Manifestations of angioedema
Angioedema (synonym: Quincke's edema) - limited, with clear edges edema with primary localization in the face, limbs, genitalia.
Angioedema - polytheologicala disease associated with both immunological and non-immunological factors. Depending on the reasons that caused angioedema, the latter is divided into angioedema, allergic, angioedema, anaphylactoid edema, angioedema, angioedema, idiopathic angioedema, angioedema, angioedema, compression angioneurotic edema after physical exertion, angioedema edema, cold. For some forms of angioedema, hereditary transmission of the defect has been proved, and it is often impossible to establish the etiologic factor. A secondary mechanism of angioedema development of individual nosological forms is possible.
The pathogenesis of angioedema is associated withdilated vessels of the deep layer of the dermis, mainly venules. Vasodilators are mediators of immediate allergies, kinins, prostaglandins. Mediators are isolated from mast cells and basophils during degranulation; due to the activation of the alternative path of system complement; when forming immune complexes; as a result of violations of the synthesis of tissue mediators from arachidonic acid.
Possible angioedema, edema, eyelids, lips,periorbital fiber, larynx, mucous membranes. Angioedema may be accompanied by nausea, vomiting, diarrhea, headache. It is most common in middle-aged women.
The clinic of certain forms of angioedema has its peculiarities.
Differential diagnosis of angioedema
Differential diagnosis of angioedemaEdema is carried out with cardiac and renal edema, myxedema, hyperhidrosis in Parkhon syndrome, obstruction of the superior vena cava, Melkerson-Rosenthal syndrome, lymphatic edema, progressive edematous exophthalmos.
Scheme of examination of the patient with angioedemaedema includes the identification of the etiological factor, the features of the immunological homeostasis, which promote the development of angioedema, the nosological form in the secondary character of angioedema. With acute angioedema, the establishment of the cause in most cases is not difficult - it is found when collecting an allergic anamnesis. With a chronic recurrent course of angioedema, radiography of the chest is necessary; carrying out of allergological diagnostics with food allergens and allergens microbial; the setting of an allergological diagnostic test provocative cold; whey protein electrophoresis; determination of factors of rheumatoid and LE cells; a blood test with the definition of a leukocyte formula; repeated studies for the presence of helminths; sowing feces for dysbiosis; skin biopsy.
Allergic angioedema, an angioedema, is caused by an allergic reaction of an immediate type and is one of its most characteristic clinical manifestations.
Etiology of allergic angioedema
Allergic angioedema can be caused by medications (especially penicillin and other antibiotics), food allergens, pollen, chemical. poison of insects.
Clinic of allergic angioedema
Allergic angioedema oftenis combined with hives or other allergic manifestations of food allergy and drug allergy; with asthma bronchial, spastic colitis, attacks of headache. Itchy skin can be expressed (when combined with urticaria) or absent. Allergic angioedema develops a few minutes after the KOH flact with an allergen, antibodies belonging to immunoglobulin E, against the allergen are detected in the blood, allergy can be transferred to the RPCA, and moderate eosinophilia may be present in the blood. Clinical manifestations disappear within a day or two. Of particular danger is the allergic angioedema of the larynx, which occurs when the hymenoptera bite the mucous membrane of the mouth, the tongue, and the region of the pharyngeal ring.
Treatment of allergic angioedema
Effective use of antihistamines(orally or parenterally, depending on the severity of the symptoms), intravenous calcium chloride and glucocorticosteroid preparations according to vital signs (laryngeal edema). With the recurrent nature of the allergic angioedema, examination is necessary for the presence of helminths.
Anaphylactoid angioedema -angioedema, caused by the release of mediators of immediate allergy (histamine, prostaglandins, etc.) due to non-immunological mechanisms. The main etiological factor of anaphylactoid angioedema is drug substances. Anaphylactoid angioedema occurs as a result of a direct degranulant effect on mast cells and basophils of tubocurarine, morphine, penicillamine. The same mechanism, as well as the activation of complement-dependent reactions, underlies anaphylactoid angioedema as a side effect of radiocontrast iodide preparations. Edema can occur immediately after the use of drugs in combination with other manifestations of anaphylactoid reaction up to a lethal outcome or develop gradually over several hours. In the latter case, the manifestations are local in nature.
In the basis of others. The mechanism of development of anaphylactoid angioedema is the dysfunction of prostaglandins, which occurs after the use of non-steroidal anti-inflammatory drugs (acetylsalicylic acid, analgin, deligil, indomethacin, etc.) as a result of their action on the enzymes of the synthesis of prostaglandins from arachidonic acid. Anaphylactoid angioedema of this type appears within 40 minutes or several hours after taking the drug, has a dense, sometimes woody, character and lasts for several days. Typical localization in the area of the upper lip. It is most often found in women, combined with rhinitis with a vasomotor, asthmatic triad. Antihistamines are not effective.
This species is extremely rare andhas a hereditary character (it is transmitted by an autosomal dominant type). Vibration angioedema appears even in early childhood in the form of sharp itching of cutaneous and edema 1-2 min after vibration. The reaction is reproducible in the laboratory: when the forearm is shaken for a few minutes, its vibrational angioedema appears. Dermographism and reaction to compression are not pronounced. The mechanism of development of vibrational angioedema is not clear. It is accompanied by histaminemia.
Idiopathic angioedema, an angioedema, associated with a hereditary defect of the inhibitor of the first component of the complement of the system.
The defect is inherited by an autosomal dominant typewith a penetrance of 100%, occurs in one person out of 10 thousand. The inhibitor is a9-neuraminoglycoprotein mole. weighing 106,000 daltons, contains 40% carbohydrates, half of which is neuraminic acid. It inhibits the activity of Clr, Cls components of complement, plasmin and kallikrein. There are two possible variants of the defect: a decrease in the inhibitor level and a weakening of its functional activity at a normal level. In view of the insufficient effect of the inhibitor, the activity of component C1 is increased.
Idiopathic angioedema can beReproduce the purified components of CI, Cls and a kinin-like polypeptide isolated from the blood of patients with idiopathic angioedema. Increasing the level of component C1 in patients causes an increase in the binding of C2 and C4 components, which are substrates of C1. The level of C4 is lower in patients during remission and with idiopathic angioedema it is not determined, C2 level during remission is normal, but decreases with an attack. However, idiopathic angioedema does not occur in individuals with deficiency of C2 and C4, i.e., besides the system complement for the genesis of the disease, other factors presumably influencing the fibrinolytic and coagulation systems are important.
In the blood and urine of patients with idiopathicangioedema is determined by an increased content of histamine, in the serum - a reduced amount of α-globulin. The severity of symptoms depends on the degree of decrease in inhibitor activity and manifests itself at 5-30% of its activity in comparison with the norm.
Clinic of idiopathic angioedema
Idiopathic angioedemais characterized by dense dough-like whitish edema, without itching, pain, and hyperemia. The development of idiopathic angioedema is associated with trauma, compression of the tissue (eg with a handshake), minor bruising, hypothermia, emotional stress, menstrual cycle. The most common are women of young and middle age. The growth of idiopathic angioedema occurs within 48-72 hours, followed by a spontaneous reverse development within three to four days. In the absence of exacerbation, the patients are practically healthy.
The greatest danger is idiopathicangioedema of the larynx, often developing in patients with idiopathic angioedema and often causing their death from asphyxia. The lethal outcome is most likely between 30-40 years. An idiopathic angioedema of the mucous membrane of the gastrointestinal tract is possible, which is manifested by severe pain in the abdomen, vomiting of bile, watery diarrhea. This localization of idiopathic angioedema can mimic the picture of the "acute abdomen", while there is no rigidity of the abdominal wall, fever and leukocytosis. Unjustified surgical intervention can in such cases cause progression of idiopathic angioedema, up to a lethal outcome.
Treatment. Patients should be warned about the danger of the disease, the possibility of developing asphyxia and the need for medical supervision during the relapse. In contrast to angioedema, allergic in idiopathic angioedema, antihistamines and glucocorticosteroids are not effective enough. A good effect is obtained by transfusion of native plasma, which compensates for the defect of the inhibitor. When a threat of asphyxia requires tracheostomy.
Prevention. With the preventive purpose in the absence of exacerbation, a long oral administration of methandrostanolone and its analogues (in particular, danazol) as well as e-aminocaproic acid is indicated. Patients should avoid provoking factors: trauma, hypothermia, cold food. Particular care is needed when removing teeth, surgical interventions.
Complement dependent angioedema, angioedema, caused by immunocomplex mechanisms of tissue damage.
Complement dependent angioedemaleading to serum sickness, collagen diseases, allergic vasculitis, occurs as a result of disorganization of components of the system complement, activation of an alternative pathway, disturbance of fibrinolytic and coagulation systems. When a biopsy of the affected area is observed necrotizing venules of the vessels of the dermis. Perhaps a combination with immunopathology of the kidneys.
Angioedema from compression
Angioneuroticeski swelling from the compression - angioedema, which occurs as a result of compression of the tissue and is not associated with a deficiency of the inhibitor of the first component of the complement of the system.
For angioedema from compressionis characterized by the involvement of deep layers of the dermis. It is often painful, can be combined with urticaria, develops within 4 hours from the moment of compression, lasts 6-24 hours. Mechanisms of formation of angioedema from compression are not defined, in some cases there is an increase in the level of histamine in the serum.
Angioedema after physical exertion
Angioneuroticeski swelling after exercise - angioedema, associated with physical exertion.
The mechanism of angioedema development afterphysical activity is not clear. It is supposed to be associated with histamineemia and an imbalance in vegetative regulation. An increase in tolerance to loads in patients after regular running has been noted.
Angioedema is a cold swelling - angioedema, caused by cooling of the skin as a result of cold air or water.
Angioedema edema cold bearsrecurrent nature, can be combined with edema of the mucous membrane of the gastrointestinal tract. Angioedema of the cold is most often found in middle-aged women, usually occurs in open areas of the skin (face, hands when cooled, accompanied by an unremarkable itching cutaneous.) In patients, there is a positive allergological diagnostic test provocative cold, in some cases it is possible to transfer allergy to cold in the FPGA, in the blood the content of histamine is increased, sometimes positive allergological diagnostic tests of skin with microbial allergens are noted. the association of the angioedema edema with chronic inflammatory processes of the gallbladder, liver, gastrointestinal tract, the presence of other focal foci of infection.The antihistamines are not effective at all times, during the period of remission, histaglobulin is shown, in some cases hyposensitization to cold with hydrotherapy with gradually decreasing temperature The secondary character of the angioedema of the cold in case of collagen diseases (lupus erythematosus red, arthritis rheumatoid, Sjögrena syndrome), lymphoproliferative diseases. Angioedema of the cold is often combined with parietal dysproteinemia: cryoglobulinemia. cryofibrinemia, a syndrome of increased blood viscosity.