Cutaneous vasculitis. What is cutaneous vasculitis, how does it manifest and how is it treated?

Vasculitis is a disease in which the walls of blood vessels located in the dermis and subcutaneous tissue are affected by the inflammatory process.

The disease can spread anywhere on the skin and even affect internal organs, destroying the vessels located in them.

Causes

The appearance of vasculitis can be caused by various factors. In most cases, the disease is caused by an allergic reaction, which causes damage and destruction of the walls of capillaries, arterioles and venules of the skin. The disease manifests itself in different ways, which depends on the nature of the affected vessels, as well as on the type of vasculitis.

• All information on the site is for informational purposes only and is NOT a guide to action!
• Can give you an ACCURATE DIAGNOSIS only DOCTOR!
• We kindly ask you NOT to self-medicate, but make an appointment with a specialist!
• Health to you and your loved ones!

The disease can be caused by other reasons:

• intoxication to which the body has been exposed for a long period;
• infectious diseases occurring in the body;
• individual intolerance to the medications used;
• injuries and hypothermia.

Attention is also paid to heredity. Sometimes vascular superficial vasculitis can develop due to a genetic predisposition.

Symptoms of superficial vasculitis

The symptoms of vasculitis depend on their type:

The redness is round in shape. Their diameter can reach one centimeter. Purple spots are characterized by the shape of small round rings.

The disease has an acute form when it occurs, and then becomes chronic. It will take a lot of time to treat it. Relapses are possible.

If patients only have a rash without any other symptoms, then photos of superficial vasculitis will help determine the type of disease

Kinds

Vasculitis can be of different types, depending on the vessels affected:

• capillary;
• phlebitis;
• arterial;
• (vessels of different groups are simultaneously affected).

Also, the disease can be primary or secondary. Primary vasculitis is an independent disease, while secondary vasculitis occurs as a consequence of a recent or existing disease in the body.

Most often, patients experience the following types of illness:

• hemorrhagic vasculitis;
• Werther–Dümling disease;
• hemorrhagic leukoclastic microbid Miescher - Shtork;
• disseminated allergic angiitis Roskam;
• three-symptom Gougereau-Duperre syndrome;

Hemorrhagic

Inflammatory processes in this disease affect capillaries located in the epidermis, kidneys, joints, and organs of the digestive system. The disease can occur in people of any age. It rarely occurs in children under 3 years of age. Most often, the disease appears between the ages of 4 and 12 years.

Photo: superficial vasculitis of the extremities

The disease can be identified by various signs:

If hemorrhagic vasculitis occurs in severe form in children, complications such as systolic murmur in the heart and headaches due to inflammation of the blood vessels in the brain are possible. In boys, testicular damage sometimes occurs.

The disease is diagnosed by laboratory examination which includes:

• general and biochemical analysis blood;
• blood clotting test;
• skin biopsy;
• immunological examination.

Patients with this disease are prescribed antiplatelet agents Curantil and Trental. If the disease occurs in a complex form, the drugs must be taken simultaneously. The patient is also prescribed anticoagulants, the dosage of which depends on the complexity of the case.

Glucosteroids are also included in the list of medications for the disease. They, like cytostatics, are prescribed for severe forms of the disease. In such cases, transfusion therapy or plasma exchange may be used.

Hemorrhagic leukoclastic microbid Miescher – Shtorka

The disease is often a consequence of chronic tonsillitis or appendicitis. Those suffering from the disease develop many spots on the skin, which are hemorrhages in the epidermis.

Blisters, blisters or nodules also appear on the skin, subject to rapid necrosis. In their place, ulcers form.

Photo: rashes with superficial vasculitis on the legs

Patients often present with:

• malaise;
• joint pain;
• slight increase in temperature.

The rash spreads over the skin of the arms and legs, torso and face. It sometimes appears on the mucous membranes of the oral cavity.

The disease is diagnosed by histological examination, which reveals the leukoclasia characteristic of this disease.

Antibacterial and antihistamines are used for treatment medications, calcium supplements, angioprotectors. Therapy is supplemented with medications for external use. These can be ointments, lotions, aniline dyes.

Werther-Dümling disease, or necrotizing nodular disease

With this disease, brown nodules with a diameter of about 5 mm or larger appear on the skin. Sometimes erythematous spots and hemorrhages appear on the skin at the same time.

A significant number of nodules after necrosis turn into ulcers of varying depths, after healing of which scars remain on the skin. The rash is located on the extremities, often near the joints, and can spread throughout the torso and genitals.

The disease in most cases affects adults. The disease develops gradually, becoming chronic and worsening.

The disease affects small veins and arteries, as a result of which the walls of the vessels change, narrowing or completely closing the lumen.

Gougereau-Duperre three-symptom syndrome

The disease causes the formation of nodules, purple spots and erythematoses on the skin. With the disease, only two or one sign may appear.

The nodules are not very different in color from healthy skin or have a reddish tint. They can appear along with purpuric spots and erythematoses throughout the body, although they are often localized on the legs.

When the disease occurs, it has an acute form. Over time, it becomes chronic and can cause complications.

The patient’s condition is often normal, only during exacerbations does joint pain appear, lymph nodes become enlarged, weakness is felt, high fever, and asthmatic attacks appear

The disease has 4 stages of development:

Ruiter's allergic arteriolitis

The inflammatory process during the disease affects the walls of arterioles.

There are 4 types of this disease:

All types of illness appear after infectious diseases that were previously observed in the patient. They can be a consequence of sore throat, pharyngitis or flu.

Disseminated allergic angiitis Roskam

With the disease, numerous hemorrhages appear, which can be located on the skin or mucous membrane. They form spontaneously or can result from injury.

Patients suffering from this disease may experience bleeding due to impaired blood clotting. The disease occurs in chronic forms Yes, relapses may occur.

The reason for its appearance is weak capillary walls. As the vessels are destroyed by inflammatory processes, small extravasates form around them.

Skin vasculitis is a group of diseases of a multifactorial nature, in which the leading symptom is inflammation of the blood vessels of the dermis and subcutaneous tissue.

The difficulty in covering this topic is that until now there is no generally accepted classification or even agreed upon terminology of vasculitis. Currently, about 50 different nosological forms have been described, and understanding this diversity is not easy. The diversity of clinical manifestations and insufficiently studied pathogenetic mechanisms have led to the fact that under different names only a variant of the main type of skin lesion may be hidden. Also, in addition to primary vasculitis, which is based on inflammatory damage to the blood vessels of the skin, there are also secondary vasculitis (specific and nonspecific), developing against the background of a certain infectious (syphilis, tuberculosis, etc.), toxic, paraneoplastic or autoimmune (systemic lupus erythematosus, dermatomyositis etc.) process. It is possible to transform skin vasculitis into a systemic process with damage internal organs and the development of severe, sometimes life-threatening complications.

Skin vasculitis is a polyetiological disease. The most common connection is with a focal infection (streptococci, staphylococci, mycobacterium tuberculosis, yeast, viruses, etc.). Increased sensitivity to a number of drugs, in particular to antibiotics and sulfa drugs. Often, despite a carefully collected anamnesis and examination, the etiological factor remains unclear. Among the risk factors for vasculitis, one should take into account: age (children and the elderly are most vulnerable), hypothermia, excessive insolation, severe physical and mental stress, trauma, surgery, liver disease, diabetes, hypertension. Pathogenetic mechanism The development of skin vasculitis is currently considered to be the formation of circulating immune complexes with their subsequent fixation in the endothelium, although this has not been definitively proven for all diseases of this group.

Skin vasculitis is a heterogeneous group of diseases, and their clinical manifestations are extremely diverse. However, there are a number of common features that unite these dermatoses:

1) inflammatory nature of skin changes;
2) symmetry of rashes;
3) tendency to edema, hemorrhage and necrosis;
4) primary localization on lower limbs;
5) evolutionary polymorphism;
6) connection with previous infectious diseases, medication, hypothermia, allergic or autoimmune diseases, and impaired venous outflow;
7) acute or worsening course.

Skin lesions with vasculitis are diverse. These may be spots, purpura, nodules, nodes, necrosis, crusts, erosions, ulcers, etc., but the main clinical differential sign is palpable purpura (hemorrhagic rash that rises above the surface of the skin and is felt on palpation).

There is no generally accepted classification of vasculitis. Vasculitis is systematized according to different principles: etiology and pathogenesis, histological picture, severity of the process, features of clinical manifestations. Most clinicians use predominantly morphological classifications cutaneous vasculitis, which is usually based on clinical changes in the skin, as well as the depth of location (and, accordingly, the caliber) of the affected vessels. There are superficial (damage to the vessels of the dermis) and deep (damage to the vessels at the border of the skin and subcutaneous tissue) vasculitis. Superficial ones include: hemorrhagic vasculitis (Henoch-Schönlein disease), allergic arteriolitis (polymorphic dermal angiitis), leukoclastic hemorrhagic Miescher-Storck microbiota, as well as chronic capillaritis (hemosiderosis): ring-shaped telangiectatic purpura of Majocchi and Schamberg's disease. To the deep: cutaneous form of periarteritis nodosa, acute and chronic erythema nodosum.

Hemorrhagic vasculitis- a systemic disease that affects small vessels of the dermis and manifests itself as palpable purpura, arthralgias, damage to the gastrointestinal tract (GIT) and glomerulonephritis. It occurs at any age, but boys aged 4 to 8 years are at greatest risk. Develops after an infectious disease, after 10-20 days. The acute onset of the disease, with fever and symptoms of intoxication, is most often observed in childhood. The following forms are distinguished hemorrhagic vasculitis: cutaneous, cutaneous-articular, cutaneous-renal, abdominal-cutaneous and mixed. The current can be lightning fast, sharp and protracted. The duration of the disease varies - from several weeks to several years.

The process begins symmetrically on the lower limbs and buttocks. The rashes are papular-hemorrhagic in nature, often with urticarial elements, and do not disappear with pressure. Their color changes depending on the time of appearance. The rash occurs in waves (once every 6-8 days), with the first waves of the rash being the most violent. Articular syndrome appears either simultaneously with skin lesions or after a few hours. Most often affected large joints(knees and ankles).

One of the variants of the disease is the so-called necrotic purpura, observed during the rapid course of the process, in which necrotic skin lesions, ulcerations, and hemorrhagic crusts appear.

The greatest difficulties are caused by the diagnosis of the abdominal form of hemorrhagic vasculitis, since skin rashes do not always precede gastrointestinal phenomena (vomiting, cramping pain in the abdomen, tension and pain on palpation, blood in the stool).

The renal form is manifested by impaired renal function varying degrees severity, from short-term unstable hematuria and albuminuria to a pronounced picture of acute glomerulonephritis. This is a late symptom and never occurs before the skin is affected.

The fulminant form of hemorrhagic vasculitis is characterized by an extremely severe course, high fever, widespread rashes on the skin and mucous membranes, viscerapathies, and can result in the death of the patient.

Diagnosis of the disease is based on typical clinical manifestations; in atypical cases, a biopsy is performed. In the abdominal form, surgical supervision is necessary. Observation by a nephrologist is recommended for three months after resolution of purpura.

The term “allergic arteriolitis” Ruiter (1948) proposed to name several related forms of vasculitis, differing in clinical manifestations, but having a number of common etiological, pathogenetic and morphological features.

The pathogenetic factors of the disease are considered to be colds and focal infections. The rashes are usually located symmetrically and are polymorphic in nature (spots, papules, vesicles, pustules, necrosis, ulcerations, telangiectasia, blisters). Depending on the predominant elements, three forms of the disease are distinguished: hemorrhagic type, polymorphic-nodular (corresponds to three-symptomatic Gougereau-Duperre disease) and nodular-necrotic (corresponds to Werther-Dümling nodular-necrotic dermatitis). When the rash regresses, cicatricial atrophies and scars may remain. The disease is prone to relapse. Often before the rash, patients complain of malaise, fatigue, headache, and at the height of the disease - pain in the joints (which sometimes swell) and in the abdomen. Diagnosis of all types of disease is difficult due to the lack of typical, characteristic symptoms. Histological examination reveals fibrinoid lesions of small-caliber vessels with the formation of infiltrative accumulations of neutrophils, eosinophils, lymphocytes, plasma cells and histiocytes.

Hemorrhagic leukoclastic microbid Miescher-Storck the clinical course is similar to other forms of polymorphic dermal vasculitis. A sign that makes it possible to distinguish this disease as an independent one is the presence of a phenomenon - leukoclasia (disintegration of the nuclei of granular leukocytes, leading to the formation of nuclear dust) during histological examination. Thus, hemorrhagic leukoclastic microbide can be interpreted as a dermatosis caused by chronic focal infection (intradermal tests with streptococcal antigen are positive), occurring with severe leukoclasia.

Chronic capillaritis (hemosiderosis), in contrast to acute purpura, is characterized by a benign course and is exclusively a skin disease.

Schamberg's disease- is a lymphocytic capillaritis, which is characterized by the presence of petechiae and brown purple spots, occurring most often on the lower extremities. Patients are concerned solely as a cosmetic defect.

Purpura Majocchi characterized by the appearance on the lower extremities of pink and liquid-red spots (without previous hyperemia, infiltration), slowly growing to form ring-shaped figures. In the central part of the spot, slight atrophy and achromia develop, and vellus hair falls out. There are no subjective sensations.

Periarteritis nodosa characterized by necrotizing inflammation of small and medium-sized arteries muscular type with subsequent formation of vascular aneurysms and damage to organs and systems. Most common in middle-aged men. Of the etiological factors, the most important are drug intolerance (antibiotics, sulfonamides), vaccination and persistence of HbsAg in the blood serum. The disease begins acutely or gradually with symptoms general- increased body temperature, rapidly increasing weight loss, pain in the joints, muscles, abdomen, skin rashes, signs of damage to the gastrointestinal tract, heart, peripheral nervous system. Over time, polyvisceral symptoms develop. Particularly characteristic of periarteritis nodosa is kidney damage with the development of hypertension, which sometimes becomes malignant with the appearance of renal failure. There are classic and cutaneous forms of the disease. Skin rashes are represented by nodules - single or in groups, dense, mobile, painful. The formation of nodes along the arteries is typical, sometimes they form strands. Localization on the extensor surfaces of the legs and forearms, on the hands, face (eyebrows, forehead, corners of the jaw) and neck. They are often not visible to the eye and can only be determined by palpation. Necrosis may develop in the center with the formation of long-lasting non-healing ulcers. Periodically, the ulcers may bleed for several hours (a symptom of a “bleeding subcutaneous node”).

Sometimes the only manifestation of the disease may be reticular or branched livedo (persistent violet-red spots), localized on the distal parts of the extremities, mainly on the extensor surfaces or lower back. It is typical to detect nodules along the course of livedo.

Diagnosis of the disease is based on a combination of damage to a number of organs and systems with signs of significant inflammation, fever, changes primarily in the kidneys, heart, and the presence of polyneuritis. There are no laboratory parameters specific for this disease. Dynamic clinical observation for the sick.

Acute erythema nodosum is panniculitis, which is characterized by the presence of painful pink nodes on the extensor surface of the lower extremities. Accompanied by fever, malaise, diarrhea, headache, conjunctivitis and cough. Among adults, erythema nodosum is 5-6 times more common in women, with a peak age of 20-30 years. The disease is based on hypersensitivity to various antigens (bacteria, viruses, fungi, tumors and diseases connective tissue). Half of the cases are idiopathic. Diagnosis is based on history and physical examination. It is necessary to carry out full analysis blood, an X-ray of the lungs (detects bilateral adenopathy in the region of the roots of the lungs), a throat smear or a rapid test for streptococci.

Chronic erythema nodosum is a group of different types of nodular dermohypodermites. Women aged 30-40 years are most often affected. Nodes of various sizes appear on the legs with reddened skin over them, without a tendency to necrosis and ulceration. Inflammatory phenomena in the area of ​​the rash and subjective sensations (arthralgia, myalgia) are mild. Clinical variants of chronic erythema nodosum have their own characteristics, for example, the tendency of the nodes to migrate (Beferstedt's erythema migrans) or the asymmetry of the process (Vilanova-Pinol hypodermatitis).

Tactics for managing a patient with skin vasculitis

1. Classify the disease (characteristic clinical picture, anamnesis, histological examination).
2. Search for an etiological factor, but in 30% of cases it cannot be established (search for foci of chronic infection, microbiological, immunological, allergological and other studies).
3. Assessment of the general condition and determination of the degree of disease activity: general blood and urine analysis, biochemical blood test, coagulogram, immunogram. Degree of vasculitis activity: I. Rashes are not abundant, body temperature is not higher than 37.5, general symptoms are insignificant, ESR is not higher than 25, C-reactive protein is not more than ++, complement is more than 30 units. II. The rash is abundant (extends beyond the lower leg), body temperature is above 37.5, general symptoms - headache, weakness, symptoms of intoxication, arthralgia; ESR is higher than 25, C-reactive protein is more than ++, complement is less than 30 units, proteinuria.
4. Assessment of signs of systemicity (research according to indications).
5. Determination of the type and regimen of treatment depending on the degree of activity: Art. I. — treatment on an outpatient basis is possible; II Art. - in the hospital. In all cases of exacerbation of skin vasculitis, it is necessary bed rest, since such patients usually have pronounced orthostasis, which should be observed before entering the regressive stage. A diet that excludes irritating foods is recommended ( alcoholic drinks, spicy, smoked, salted and fried foods, canned food, chocolate, strong tea and coffee, citrus fruits).
6. Etiological treatment. If it is possible to eliminate the causative agent (drug, chemicals, infection), then resolution of the skin lesions follows quickly and no other treatment is required. But we must remember that when sanitizing foci of infection, an increase in the vascular process may be observed.
7. Pathogenetic treatment.
8. Preventive measures: clinical examination, prevention of provoking factors (infections, hypothermia, insolation, stress, etc.), rational use of medicines, employment, physical therapy, Spa treatment.

Treatment of hemorrhagic vasculitis

1. Glucocorticosteroids (prednisolone up to 1.5 mg/kg) alleviate the manifestation of skin-articular syndrome, but do not shorten the disease or prevent kidney damage. Prescribed in severe cases and under the cover of heparin, because they increase blood clotting.
2. Nonsteroidal anti-inflammatory drugs (NSAIDs) in usual therapeutic dosages. The choice of a specific drug is not of fundamental importance (indomethacin, diclofenac, acetylsalicylic acid).
3. Anticoagulants and antiplatelet agents. Heparin for a common process 300-400 IU/kg/day. The duration of the course should be at least 3-5 weeks. Under coagulogram control.
4. Therapeutic plasmapheresis when the manifestations of the disease are not eliminated by the listed means.
5. Nicotinic acid in tolerable doses intravenously.
6. You should not use: antihistamines (possibly only at the very beginning of the disease), calcium supplements, all vitamins.

Treatment of skin vasculitis

1) NSAIDs (naproxen, diclofenac, Reopirin, indomethacin, etc.);
2) salicylates;
3) Ca preparations;
4) vitamins P, C, antioxidant complex;
5) vasodilators (xanthinol nicotinate, pentoxifylline);
6) 2% solution of potassium iodide, 1 tbsp. l. 3 times a day (erythema nodosum);
7) anticoagulants and antiplatelet agents;
8) methods of detoxification via intravenous drip;
9) glucocorticosteroids (GCS) 30-35 mg/day for 8-10 days;
10) cytostatics;
11) ultra-high frequency therapy, diathermy, inductothermy, ultrasound with hydrocortisone, ultraviolet irradiation.

External treatment. For erosive and ulcerative rashes

1) 1-2% solutions of aniline dyes;
2) epithelializing ointments (solcoseryl);
3) ointments containing glucocorticoids, etc.;
4) lotions or ointments with proteolytic enzymes (Chymopsin, Iruksol);
5) Dimexide applications;

For knots - dry heat.

Treatment should not end with the disappearance of clinical manifestations of the disease. It continues until laboratory parameters are completely normalized, and in the next six months to a year, patients are given maintenance treatment

Literature

1. Adaskevich V. P., Kozin V. M. Skin and venereal diseases. M.: Med. lit., 2006, p. 237-245.
2. Kulaga V.V., Romanenko I.M., Afonin S.L. Allergic diseases blood vessels of the skin. Lugansk: “Etalon-2”, 2006. 168 p.
3. Berenbein B. A., Studnitsin A. A. and others. Differential diagnosis of skin diseases. M. Medicine, 1989. 672 p.

I. B. Mertsalova, Candidate of Medical Sciences

RMAPO, Moscow

In fact, the cutaneous form of vasculitis is an inflammation of dermal vessels of different sizes. At the same time, today there is no clear and generally accepted classification of this disease. Likewise, the reasons for its occurrence are not fully understood. Presumably, skin vasculitis can occur for the following reasons:

• Focal infections (viruses, mycobacterium tuberculosis, staphylococci, streptococci, yeasts, etc.).
• Hypersensitivity or intolerance to certain medications. Most often these are sulfonamides and antibiotics.
• Hypothermia.
• Solar exposure.
• Liver diseases.
• Excessive physical activity.
• Mental stress and trauma.
• Hypertension.
• Diabetes.
• Mechanical damage skin and surgery.

Thus, there are many prerequisites for the appearance of cutaneous vasculitis in any person, but children and the elderly are at particular risk.

Symptoms and signs of skin vasculitis (photos)

Symptoms of cutaneous vasculitis are heterogeneous and very diverse. However, there are common signs that unite this type of dermatosis:

Skin vasculitis (photo) can look different. Basically, this is a hemorrhagic rash that rises above healthy areas of the skin and is clearly felt upon palpation. But it can also be crusts, necrosis, ulcers, erosions, nodules, purpuras or simply bluish spots.

Treatment of skin vasculitis

Before starting treatment for cutaneous vasculitis, evaluate general state patient, the degree of activity and systemicity of the disease is determined. For this purpose, blood and urine tests are taken, an immunogram and coagulogram are compiled, a histological examination and a number of other diagnostic measures are carried out.

In almost all cases, the patient is prescribed bed rest, since very often vasculitis limited to the skin is accompanied by orthostatic hypotension(sudden confusion or fainting).

Angiitis or vasculitis of the skin are usually treated with the following drugs:

• Glucocorticosteroids alleviate the symptoms of dermal damage, but do not eliminate the cause of the disease. As a rule, in severe cases, Prednisolone is prescribed, which is prescribed together with Heparin, thus increasing blood clotting.
• Diclofenac, Indomethacin, Acetylsalicylic acid are non-steroidal anti-inflammatory drugs (NSAIDs).
• Heparin is an anticoagulant and antiplatelet agent (prevents thrombus formation). Take the drug for at least 3-5 weeks with constant diagnosis of the hemostatic system.
• If the above remedies do not produce results, plasmapheresis is prescribed.
• Nicotinic acid (after tolerance test).

When treating skin vasculitis, it is not recommended to use antihistamines, as well as any vitamins and calcium supplements. Therapeutic measures cannot be completed when external symptoms disappear. They must be carried out until laboratory parameters will not completely return to normal. And in order to avoid relapses of skin vasculitis, maintenance treatment must be carried out for 6-12 months after recovery.

Interesting materials on this topic!

The appearance of vasculitis on the legs
The reason that provokes vasculitis in the legs is the inflammatory process of the vascular walls, which disrupts them...

Is there renal vasculitis?
About half of people suffering from hemorrhagic or cryoglobulinemic vasculitis inflammatory processes affect...

Does vasculitis affect the eyes?
One of the forms of inflammation of the retinal vessels is eye vasculitis. The disease can be recognized by...

Reviews and comments

Zoya Petrovna- 26 Feb 2019, 15:11

A year ago, I got a job as a consultant in a women's fashion store. I really like my work, because thanks to me, women transform themselves, start a new life, build families, and simply enjoy new purchases. It was only after I had been running around on my feet with such enthusiasm for a year, serving my clients, that I began to feel that my legs were getting tired. But according to the dress code, I’m not allowed to wear heels. At work, an employee advised me to buy

Vasculitis (angiitis) of the skin is a dermatosis, in the clinical and pathomorphological picture of which the initial and leading element is nonspecific inflammation of the walls of the dermal and hypodermal blood vessels of various sizes.

Etiology and epidemiology of vasculitis

According to statistics, the incidence of skin vasculitis averages 38 cases per million, with women predominantly affected.

Vasculitis is currently considered as a polyetiological disease. The leading theory is the immune complex genesis of vasculitis. Most common cause development of vasculitis limited to the skin are various acute or chronic infections: bacterial (staphylococci, streptococci, enterococci, yersinia, mycobacteria), viral (Epstein-Barr virus, hepatitis B and C viruses, HIV, parvovirus, cytomegalovirus, herpes simplex virus, influenza) and fungal. Microbial agents play the role of antigens, which, interacting with antibodies, form immune complexes that settle on the walls of blood vessels.Among exogenous sensitizing factors, a special place is given to medicines, the intake of which is associated with the development of 10% of cases of skin vasculitis. Most often, antibacterial drugs (penicillins, tetracyclines, sulfonamides, cephalosporins), diuretics (furosemide, hypothiazide), anticonvulsants (phenytoin), allopurinol lead to vascular damage. Medicinal substances, being part of immune complexes, damage the walls of blood vessels and also stimulate the production of antibodies to the cytoplasm of neutrophils.

Neoplasms can also act as a provoking factor. As a result of the production of defective proteins by tumor cells, the formation of immune complexes occurs. According to another theory, the similarity in the antigenic composition of the tumor and endothelial cells may lead to the production of autoantibodies.

A significant role in the development of vasculitis limited to the skin may play chronic intoxication, endocrinopathies, various types of metabolic disorders, as well as repeated cooling, mental and physical stress, photosensitivity, venous stagnation.

Skin vasculitis often acts as a skin syndrome in diffuse connective tissue diseases (SLE, rheumatoid arthritis, etc.), cryoglobulinemia, and blood diseases.

Classification of vasculitis

Currently, there is no single generally accepted classification of skin vasculitis. For practical purposes, the working classification of skin vasculitis developed by O.L. is used. Ivanov (1997).

Classification of skin vasculitis

Erythema nodosum (L52), previously considered a variant of vasculitis limited to the skin, is now classified as a panniculitis.

Clinical picture (symptoms) of vasculitis

Clinical manifestations of skin vasculitis are extremely diverse. However, there are a number of common features that clinically unite this polymorphic group of dermatoses:

• inflammatory nature of skin changes;
• tendency of rashes to edema, hemorrhage, necrosis;
• symmetry of the lesion;
• polymorphism of precipitating elements (usually evolutionary);
• primary or predominant localization on the lower extremities (primarily on the legs);
• the presence of concomitant vascular, allergic, rheumatic, autoimmune and other systemic diseases;
• frequent association with previous infection or drug intolerance;
• acute or periodically worsening course.

The disease has a chronic relapsing course and is distinguished by various morphological manifestations. The rash initially appears on the legs, but can also appear on other parts of the skin, less often on the mucous membranes. Characteristic features are blisters, hemorrhagic spots of various sizes, inflammatory nodules and plaques, superficial nodes, papulonecrotic rashes, vesicles, blisters, pustules, erosions, superficial necrosis, ulcers, scars. The rash is sometimes accompanied by fever, general weakness, arthralgia, and headache. The rash that appears usually lasts for a long period (from several weeks to several months) and tends to recur.

Depending on the clinical picture of the disease, various types of polymorphic dermal vasculitis are distinguished:

– Urticarial type. As a rule, it simulates the picture of chronic recurrent urticaria, manifesting itself as blisters of various sizes that appear in different areas of the skin. However, unlike urticaria, blisters with urticarial vasculitis are persistent, persisting for 1–3 days (sometimes longer). Instead of severe itching, patients usually experience a burning sensation or a feeling of irritation in the skin. Rashes are often accompanied by arthralgia, sometimes abdominal pain, i.e. signs of systemic damage. Examination may reveal glomerulonephritis. Patients experience an increase in erythrocyte sedimentation rate (ESR), hypocomplementemia, an increase in lactate dehydrogenase activity, and changes in the ratio of immunoglobulins. Treatment with antihistamines usually has no effect. The final diagnosis is made after pathohistological examination of the skin, which reveals a picture of leukocytoclastic vasculitis.

– The hemorrhagic type of dermal vasculitis is the most common. A typical manifestation of this variant is the so-called palpable purpura - edematous hemorrhagic spots of various sizes, usually localized on the legs and back of the feet, easily determined not only visually, but also by palpation, which is how they differ from other purpuras. However, the first rashes of the hemorrhagic type are usually small swollen inflammatory spots that resemble blisters and soon transform into a hemorrhagic rash. With further increase inflammatory phenomena Against the background of confluent purpura and ecchymosis, hemorrhagic blisters can form, leaving deep erosions or ulcers after opening. The rash is usually accompanied by moderate swelling of the lower extremities. Hemorrhagic spots may appear on the mucous membrane of the mouth and pharynx.

The described hemorrhagic rashes that occur acutely after colds(usually after a sore throat) and accompanied by fever, severe arthralgia, abdominal pain and bloody stools constitute the clinical picture of anaphylactoid Henoch-Schönlein purpura, which is more often observed in children.

– Papulonodular type is quite rare. It is characterized by the appearance of smooth, flattened, rounded inflammatory nodules the size of a lentil or a small coin, as well as small superficial, blurred, edematous pale pink nodules the size of a hazelnut, painful on palpation. The rashes are localized on the extremities, usually on the lower extremities, rarely on the torso, and are not accompanied by pronounced subjective sensations.

– The papulonecrotic type is manifested by small flat or hemispherical inflammatory non-scaling nodules, in the central part of which a dry necrotic scab soon forms, usually in the form of a black crust. When the scab is removed, small rounded superficial ulcers are exposed, and after the papules are reabsorbed, small “stamped” scars remain. The rashes are located, as a rule, on the extensor surfaces of the extremities and clinically completely simulate papulonecrotic tuberculosis, with which the most careful differential diagnosis should be carried out.

– The pustular-ulcerative type usually begins with small vesiculopustules, reminiscent of acne or folliculitis, quickly transforming into ulcerative lesions with a tendency to steady eccentric growth due to the disintegration of the edematous bluish-red peripheral ridge. The lesion can be localized on any part of the skin, most often on the legs, in the lower half of the abdomen. After healing of the ulcers, flat or hypertrophic scars remain, retaining an inflammatory color for a long time.

– Necrotizing-ulcerative type is the most severe variant of dermal vasculitis. It has an acute (sometimes lightning-fast) onset and a subsequent protracted course (if the process does not end quickly fatal). Due to acute thrombosis Inflamed blood vessels cause necrosis (infarction) of one or another area of ​​the skin, manifested by necrosis in the form of an extensive black scab, the formation of which may be preceded by an extensive hemorrhagic spot or bubble. The process usually develops over several hours, accompanied by severe pain and fever. The lesion is most often located on the lower extremities and buttocks. Purulent-necrotic scab persists long time. The ulcers formed after its rejection have different sizes and shapes, are covered with purulent discharge, and scar extremely slowly.

– The polymorphic type is characterized by a combination of various eruptive elements characteristic of other types of dermal vasculitis. More often, a combination of edematous inflammatory spots, hemorrhagic purpuric rashes and superficial edematous small nodes is noted, which constitutes the classic picture of the so-called three-symptom Gougereau-Duperre syndrome and the polymorphonodular type of Ruiter’s arteriolitis, which is identical to it.

Chronic dermal capillaritis affecting the papillary capillaries. Depending on the clinical characteristics, the following varieties (types) are distinguished:

– Petechial type (persistent progressive pigmented purpura of Schamberg) – the main disease of this group, parental to its other forms, is characterized by multiple small (point-like) hemorrhagic spots without edema (petechiae) resulting in persistent brownish-yellow spots of hemosiderosis of varying sizes and shapes; the rashes are most often located on the lower extremities, are not accompanied by subjective sensations, and occur almost exclusively in men.

– Telangiectatic type (telangiectatic purpura of Majocchi) is most often manifested by peculiar medallion spots, the central zone of which consists of small telangiectasias (on slightly atrophic skin), and the peripheral zone of small petechiae against the background of hemosiderosis.

– Lichenoid type (pigmented purpuric lichenoid angiodermatitis of Gougerot-Blum) is characterized by disseminated small lichenoid shiny, almost flesh-colored nodules, combined with petechial rashes, spots of hemosiderosis and sometimes small telangiectasias.

– The eczematoid type (Dukas-Kapetanakis eczematoid purpura) is distinguished by the occurrence in the foci, in addition to petechiae and hemosiderosis, of eczematization phenomena (swelling, diffuse redness, papulovesicles, crusts), accompanied by itching.

Livedo angiitis

This type of dermo-hypodermal vasculitis occurs almost exclusively in women, usually during puberty. Its first symptom is persistent livedo - bluish spots of various sizes and shapes, forming a bizarre looping network on the lower extremities, less often on the forearms, hands, face and torso. The color of the spots sharply intensifies upon cooling. Over time, the intensity of livedo becomes more pronounced; against its background (mainly in the area of ​​​​the ankles and dorsum of the feet), small hemorrhages and necrosis occur, and ulcerations form. In severe cases, against the background of large bluish-purple livedo spots, painful nodular seals are formed that undergo extensive necrosis with the subsequent formation of deep, slowly healing ulcers. Patients feel chilliness, nagging pain in the extremities, severe throbbing pain in nodes and ulcers. After the ulcers heal, whitish scars with an area of ​​hyperpigmentation remain.

Diagnosis of skin vasculitis

Diagnosis of skin vasculitis in typical cases does not present significant difficulties; it is based on clinical and anamnestic data (the presence of a previous general or focal infection, taking allergenic medications, exposure to mechanical, physical and chemical agents, prolonged standing, hypothermia, etc.).
In the clinical picture, it is important to determine the degree of activity of the pathological process (Table 2). There are two degrees of activity of the skin process in vasculitis:

— I degree. Skin lesions are limited in nature, there are no general symptoms (fever, headache, general weakness, etc.), there are no signs of involvement in pathological process other organs, laboratory parameters - without significant deviations from normal values.

— II degree. The process is disseminated in nature, general symptoms are noted, signs of a systemic process are identified (arthralgia, myalgia, neuropathy, etc.)

To confirm the diagnosis in the case of a persistent course of the disease or its atypical variety, it is necessary to conduct a pathohistological examination of the affected area of ​​the skin.

The most characteristic pathohistological signs of cutaneous vasculitis: swelling and proliferation of the endothelium of blood vessels, infiltration of vascular walls and their circumference with lymphocytes, histiocytes, neutrophils, eosinophils and other cellular elements, the phenomenon of leukocytoclasia (destruction of leukocyte nuclei up to the formation of “nuclear dust”), fibrinoid changes in vascular walls and surrounding tissue up to complete or segmental necrosis, the presence of erythrocyte extravasates. The main pathohistological criterion for vasculitis is always the presence of signs of isolated inflammation of the vascular wall.

Differential diagnosis

Differential diagnosis of skin vasculitis is carried out with skin tuberculosis, eczema and pyoderma.

Treatment of skin vasculitis

Treatment Goals

• achieving remission

General notes on therapy

When treating one or another form of cutaneous vasculitis, the clinical diagnosis, the stage of the process and the degree of its activity, as well as concomitant pathology are taken into account.

During exacerbation of vasculitis, patients are advised to bed rest, especially if the lesions are localized on the lower extremities, which should be observed until the transition to the regressive stage.

It is necessary to correct or radically eliminate identified concomitant diseases that can maintain and worsen the course of cutaneous vasculitis (foci of chronic infection, for example, chronic tonsillitis, hypertension, diabetes mellitus, chronic venous insufficiency, uterine fibroids, etc.). In cases where vasculitis acts as a syndrome of any general illness(systemic vasculitis, diffuse disease connective tissue, leukemia, malignant neoplasm etc.), first of all, complete treatment of the underlying process must be ensured.

The drug therapy used should be aimed at suppressing the inflammatory reaction, normalizing microcirculation in the skin and treating existing complications.

Treatment methods for skin vasculitis:

Polymorphic dermal vasculitis

Activity level I

• nimesulide 100 mg
• meloxicam 7.5 mg
• indomethacin 25 mg

Antibacterial drugs

• ciprofloxacin 250 mg
• doxycycline 100 mg
• azithromycin 500 mg
• Hydroxychloroquine 200 mg
• chloroquine 250 mg

Antihistamines

• Levocetirizine 5 mg
• desloratadine 5 mg
• fexofenadine 180 mg

Glucocorticosteroids:

• betamethasone 0.1 cream or ointment, nano
• methylprednisolone aceponate 0.1% cream or ointment,

Activity level II

• prednisolone
• betamethasone

To reduce adverse events during systemic therapy with glucocorticosteroids, the following are prescribed:

Proton pump inhibitors:

• omeprazole 20 mg

Macro- and microelements in combinations:

• potassium and magnesium aspartate 1 tablet each.

Bone and bone metabolism correctors cartilage tissue in combinations:

• calcium carbonate + colecalceferol 1 tablet.

Nonsteroidal anti-inflammatory drugs

• diclofenac 50 mg
• nimesulide 100 mg
• meloxicam 7.5 mg

Cytostatics

• azathioprine 50 mg

Antibacterial drugs

• ofloxacin 400 mg
• amoxicillin + clavulanic acid 875+125 mg

• pentoxifylline 100 mg
• anavenol

• hesperidin+diosmin 500 mg

Antihistamines

• ebastine 10 mg
• cetirizine 10
• Levocetirizine 5 mg

Anticoagulants

• nadroparin calcium

Detoxification therapy

• dextran 200–400 ml

on ulcerative defects with copious purulent discharge and necrotic masses on the surface:

• Trypsin + Chymotrypsin – lyophilisate for preparing a solution for local and external use
• ointment dressing (sterile

antibacterial agents in combinations:

• dioxomethyltetrahydropyrimidine + chloramphenicol ointment

antiseptics and disinfectants:

• silver sulfathiazole 2% cream,

Chronic pigmentary purpura

Quinolines

• Hydroxychloroquine 200 mg
• chloroquine 250 mg

Vitamins

• ascorbic acid+rutoside
• vitamin E + retinol

Angioprotectors and microcirculation correctors

• pentoxifylline 100 mg
• dipyridamole 25 mg

Venotonic and venoprotective agents

• hesperidin+diosmin 500 mg

Externally:

Glucocorticosteroids:

• mometasone furoate 0.1% cream or ointment,
• betamethasone 0.1 cream or ointment,
• methylprednisolone aceponate 0.1% cream or ointment

• troxerutin 2% gel

Anticoagulants:

• heparin sodium ointment,

Livedo angiitis

Activity level I

Nonsteroidal anti-inflammatory drugs

• diclofenac 50 mg
• nimesulide 100 mg

Quinolines

• Hydroxychloroquine 200 mg
• chloroquine 250 mg

Angioprotectors and microcirculation correctors

• pentoxifylline 100 mg
• anavenol 2 tablets.

Venotonic and venoprotective agents

• Hesperidin+Diosmin 500 mg

Vitamins

• ascorbic acid+rutoside
• vitamin E + retinol

Activity level II

Systemic glucocorticosteroids

• prednisolone
• betamethasone

Cytostatics

• azathioprine 50 mg

Angioprotectors and microcirculation correctors

• pentoxifylline 100 mg

Anticoagulants

• nadroparin calcium

Detoxification therapy

Substitutes for plasma and other blood components

• dextran 200–400 ml

Externally:

Glucocorticosteroids:

• betamethasone + gentamicin cream or ointment,
• clioquinol + flumethasone ointment,

Angioprotectors and microcirculation correctors:

• troxerutin 2% gel

Special situations

Therapy for patients with vasculitis during pregnancy is prescribed taking into account the restrictions prescribed in the instructions for the medications.

Indications for hospitalization

• Vasculitis limited to the skin, II degree of activity;
• Vasculitis limited to the skin, stage I activity in a progressive stage with no effect from treatment on an outpatient basis;
• The presence of a secondary infection in the lesions that cannot be controlled on an outpatient basis

Requirements for treatment results

- regression of rashes

Prevention of vasculitis

As preventive measures, risk factors (hypothermia, physical and nervous stress, long walking, bruises) should be excluded. Patients are advised to adhere to healthy image life.

After the treatment, sanatorium treatment using sulfur, carbon dioxide and radon baths to consolidate the results obtained.

IF YOU HAVE ANY QUESTIONS ABOUT THIS DISEASE, CONTACT DOCTOR DERMATOVENEROLOGIST KH.M. ADAEV:

WHATSAPP 8 989 933 87 34

EMAIL: [email protected]

INSTAGRAM @DERMATOLOG_95

Among dermal (superficial) angiitis, polymorphic dermal angiitis, which is the classic form of allergic skin vasculitis and occurs most often (in 37.3% of cases, according to our observations), is of primary importance.

Polymorphic dermal angiitis

Blisters, hemorrhagic spots of various sizes, inflammatory nodules and plaques, superficial nodes, papulonecrotic rashes, vesicles, bubbles, pustules, erosions, superficial necrosis, ulcers, scars are observed. The rash is sometimes accompanied by fever, general weakness, arthralgia, and headache.

The rash that appears usually lasts for a long period (from several weeks to several months), in about half of the cases it tends to recur. The onset of the disease and its relapses are often provoked by acute infectious diseases (sore throat, influenza, acute respiratory infections), hypothermia, physical or nervous overstrain, less often - taking various medications or food intolerance.

Depending on the availability of certain morphological elements rashes distinguish various types of superficial dermal angiitis.

However, often different elements are combined, creating a picture of a polymorphic type of angiitis:

1. The urticarial type, as a rule, simulates the picture of chronic recurrent urticaria, manifesting itself as blisters of various sizes that appear in different areas of the skin.

However, unlike urticaria, blisters with urticarial angitis are persistent, persisting for 1-3 days (sometimes longer). Instead of severe itching, patients usually experience a burning sensation or a feeling of irritation in the skin. Rashes are often accompanied by arthralgia, sometimes abdominal pain, that is, signs of systemic damage.

Examination may reveal glomerulonephritis. Patients also note an increase in ESR, hypocomplementemia, an increase in the level of lactate dehydrogenase, positive inflammatory tests, and changes in the ratio of immunoglobulins. Treatment with antihistamines usually has no effect. Patients with urticarial angiitis are usually middle-aged women. The diagnosis is finally resolved by pathohistological examination of the skin, revealing a picture of leukocytoclastic angiitis.

2. The hemorrhagic type is most typical for superficial angiitis. The most typical manifestation This variant is the so-called “palpable purpura” - edematous hemorrhagic spots-papules of various sizes, usually localized on the legs and dorsum of the feet, easily determined not only visually, but also by palpation, how they differ from other purpuras, in particular with Schamberg’s disease - Majocchi. However, the first rashes of the hemorrhagic type are usually small swollen inflammatory spots that resemble blisters and soon transform into a hemorrhagic rash.

With a further increase in inflammatory phenomena against the background of confluent purpura and ecchymosis, hemorrhagic blisters can form, leaving painful erosions or ulcers after opening. In mild cases, changes may be limited to slightly edematous petechial or small purpuric elements without pronounced subjective sensations.

In disseminated forms, the rash spreads to the thighs, buttocks, torso, and upper limbs. Orthostatism is pathognomonic - worsening of the rash after standing. The rash is usually accompanied by moderate swelling of the lower extremities. Hemorrhagic spots may appear on the mucous membranes of the mouth and pharynx.

The described hemorrhagic rashes that occur acutely after a cold (usually after a sore throat) and are accompanied by fever, severe arthralgia, abdominal pain and bloody stools constitute the clinical picture of Henoch-Schönlein purpura, which is observed more often in children. However, Henoch-Schönlein disease often occurs in adults.

According to our observations, hemorrhagic blisters have become more common, and they may not immediately acquire a hemorrhagic character, since their contents may initially be serous, sometimes purulent. The blisters are usually localized on the front surface of the legs, less often on the back of the feet, have a tense tire, their sizes vary from the head of a match to a chicken egg.

Among other clinical features hemorrhagic type We consider it necessary to highlight the increased incidence of chronic recurrent course of the process and the frequent signs of pronounced laboratory activity, indicating the systemic nature of the disease with latent or subclinical damage to other organs (primarily joints and kidneys).

The pathohistological picture of the hemorrhagic type of dermal angiitis usually corresponds to leukocytoclastic vasculitis, especially in acute cases. The main changes concern postcapillary venules and subpapillary arterioles, in the walls of which edema, swelling of the endothelium, muff-like infiltration with a predominance of segmented neutrophils, and fibrinoid changes are observed. A characteristic feature is leukocytoclasia (disintegration of leukocyte nuclei with the formation of “nuclear debris” and “nuclear dust”).

Along the course of the altered vessels, erythrocyte extravasates are noted. Capillaries and arterioles and venules of the middle dermis may also undergo changes. In chronic forms, the perivascular infiltrate has a predominantly lymphohistiocytic composition with an admixture of polynuclear cells and plasma cells.

3. Lapulonodular type is quite rare. It is characterized by the appearance of smooth, flattened inflammatory nodules of rounded outline the size of a lentil or a small coin, as well as small superficial, mildly swollen, pale pink nodules the size of a hazelnut, painful on palpation.

In rare cases, plaque formation may occur. The rashes are localized on the extremities, usually the lower ones, rarely on the torso, and are not accompanied by pronounced subjective sensations. Patients with this type of dermal angiitis are most often diagnosed with chronic papular dermatoses (lichen planus, granuloma annulare, lichenoid parapsoriasis, sarcoidosis, etc.). Due to the usually chronic course of the process, the perivascular infiltrate during pathohistological examination of the skin often has a predominantly lymphohistiocytic composition with an admixture of polymorphonuclear leukocytes.

4. The papulonecrotic type is manifested by small flat or semi-spherical inflammatory non-scaling nodules, in the central part of which a dry necrotic scab soon forms, usually in the form of a black crust.

When the scab is torn off, small rounded superficial ulcers are exposed, and after the papules are reabsorbed, small “stamped” scars remain. The rashes are located, as a rule, on the extensor surfaces of the limbs, buttocks and clinically completely simulate papulonecrotic tuberculosis.

In all cases, detailed differentiation with tuberculosis is carried out according to known parameters (history, X-ray examination, tuberculin tests, microscopy and culture). Koch's bacilli (VC), pathohistological examination of the skin, and, if necessary, anti-tuberculosis treatment). The papulonecrotic type of angiitis is sufficiently fully covered in the literature, so we will dwell only on some of its features that occurred in our patients.

Along with rashes of typical size, large elements of the same nature may appear. We observed coin-shaped papulonecrotic efflorescence on the legs and dorsum of the feet, which, upon regression, left large “stamped” scars the size of up to a 1-kopeck coin. A combination of papulonecrotic and nodular (nodular) elements is also possible.

Probably, similar cases are closer to the polymorphic type of angiitis. We observed polar types in terms of the prevalence of the lesion: from long-term recurrent papulonecrotic elements on only one leg to generalized rashes that occupied the entire skin, with the exception of the face and scalp.

The pathohistological picture of the papulonecrotic type in most patients corresponded to leukocytoclastic vasculitis; in a number of chronic cases, the perivascular infiltrate had a predominantly lymphohistiocytic composition.

Close to papulonecrotic angiitis in its morphological skin symptoms is atrophying Degos papulosis, which, as a rule, has a poor prognosis for life and is therefore called malignant. This rare but characteristic clinical form of dermal angiitis was first described in 1940 by the Austrian physician Kohlmeier, and in 1942 by the French dermatologists Degos, Delort and Tricot.

In 1952, Degos proposed calling this disease malignant wasting papulosis, or fatal intestinal cutaneous syndrome.

The disease is characterized by very pathognomonic skin rashes and accompanying, subsequently progressive abdominal symptoms.

Skin lesions begin with the appearance of round, hemispherical, edematous, pale pink papules with a diameter of 2 to 5 mm, slightly rising above the level of healthy skin. Their central part gradually sinks and becomes faience-white. Over time, this depression expands and becomes covered with dry white scales and crusts. Elements may increase in size and acquire oval or irregular shapes.

The developed eruptive element consists of two zones: central - sunken, atrophic, bright white, as if corrugated, with loosely fitting scales and crusts - and peripheral - roll-shaped, bright pink, sometimes brownish or bluish, swollen, smooth, often with telangiectasias . The elements are located in isolation and only occasionally tend to merge.

The favorite localization of the rash is the torso and proximal parts extremities, although rashes can occur on any part of the skin, with the exception of the palms, soles and scalp. In rare cases, the mucous membranes are also affected. The rash usually appears in spurts.

The rash is quite persistent. The development cycle of one element, in contrast to ordinary papulonecrotic angiitis, lasts many months. In place of the disappeared papules, smallpox-like scars remain. At the same time, you can see on the skin elements located on different stages evolution. Subjective sensations are usually absent. The appearance of the rash may be preceded by a flu-like prodrome.

Several weeks, months, and sometimes years after the appearance of the rash, patients develop steadily progressive abdominal symptoms in the form of periodically increasing abdominal pain, often accompanied by vomiting, black stools, and fever. Fluoroscopy of the gastrointestinal tract shows no changes; gastroscopy sometimes reveals small erosions.

IN terminal stage a picture of severe peritonitis is observed. Intestinal symptoms are explained by multiple perforations of various parts of the gastrointestinal tract, mainly the small intestine.

The disease most often affects young and mature men. On section, as well as during laparoscopy and laparotomy, numerous depressed round yellowish subserous plaques with a diameter of up to 1 cm are found in the intestinal wall, located mainly in the places of attachment of the mesentery.

Histologically, proliferative endothrombovasculitis of the terminal arteries and veins with extensive foci of necrosis is determined in the skin and intestines. Similar changes, although less pronounced, can occur in the brain, kidneys, bladder, myocardium and pericardium, and therefore it is proposed to call the syndrome not cutaneous-intestinal, but cutaneous-visceral. The disease in the vast majority of cases is fatal, but many years can pass between the appearance of the rash and the death of the patient.

5. The pustular-ulcerative type begins with small vesiculopustules, reminiscent of acne or folliculitis, quickly transforming into ulcerative lesions with a tendency to steady eccentric growth due to the disintegration of the edematous bluish-red peripheral ridge.

A calmer course of the process is possible, without progressive growth of ulcers. The lesion can be localized on any part of the skin, most often on the legs, fingers, in the lower half of the abdomen, usually accompanied by pain and fever. After the ulcers heal, flat or hypertrophic scars remain that retain an inflammatory color for a long time.

6. Necrotic-ulcerative type is the most severe variant of dermal angiitis. It has an acute (sometimes lightning-fast) onset and a subsequent protracted course (if the process does not end quickly in death).

As a result of acute thrombosis of inflamed blood vessels, necrosis (infarction) of one or another area of ​​the skin occurs, manifested by necrosis in the form of an extensive black scab, the formation of which may be preceded by an extensive hemorrhagic spot or bubble.

The process usually develops over several hours, accompanied by severe local pain and fever. The lesion is most often located on the lower extremities and buttocks. Purulent-necrotic scab persists for a long time. The ulcers formed after its rejection have different sizes and shapes, contain purulent discharge, and slowly scar.

7. The polymorphic type is characterized by a combination of various eruptive elements characteristic of other types of dermal angiitis. More often there is a combination of edematous inflammatory spots, hemorrhagic purpuric rashes and superficial edematous small nodes, which constitutes the classic picture of the so-called three-symptom Gougereau-Duperre syndrome and the identical polymorphonodular type of Ruiter arteriolitis.

Thus, the various types of polymorphic dermal angiitis differ from each other mainly by clinical and morphological features, on the basis of which we distinguished them in our clinical classification cutaneous angiitis, while their pathohistological structure is of the same type and depends more on the stage and nature of the disease than on its clinical form.

Chronic pigmentary purpura

Based on generalized literature data and our own material, depending on the clinical characteristics, we consider it advisable to distinguish the following varieties (types):

1) petechial type (persistent progressive pigmentary purpura of Schamberg, progressive pigmentary dermatosis of Schamberg) - the main disease of this group, characterized by multiple small (point) hemorrhagic spots without edema (petechiae) resulting in persistent brownish-yellow spots of hemosiderosis of various sizes and shapes ; rashes are most often located on the lower extremities, are not accompanied by subjective sensations, and are observed almost exclusively in men;

2) telangiectatic type (telangiectatic purpura Majocchi, Majocchi disease) is most often manifested by peculiar medallion spots, the central zone of which consists of small telangiectasias (on slightly atrophic skin), and the peripheral zone of small petechiae against the background of hemosiderosis, i.e. here elements of Schamberg's disease are associated with telangiectasias;

3) lichenoid type (pigmented purpuric lichenoid angiodermatitis of Gougerot-Blum) is characterized by disseminated small lichenoid shiny, almost flesh-colored nodules, combined with petechial rashes, spots of hemosiderosis and sometimes small telangiectasia, which suggests Schamberg-Majocca disease with lichenoid rashes ;

4) eczematoid type (eczematoid Doukas-Kapetanakis purpura) is characterized by the presence in the lesions, in addition to petechiae and hemosiderosis, of eczematization phenomena (swelling, diffuse redness, papulovesicles, crusts), accompanied by severe itching.

Dermo-hypodermal angiitis

Since in these cases they are affected blood vessels, located on the border of the skin itself and subcutaneous tissue, and both more superficial (dermal) and deep (hypodermal) arteries and arterioles can additionally be involved, we considered the most appropriate name for this group - “dermo-hypodermal angiitis”.

In this group, we distinguish two clinical forms: nodose arteritis and livedoangiitis. The most characteristic pathohistological feature of these clinical forms is damage to larger blood vessels with the development of fibrinoid changes in their walls, up to segmental necrosis, and a significant proportion of polymorphonuclear leukocytes in the perivascular infiltrate.

Nodose arteritis is the “male version” of the cutaneous form of polyarteritis nodosa. Its manifestations are very monomorphic and are usually represented by dermo-hypodermal nodes of various sizes, located in different areas of the skin.

They are often localized along large blood vessels. Nodes at the beginning of development can only be determined by palpation. Patients find them when palpating in places where pain appears. They are of a dense consistency, round, not sharply contoured, from the size of a pea to a hazelnut, the skin over them, as a rule, is not changed.

Over time, they become larger and bulge slightly above the surface of the skin, the color of which may become bluish-pink. However, more often the nodes remain barely noticeable when examined by eye and are well identified by palpation. They are usually very painful, which is the main complaint of patients. Nodes are most often localized on the extremities (legs, thighs, shoulders), less often on the torso, but can occur on any part of the skin.

As a rule, the disease affects boys and young men whose examination reveals signs of a systemic disease (arthralgia, fever, general weakness, pallor of the skin, weight loss, abnormal test results). The process is characterized by a recurrent course with exacerbations in the cold or transitional seasons.

Livedoangiitis occurs almost exclusively in women, usually occurring during puberty. Its first symptom is persistent livedo: bluish spots of various sizes and shapes, forming a bizarre looping network on the lower extremities, less often on the forearms, hands, face and torso. The color of the spots sharply intensifies upon cooling.

Over time, the intensity of livedo becomes more pronounced; against its background (mainly in the area of ​​​​the ankles and dorsum of the feet), small hemorrhages and necrosis occur, and ulcerations form.

In severe cases, against the background of large bluish-purple livedo spots, painful nodular seals are formed that undergo extensive necrosis with the subsequent formation of deep, slowly healing ulcers. Patients feel chilliness, nagging pain in the extremities, severe throbbing pain in nodes and ulcers. After the ulcers heal, whitish scars remain with a zone of hyperpigmentation around them.

Livedoangiitis is not uncommon. Although puberty and young age are typical for its onset, it can also occur at other ages. age periods. In one of our patients it began in three years old, the other - at 57 years old.

Along with softened forms, manifested mainly by persistent reticulate or branched livedo, we also observed very severe cases diseases with extensive necrosis and long-term non-healing ulcers.

In one of the already mentioned patients, where the process began at the age of three and manifested itself for 20 years as widespread branched livedo, after severe and prolonged hypothermia, gangrene of the distal extremities developed, ending in the amputation of several fingers and toes. Factors that provoke the onset or exacerbation of the disease, in addition to hypothermia, are often pregnancy, gynecological operations, tonsillitis, and acute respiratory infections. The onset of the disease may be preceded by a prolonged low-grade fever.

Almost all patients at one stage or another of the disease have more or less pronounced laboratory activity (increased ESR, decreased hemoglobin, number of red blood cells and platelets, increased total protein, hypergammaglobulinemia).

The pathohistological structure of skin lesions depends on the duration of the disease and the stage of development of the process, but in all cases it fits into the picture of dermal and/or hypodermal vasculitis of varying severity.

Usually there is swelling of the vascular walls, swelling and proliferation of the endothelium, infiltration of the vascular walls and their circumference with lymphocytes, histiocytes, fibroblasts, plasmacytes, and neutrophils. The latter can form accumulations like abscesses. The walls of the vessels thicken, undergo fibrinoid changes, and often become hyalinized. The lumen of the vessels can narrow, become obliterated and thrombose.

In cases of moderate livedo, the effects of vasculitis are minor and are limited to swelling of the vascular endothelium, swelling of the walls and a small perivascular lymphohistiocytic infiltration.

Hypodermal angiitis. Erythema nodosum

Perhaps over time the term “angiitis nodosum” will be adopted, but for now the designation “erythema nodosum” remains more convenient in practical terms. It is customary to distinguish between acute and chronic erythema nodosum. Separately, they describe a common clinical variant - “migratory erythema nodosum”, which usually has subacute course.

At the same time, there are cases of erythema nodosum of short duration, differing in clinical presentation and course from the polar types of both acute and chronic forms, which we propose to call subacute erythema nodosum, including migratory erythema nodosum.

Thus, the principle of classifying erythema nodosum according to the course of the process (according to the severity of inflammatory phenomena and the duration of the process) will be fully observed.

Naturally, this division seems somewhat arbitrary to us, but at the same time one cannot help but note its certain logic:

1. Acute erythema nodosum is a classic, although not the most common, variant of the disease. It manifests itself as a rapid rash on the legs (rarely on other parts of the extremities) of bright red, swollen, painful nodes the size of a child’s palm against the background of general swelling of the legs and feet.

There is an increase in body temperature to 38-39 ° C, general weakness, headache, arthralgia. The disease is usually preceded by a cold or a sore throat. The nodes disappear without a trace within 2-3 weeks, successively changing their color to bluish, greenish (“blooming bruise”). There is no ulceration of the nodes. Relapses, as a rule, are not observed.

This is how the process proceeded in almost all of our patients. Pathohistological changes were localized mainly in the subcutaneous tissue, while usually only nonspecific perivascular infiltration was found in the dermis. In fresh lesions, scattered accumulations of lymphoid and histiocytic cells (sometimes with an admixture of neutrophils and eosinophils) were observed, mainly between the fat lobules.

There were no abscesses or necrosis in the adipose tissue. Small veins and small blood vessels of the interlobular septa were involved in the process, which was expressed by inflammatory infiltration of their walls and proliferation of the intima. In older lesions, the infiltrate was almost exclusively lymphohistiocytic in composition, with occasional giant cells present.

2. Subacute erythema nodosum differs from acute erythema nodosum by its tendency to protracted current, less pronounced inflammatory reaction in the foci, milder general phenomena and less high laboratory activity.

In approximately half of patients with a subacute course of the process, the so-called migratory erythema nodosum is observed. It can sometimes be considered as a transitional form between subacute and chronic erythema nodosum.

Migratory erythema nodosum usually has a subacute course and peculiar dynamics of the main lesion. The process, as a rule, is asymmetrical in nature and begins with a single flat node on the anterolateral surface of the leg.

The node has a pinkish-bluish color, a pasty consistency and quite quickly increases in size due to peripheral growth, soon turning into a large deep plaque with a sunken and paler center and a wide swell-like, more saturated peripheral zone. It may be accompanied by single small nodes, including on the opposite shin; the lesion persists from several weeks to several months. General phenomena are possible (low-grade fever, malaise, arthralgia).

Histological examination, along with the phenomena of infiltrative-productive vasculitis of small hypodermal vessels, as a rule, reveals a widespread inflammatory infiltrate along the interlobular septa, which apparently causes the eccentric growth of lesions.

3. Chronic erythema nodosum is one of the most common forms cutaneous angiitis - characterized by a persistent recurrent course, usually occurs in middle-aged and elderly women, often against the background of general vascular and allergic diseases, chronic focal infection and inflammatory or tumor processes in the pelvic organs (chronic adnexitis, uterine fibroids).

Exacerbations more often occur in spring and autumn and are characterized by the appearance of a small number of bluish-pink dense, moderately painful nodes the size of a forest or Walnut. At the beginning of their development, the nodes may not change the color of the skin, may not rise above it, and can only be determined by palpation.

The almost exclusive localization of nodes is the lower leg (usually their anterior and lateral surfaces). There is moderate swelling of the legs and feet. General phenomena are inconsistent and poorly expressed. Relapses last several months, during which some nodes may resolve and others appear to replace them.

In chronic erythema nodosum, the pathomorphological picture is similar to that in late lesions of the acute type. Along with the phenomena of hypodermal vasculitis of medium and small vessels, thickening of the walls and proliferation of the endothelium of the capillaries of the interlobular septa are often encountered.

Among the cellular infiltrate, in addition to lymphoid and histiocytic elements, giant cells are often found. In some patients, the appearance of clearly defined nodes is often preceded by a rather long prodromal period, consisting of general weakness, malaise, feeling of “tired legs.”

To these nonspecific symptoms later swelling of the legs and feet occurs. The described prodromal period can be prolonged due to the masking of the first nodes by edema, as well as due to the absence of inflammatory coloration of the nodes at first.

Among other features noted in our patients with chronic erythema nodosum, it is worth emphasizing the provoking role in the development of the disease, in addition to foci of chronic infection, also of previous benign tumor or stenotic processes in the pelvic area.

In 2/3 of women with chronic erythema nodosum, we noted a high frequency of pregnancies and childbirths in the anamnesis, which often occurred with complications (in particular, in the form of postpartum thrombophlebitis). At the same time, many patients experienced hereditary predisposition to vascular pathology (the presence of cardiovascular diseases in blood relatives, primarily parents - hypertension, stroke, myocardial infarction, thrombophlebitis, vascular dystonia, etc.).

There was also an increased frequency in the family history tumor diseases(in particular, the death of parents from cancer of different localizations), which indirectly indicates relative inferiority immune system. Apparently, the complex of the listed factors contributes to the emergence and chronic course this type of erythema nodosum.

Ulcerative angiitis in a broad sense can be considered as an ulcerative form of chronic erythema nodosum, but with very characteristic clinical features that bring it closer to erythema induratum.

The process from the very beginning has a torpid course and is manifested by dense, rather large, slightly painful bluish-red nodes, prone to decay and ulceration with the formation of sluggish scarring ulcers. The skin over fresh nodules may have a normal color, but sometimes the process can begin with a bluish spot, which transforms over time into a nodular seal and ulcer.

After the ulcers have healed, hard or retracted scars remain, the area of ​​which can again thicken and ulcerate during exacerbations. The typical location is the back surface of the legs (calf region), but the nodes can also be located in other areas. Characterized by persistent pastiness of the legs. The process has a chronic relapsing course, observed in middle-aged women, sometimes in men.

The clinical picture of nodular-ulcerative angiitis often completely simulates erythema induratum of Bazin, for which the most careful examination should be carried out. differential diagnosis.

Half of the patients had previous or current inflammatory, tumor or other stenotic processes of pelvic localization (hernia repair, cesarean section, appendectomy or uterine amputation, fracture of the pelvic bones, septic abortions, chronic adnexitis, difficult labor with postpartum phlebitis, uterine fibroids, ectopic pregnancy) . Frequent pregnancies have been observed in many women. Local injuries play a major role in the occurrence of nodes.

Laboratory activity is variable, often low. However, sometimes there is an increase in ESR, moderate leukocytosis or leukopenia with neutrophilia and lymphopenia. Laboratory abnormalities usually correlate with the progressive stage of the process and the degree of its severity.

Pathohistological examination of the lesions revealed a picture of infiltrative-productive, often granulomatous hypodermal, vasculitis with thickening and obliteration of arterioles and veins of the subcutaneous tissue, phenomena of its fibrosis, the formation of epithelioid cell infiltrates between the fat cells with an admixture of lymphoid and giant cells foreign bodies or Pirogov-Langhans along the periphery, with small foci of necrosis.

The perivascular and vascular infiltrate was predominantly lymphohistiocytic in nature. We consider it reasonable to classify nodular-ulcerative angiitis as a group of granulomatous vasculitis.

The results of histochemical studies confirm the inflammatory-allergic nature of vascular damage in various types angiitis of the skin, which is accompanied by an increase in the permeability of the vascular walls with their plasmatic impregnation, degranulation of mast cells, thickening and disintegration of the basement membrane of blood vessels, splitting of their elastic framework, destruction of the collagen complex, with the accumulation of acidic and neutral mucopolysaccharides.

When studying the ultrastructural features of the affected skin, the main attention is usually paid to the state of the vascular walls during angiitis. It is necessary to note the uniformity of detected ultrastructural changes in various clinical types of skin angiitis. First of all, attention is drawn to the constant and significant changes in vascular endothelial cells.

Endothelial cells appeared swollen and contained an increased number of micropinocytotic vesicles. On the free surface of endothelial cells, a significant number of cytoplasmic projections were detected. An increase in the number of ribosomes was also noted.

The perinuclear space was sometimes expanded. The basement membrane of the vessels was often thickened, and in places destroyed and split. The process, as a rule, was localized in postcapillary venules with a minimal muscular layer and a significant number of pericytes.

Occasionally, gaps were observed between endothelial cells. Among the infiltrate cells, neutrophils and mononuclear cells usually prevailed. In the lumen of the vessels, there were accumulations of platelets, sometimes penetrating between the endothelial cells.

The described changes were more pronounced in polymorphic dermal angiitis. Thus, electron microscopy data indicate varying degrees of damage to the vascular wall in certain clinical variants of cutaneous angiitis.

Nasonov E.L., Baranov A.A., Shilkina N.P.