виде аэрозолей, кремов и мазей, ванны с антисептиками, комплексные препараты, содержащие глюкокортикостероид и антибиотик, средства, стимулирующие эпителизацию. Эрозии можно смазывать водными растворами анилиновых красителей.

Герпетиформный дерматит Дюринга – хроническое рецидивирующее заболевание, которое проявляется зудящей полиморфной сыпью. Болеют взрослые 20-60 лет, редко – дети.

Этиология и патогенез не выяснены, ведущая теория – аутоиммунная. Указывают на значение в развитии заболевания глютеновой энтеропатии (целиакии). Провоцирующими факторами являются препараты йода, пища, богатая глютеном (белок злаковых).

Clinic. Заболевание сопровождается сильным жжением, зудом, болезненностью, покалыванием. Сыпь представлена эритематозными пятнами, волдырями, папулами, пузырьками или пузырями. Вторичные элементы представлены экскориациями, эрозиями, корками, вторичной гиперпигментацией. Высыпания симметричны и располагаются группами. Локализация - разгибательные поверхности локтевых и коленных суставов, ягодицы, кресцовая и межлопаточная области. Симптомы Никольского и Асбо-Ганзена отрицательные.

Диагностика. Проба Ядасона с 50% мазью йодида калия, патогистология ( субэпидермальные пузыри, эозинофильно-нейтрофильная инфильтрация дермы, микроабсцессы , содержащие эозинофилы и нейтрофилы в верхних отделах сосочков дермы), иммунофлюоресцентное исследование ( отложения Ig A в верхней части сосочков дермы).

Дифференциальный диагноз проводят с вульгарной пузырчаткой, токсикодермиями, сопровождающимися пузырным синдромом, другими пузырными дерматозами, аллергическим контактным дерматитом и др.

Treatment. Основным методом лечения являются сульфоны (диаминодифенилсульфон, дапсон). Можно назначать сульфаниламиды, глюкокортикостероиды. Наружно назначают мази с антибиотиками, водные растворы анилиновых красителей. Обязательна диета, исключающая продукты богатые йодом и глютенами (йодированная соль, морепродукты, бобовые).
<< Previous Next >>
= Skip to textbook content =

Тема № 6. Диффузные болезни соединительной ткани. Пузырные дерматозы

  1. ЛЕКЦИЯ № 9. Дифференциальный диагноз диффузных болезней соединительной ткани у детей. Clinic, diagnosis, treatment
    Классификация ревматических болезней. 1. Ревматизм. 2. Ювенильный ревматоидный артрит. 3. Анкилозирующий спондилит. 4. Другие спондилоартропатии. 5. Системная красная волчанка. 6. Васкулиты: 1) геморрагический васкулит (Шеклейн—Геноха); 2) узелковый периартериит (полиартериит у детей раннего возраста, болезнь Кавасаки, болезнь Вегенера); 3) артериит Такаясу. 7. Дерматомиозит. 8. Склеродермия. 9.
  2. Diffuse connective tissue diseases
    Diffuse connective tissue diseases (synonym: collagenoses, collagen diseases) is a group concept that combines several diseases in which diffuse damage to the connective tissue and blood vessels is noted. In 1942, Klemperer (P. Klemperer) proposed calling diffuse collagen disease diseases that are anatomically characterized by generalized alteration (damage)
    Diffuse diseases of the connective tissue is a group of diseases that are characterized by a systemic type of inflammation of various organs and systems as a result of the combined development of autoimmune and immunocomplex processes, as well as excessive fibrosis. A feature of this group of diseases is the multifactorial type of predisposition with a specific role of immunogenetic
  4. Diffuse connective tissue diseases
    DIFFUSIVE DISEASES OF THE CONNECTIVE TISSUE (DZST), or collagenosis (a term having historical significance), is a group of diseases characterized by a systemic immuno-inflammatory lesion of the connective tissue and its derivatives. This concept is a group, but not nosological, in connection with which this term should not denote individual nosological forms. DZST combine enough
    By this concept is meant a number of nosological forms characterized by a systemic type of damage to various organs and systems, the development of autoimmune and immune complex processes, and excessive fibrosis. In this lecture we will focus on three large collagenoses: systemic lupus erythematosus, systemic scleroderma and dermatomyositis. A few years ago in this group
  6. Diffuse connective tissue diseases
    Diffuse connective diseases
    Recently, rheumatic lesions of the nervous system are considered as a primary, systemic, gradually developing destructive process in the connective tissue. The term “collagenoses” was proposed by Klemperer and Ber in 1940. Based on the morphological characteristics of systemic lesions of the connective tissue, they combined a number of diseases into a group of collagenoses. It was later established that in
    Диффузные забол-ия соед.тк (ДБСТ)– группа нозологических форм, х-ся системным аутоиммунным и иммуно комплексным воспалением или избыточным фиброзо образованием (при системной склеродермией) В ДБСТ включены: СКВ, системная склеродермия, дерматомиозит, синдром Шегрена, диффузный эозинофильный фасцит, смешанные соединительно тканое заболевание и ревматичесая полимиалгия Этиология – можно
  10. Connective tissue disease
    Lupus erythematosus, or scarring erythematosis, is a chronic disease with damage to the connective tissue. Etiology and pathogenesis are not fully established. There are a number of theories of the onset of the disease (autoimmune, genetic, endocrine, viral, etc.), but none of them can explain the whole essence of the disease. It has been proven that mechanical,
    Darwin J. Prockop Hereditary connective tissue diseases are among the most common genetic syndromes. These include most often imperfect osteogenesis, Ehlers-Danlos and Marfan syndromes. The classification of these syndromes is usually based on the work of McKusick, who analyzed signs, symptoms, and morphological changes in
    Пузырные (буллёзные) дерматозы – группа заболеваний, основным морфологическим элементом которых является пузырь с локализацией как на коже, так и на слизистых оболочках. Классификация пузырных дерматозов 1) Пузырчатка истинная (акантолотическая). 2) Герпетиформный дерматит Дюринга. 3) Пемфигоиды (неакантолитическая пузырчатка): – буллёзный пемфигоид Левера;
  13. Пузырные дерматозы
    Пузырчатка. Этиология и патогенез болезни полностью не изучены. Существует множество теорий возникновения патологического процесса: теория задержки хлоридов, токсического, неврогенного, энзимного, бактериального, вирусного, аутоиммунного происхождения. Различают следующие клинические формы: вульгарная, листовидная, вегетирующая, себорейная (эритематозная). Чаще встречается вульгарная форма
  14. Пузырные дерматозы
  15. Types of connective tissue.
    {foto11} Connective tissue. From left to right: loose connective tissue, dense connective tissue, cartilage, bone, blood. Loose connective tissue consists of cells scattered in the intercellular substance, and intertwined disordered fibers. Wavy bundles of fibers consist of collagen, and straight ones of elastin; their combination provides the strength and elasticity of the connective
  16. Abstract. Biochemistry of bone and connective tissue., 2008
    single fabric. Composition and structure. Glucosaminoglycans. Glycoproteins. Fibers of connective tissue. Collagen synthesis. Biochemical changes in connective tissue during aging and some pathological processes. Factors that regulate the metabolism of connective tissue. Bone. Osteoblast. Osteocyte. Osteoclast. The chemical composition of bone tissue. Bone formation. Factors
    Connective tissue is characterized by the ability to synthesize collagen, elastin, etc. (7, 9, 13, 14, 21). The secretion of these substances into the intercellular space and the formation of a matrix from them, which then combines with calcium ions, ends with the formation of dense tissue formations that hold together unlike cells and tissues, which determines the function of the tissue as connecting (Table.
Medical portal "MedguideBook" © 2014-2019