With hives there is swelling
With hives on the skin appear blisters -pale itchy papules and plaques due to short-term edema of the dermis. Quincke's edema is characterized by swelling of the dermis and subcutaneous tissue, which captures vast areas. Both diseases can have acute or chronic recurrent course. Urticaria and Quincke's edema occur both together and separately. With hereditary Quinck's edema, urticaria is extremely rare.
Synonyms: urticaria - urticaria; Edema Quincke - oedema Quincke, angioedema, giant urticaria.
Every sixth person (according to other estimates - one in four) at least once in a lifetime suffers urticaria or angioedema. In 25% of patients with urticaria, it sooner or later becomes chronic.
Urticaria and angioedema may be due toallergic reactions (in which the main role is played by IgE and complement), physical factors (cold, sunlight, pressure) and idiosyncrasy. The development of the syndrome, known as the periodic edema of Quincke with eosinophilia, is associated with the pathogenic action of the main basic protein of eosinophils. Drug hives - see p. 588, contact urticaria - see p. 50.
Classification and mechanisms of development Allergic reactions
• Allergic reactions of immediate type. IgE is mediated. Appear on food allergens (milk, eggs, wheat, crustaceans, mollusks, nuts), medicines (penicillins - see page 588) and antigens of parasites. Patients often suffer from other allergic diseases.
• Immunocomplex allergic reactions. Immune complexes activate complement with formation of fragments СЗа and С5а, which cause degranulation of mast cells. Observed with serum sickness, whole blood transfusion, the introduction of immunoglobulins.
Urticaria dermographism. The appearance of blisters with mechanical skin irritation is observed in 4.2% of people. Harmless and painless phenomenon. Blisters disappear for 30 minutes (Figure 15-3).
• Cold urticaria. Usually occurs in children and young people. The diagnosis allows you to put a provocative test with a piece of ice.
• Solar urticaria. Occurs under the action of ultraviolet radiation with a wavelength of 290-500 nm; one of the mediators is histamine.
• Cholinergic urticaria. With intense physical exertion, small blisters appear at the same time with the sweat (Figure 15-4).
• Quincke's vibratory edema. In the history - swelling, provoked by pressure or vibration (swelling of the buttocks during sitting, swelling of the hand after work with a hammer, swelling of the feet after walking). Laboratory examination of pathology does not reveal; There is no fever. Means with antihistamine activity are ineffective, and corticosteroids often bring relief. When a skin biopsy reveals a significant lymphocytic infiltration of the deep layers of the dermis. To the swelling of Quincke can join and urticaria. Quincke's vibratory edema can be familial (inherited by autosomal dominant) and sporadic in nature. The appearance of edema is explained by the release of histamine from mast cells under the influence of vibration. So, rubbing the back with a towel causes swelling, and a constant pressure with the same force, but without friction - no.
Pseudoallergic (anaphylactoid) reactions. Hives, Quincke's edema and even anaphylactic shock can be caused by drugs releasing histamine (in particular, radiopaque), and inhibitors of the synthesis of pro-staglandins (salicylates, azo dyes and benzoic acid salts). For details, see p. 588.
Five minutes after the skin was held with a wooden stick, blisters appeared. The patient had suffered from generalized itching for several months, but he still did not have urticaria
Urticary vasculitis. Urtkarny vasku-lit - this is vasculitis of the skin, which is accompanied by the appearance of blisters. Unlike other forms of hives, the size, shape and location of the blisters vary slowly. Blisters last 12-24 hours and longer. With urticarious vasculitis, there is often a hemorrhagic rash and residual hyperpigmentation caused by the deposition of hemosiderin. The disease is often combined with a deficiency of complement components and kidney diseases. In addition, it occurs in systemic lupus erythematosus and Sjogren's syndrome. For details, see p. 392.
Hereditary edema of Quincke. This severe disease is inherited autosomal dominant and is manifested by facial swelling (Figure 15-5) and limbs, laryngeal edema and acute abdominal pain due to edema of the intestinal mucosa. Hives usually do not, but there are rashes that resemble rheumatic ring-shaped erythema. A laboratory study reveals a decrease in
85%) or activity (in 15%) of the inhibitorC1-esterase, a decrease in C4 level and normal levels of complement components C1 and C3. Edema develops due to excessive production of bradykinin, since the C1-esterase inhibitor is at the same time an inhibitor of factor XII and kallikrein, the two enzymes involved in the synthesis of kinins.
Periodontal edema of Quincke with eosinophilia. Quincke's severe swelling is characteristic; urticaria, which is accompanied by severe itching and covers the face, neck, limbs and trunk; fever and a significant increase in weight (by 10-18%) due to water retention.
The disease lasts 7-10 days. Internal organs are not affected. A laboratory study reveals high leukocytosis (from 20,000 to 70,000 μg1) and eosinophilia (60-80% of eosinophils). The more severe the disease, the stronger the change in laboratory parameters. Family history is not burdensome. The forecast is favorable.
Acute urticaria (less than 30 days). Characterized by large blisters and a combination with edema Quincke. Allergic reactions of immediate type (allergy to food products, parasites or penicillins, allergic diseases in the anamnesis) or immunocomplex allergic reactions (serum sickness, whole blood transfusion, the introduction of immunoglobulins, penicillins) are at the basis of allergic reactions. Chronic urticaria (more than 30 days). Almost never is mediated by allergic reactions of immediate type. In 80-90% of patients the cause remains unexplained. Such urticaria is called idiopathic. The emergence is often promoted by emotional overload. Intolerance of salicylates, salts of benzoic acid is noted. Mostly adults are sick, women - 2 times more often. About 40% of patients with hives lasting more than 6 months continue to suffer from it in 10 years.
The rashes last for several hours.
Itching, pain when walking (with damage to the soles), hot flashes, burning, choking.
Hoarseness, stridor, shortness of breath (Quincke's swelling). Fever (serum sickness, periodic Quincke's edema with eosinophilia). Arthralgia (serum sickness, ur-tic vasculitis, hepatitis B).
• Small blisters: short-lived, multiple (1-2 mm diameter rash is typical for cholinergic urticaria, Figures 15-4).
• Blisters: 1 to 8 cm in diameter (Figure 15-6).
• Quincke's Edema: enlargement of the face (eyelids, lips, tongue) or limb without changing skin color (Figure 15-5).
Colour. Pale pink; large blisters have a white center and a reddish rim along the periphery.
The form. Oval, ring-shaped, arc-shaped, polycyclic, quaint.
Location. Rashes form rings, arcs, lines (Figure 15-3). Term of life. Few hours. Localization. The defeat can be local, regional and generalized (see Figure 22-2). Favorite localization: places subject to compression, the action of sunlight (solar urticaria); trunk, hands and feet, lips, tongue, ears.
Insect bites, drug urticaria, contact urticaria, urticarial vasculitis, hepatitis B.
Every patient needs to exclude systemic lupus erythematosus, urticarial vasculitis, lymphoma.
The epidermis is not changed. Edema of the dermis and subcutaneous tissue and dilated venules without signs of vascular damage, without "nuclear dust" (neutrophil disintegration) and without diaplegis of erythrocytes. Degranulation of mast cells. In the perivascular space - T-helpers, carrying antigens HLA-DR. On endothelial cells, the factor of intercellular adhesion of ICAM 1 and E-selectin is detected, and on the cells of the perivascular space - the VCAM 1 vascular cell adhesion factor.
Determination of hepatitis B virus antigens. Radioallergosorbent test (detection of IgE antibodies to the allergen).
Increased ESR and deficiency of complement componentswith urticaria vasculitis. Short-term eosinophilia in allergic reactions to food, parasitic and medicinal allergens. High leukocytosis and eosinophilia with periodic edema of Quincke with eosinophilia.
Measure the level and function of the C1-esterase inhibitor.
With abdominal pain, it can reveal the swelling of the intestinal mucosa.
Allows to exclude parasitic disease.
After 30 minutes of intensive exercise, the skin on the neck turned red (an axon-reflex) and small blisters appeared on it
Diagnostic signs of chronic urticariaare presented in Table. 15-A. Find out what medicines the patient took (including aspirin and other NSAIDs). When suspected of hives caused by physical factors, resort to provocative tests. Methods for diagnosing cholinergic urticaria - physical activity before the appearance of sweat and intradermal injection of acetylcholine or methacholine (cause the appearance of small blisters). The solar urticaria is diagnosed with a photographic test with UV-A, UV-B and visible radiation. For the diagnosis of cold urticaria, a piece of ice or a test tube with iced water is applied to the skin. If the blisters hold for more than 24 hours, you need to suspect the dermal vasculitis and conduct a skin biopsy. Periodic edema of Quincke with eosinophilia is manifested by fever, high leukocytosis (mainly due to eosinophils) and weight loss due to water retention. Another characteristic feature is the cyclicity of the disease: exacerbations occur at intervals of several years. With the hereditary edema of Quincke, first, family history is burdensome; secondly, edema of the face and limbs can be caused by a minor injury; thirdly, abdominal pain is observed and, fourth, the levels of the complement component of C4 and the C1-esterase inhibitor are reduced.
With allergic reactions of immediate typeinteraction of the allergen with IgE antibodies fixed on the surface of basophils and mast cells leads to the activation of these cells and the release of histamine, leukotrienes and prostaglandins. These substances increase the permeability of venules and cause the release of biologically active substances from other cells.
In immunocomplex allergic reactionsthe complement is activated by immune complexes. This is accompanied by the release of anaphylatoxin-nov - fragments of C3a and C5a, which cause degranulation of mast cells.
In the pathogenesis of chronic urticaria, the main roleare removed by histamine released from the mast cells. It is believed that other mediators, including eicosanoids and neuropeptides, also participate in the development of the edema of the dermis, but direct evidence has not yet been obtained. Intolerance to salicylates, various food preservatives and additives (for example, benzoic acid and sodium benzoate) and some azo dyes (tartrazine and orange food color added to soft drinks) appears to be due to abnormal arachidonic acid metabolism.
From serum of patients with idiopathic chronicurticaria was isolated biologically active substance, which causes the appearance of blisters. This substance has a molecular weight of about 1 million, refers to class G immunoglobulins and releases histamine from both basophils and mast cells (Hide M. et al., Auto-antibodies against the high-affinity IgE receptor as a cause of histamine release in chronic urticaria, N. Engl, J. Med. 1993, 328: 1599). Further studies have shown that it is an IgG autoantibody against the a-subunit of high-affinity IgE receptors (anti-PCect autoantibody). These autoantibodies were detected in the serum of 48% of the examined patients; moreover, the higher the ability of serum to release histamine, the more severe the chronic urticaria. The remission that occurs in the treatment of such patients with plasmapheresis, cyclosporine, and / or the introduction of immunoglobulin is currently explained by neutralization of anti-Pc.Yu-autoantibodies. In favor of the hypothesis of autoimmune lesion of mast cells, a frequent (in 14% of cases) combination of chronic urticaria with chronic lymphocytic thyroiditis also testifies.
With the hereditary edema of Quincke, a decrease in the level of the C-1-esterase inhibitor or its functional insufficiency leads to an enhanced synthesis of kinins.
The cause of the periodic edema of Quinckeeosinophilia is believed to infiltrate the dermis with eosinophils. Development of Quincke's edema and hives accompanied by increased eosinophilia, and remission - its weakening. In eosinophils, morphological changes are observed up to complete destruction. The contents of the destroyed eosinophils enters the dermis, the main basic protein of eosinophils is deposited in collagen bundles and causes degranulation of the mast cells.
Approximately half of the patients who survivedurticaria without Quincke's edema, there are no relapses during the first year; in 20% of them the disease lasts more than 20 years. The prognosis for hives and swelling of Quincke is favorable. An exception is the hereditary edema of Quincke, which, without treatment, can result in death.
Attempts to eliminate the provoking factor - chemical substance or drug (aspirin, food additives) - are rarely crowned with success, especially with chronic urticaria.
Means with antihistaminic activity Begin withH, -blockers, for example, hydroxysin or fexofenadine. If they are ineffective, add H2-blockers (cimetidine) and drugs that stabilize the mast cells (ketotifen). Treatment with doxepin (tricyclic antidepressant with antihistamine activity) is justified only in those cases when severe urticaria is accompanied by anxiety and depression.
It is indicated for periodic edema of Quincke with eosinophilia.
With the hereditary edema Quincke is shownlong-term treatment with danazol. In case of exacerbations, freshly frozen plasma is poured or a concentrate of C1-esterase inhibitor is administered (Kunschak M. A randomized, controlled trial to study the efficacy and safety of the Cl. Inhibitor concentrate in treating hereditary angioedema.Transfusion, 1998, 38: 540).
Severe swelling of the face during exacerbation (A); next - a patient during remission (B). Looking at these two pictures, it's hard to guess that this is the same person
Diagnostic signs of various forms of chronic urticaria
If you look closely at the photograph, you can see that the blisters have a pale middle and a reddish color along the edges