Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042109 (urticaria)
 6,569 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In summary, HAE is a dominantly inherited form of angioedema which is manifested by nonpainful, nonerythematous, nonpruritic and nonpitting swelling of the extremities, face, gastrointestinal and respiratory tracts unaccompanied by urticaria. These patients have deficient activity of the C1 INH and the laboratory diagnosis can be easily made by finding low C4 and C1 inh levels during an attack. Effective and specific therapy is now available that prevents the clinical syndrome and corrects the serologic hallmarks of the disease.
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PMID:Diagnosis and management of hereditary angioedema (HAE). 37 41

The cause of urticaria and angioedema often is difficult to ascertain. In most cases the conditions are transient, but a chronic idiopathic form does occur and may be intractable. Acute urticaria and angioedema usually result from an IgE-mediated mechanism; success in treatment depends on recognition of the underlying factor. Chronic urticaria may ultimately necessitate use of corticosteroids. Hereditary angioedema is easily differentiated from idiopathic angioedema by the family history and absence of pruritus.
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PMID:Urticaria and angioedema. Common clinical problems. 42 57

Urticaria and angioedema are usually the clinical consequence of vasoactive mediators derived from mast cells in the skin or mucosal tissues. Efforts to classify mast cell-mediated causes of urticaria and angioedema have generally been frustrated by their diverse pathogenesis and clinical course. The term acute is typically used to describe fleeting lesions whose recurrence does not extend beyond 6 weeks. Chronic is the term used to describe lesions that persist for more than a few hours but usually less than a day, and recurrences extend for more than 6 weeks. These definitions do not take histology into account. Skin biopsies of fleeting lesions demonstrate a paucity of inflammatory cells, whereas more persistent lesions display a spectrum of perivascular cuffing by predominantly T cells and monocytes. The presence of leukocytoclastic vasculitis in persistent lesions indicates an underlying immune complex disease. Many of the physical urticarias have fleeting lesions that can be induced with the appropriate stimulus for years. This review article has emphasized the clinical course and histology of urticaria and angioedema lesions in an effort to provide a more complete understanding of the pathogenesis and appropriate treatment. Clearly, avoidance of an identifiable inciting stimulus is optimum management, although most patients have no etiology defined or the cause is not realistically avoidable. At present, treatment options for these patients rely on antihistamines to control the immediate consequence of mast cell degranulation. Corticosteroids are reserved for the treatment of patients whose urticaria or angioedema lesions persist, reflecting the increasing involvement of mononuclear cells in the disease process. For leukocytoclastic vasculitis, corticosteroids are indicated, and cytotoxic drugs may be required for adequate treatment. Future treatments of urticaria and angioedema will evolve based on elucidation of the relevant cells and soluble mediators and will include counterregulatory or antagonistic peptides and drugs. C1 esterase inhibitor deficiency is a relatively uncommon cause of angioedema but is important to understand because of its ability to clinically mimic mast cell-mediated angioedemas and its unique pathogenesis and treatment. HAE can be divided into two serologic subtypes that simply reflect the location of the defect in one of the codominantly expressed C1-INH genes on chromosome 11. AAE can be divided into two serologic subtypes. AAE type I is due to massive consumption of C1-INH, presumably by tumor-related immune complexes. AAE type II is due to an anti-C1-INH autoantibody.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Urticaria and angioedema. 161 35

Because urticaria clears spontaneously in most patients, an extensive workup is not advised during the early weeks of an urticarial eruption. Whether and when to perform a screening workup or a more extensive workup depend on the degree of suspicion that the patient is ill, the urgency with which the patient presses for an answer, and the presence or absence of signs or symptoms that might lead the physician to pursue a diagnosis other than chronic idiopathic urticaria. Angioedema may occur with urticaria, and when it does, the prognosis is worse. Whereas urticaria manifests as circumscribed edema involving the superficial dermis, angioedema involves primarily the deep dermis or subcutaneous or deeper layers. Individual urticarial lesions usually disappear within 2 to 4 hours, whereas those of angioedema can persist for 72 hours. The workup for patients with chronic angioedema can be similar to that for patients with urticaria. However, several additional diagnostic possibilities should be pursued in patients with angioedema, such as hereditary angioedema caused by C1-esterase inhibitor deficiency, because anabolic steroids are effective in the treatment of these conditions.
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PMID:Urticaria and angioedema: diagnosis and evaluation. 186 91

Previous reports have suggested that the etiology of chronic urticaria/angioedema (greater than 6 weeks) can be identified 10% to 30% of the time while few reports have addressed the natural history of chronic urticaria/angioedema. An analysis of all patients referred to the authors' practice between 1983-1985 with a diagnosis of urticaria/angioedema was performed. Patients with hereditary angioedema were excluded. Eighty-six of the 214 patients had chronic urticaria/angioedema. In the remaining 128 cases of acute urticaria there were four exercise induced, nine contact, six cold induced, six drug induced, 11 food induced, one viral hepatitis associated, 29 with dermographism, and 62 undetermined. An etiology could not be determined in any of the patients with chronic urticaria/angioedema. Laboratory tests, including CBC, chemistry panel, urinalysis, ANA, rheumatoid factor, complement studies, sedimentation rate, and skin tests were all noninformative. Chronic angioedema without urticaria occurred in only nine cases, 31 cases had chronic urticaria alone, and 46 cases had both chronic urticaria and angioedema. Of the patients followed over the 3-year period, 27 resolved while 48 continued to have urticaria/angioedema. Response to medications was variable and will be discussed. Our study suggests that an etiology is determined in much less than 10% of patients with chronic urticaria; fortunately, 32% of our cases resolved over a 3-year period.
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PMID:The natural history and response to therapy of chronic urticaria and angioedema. 256 92

Urticaria/angioedema is very common and usually not very serious. The main diagnostic task is the history, asking about pharmaceutical agents, foods, focuses of infection, physical agents, and psychogenic factors as well as inhalants, insect bites, internal diseases, immune complex diseases, contactants, and genetic factors. The main therapeutic tool is to eliminate the offending agent. If this cannot be done, therapy should begin with an H1 antihistamine pushed to tolerance or clearing. Life-threatening laryngeal edema and/or anaphylactic shock are extremely rare. Laryngeal edema is usually a component of hereditary angioedema. In such cases, subcutaneous epinephrine is the drug of choice. Laboratory investigation in chronic urticaria should include CBC, erythrocyte sedimentation rate, and a serum multiphasic analysis. A myriad of laboratory tests can be done in chronic urticaria, but some cost-yield effective ones are a test for antinuclear antibodies and x-rays of the sinuses and dentition.
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PMID:Urticaria. 286 29

During the last few years, the structure and function of human C1-inhibitor have been elucidated. Chromogenic substrate assays for determination of C1-inhibitor activity in plasma are available, and have proved to be of value not only for the diagnosis of hereditary angioedema but also in acquired diseases involving C1-inhibitor, such as cold urticaria and autoimmune disorders as well as acute-phase types of disease states.
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PMID:Aspects of C1-inhibitor biochemistry and pathophysiology. 332 41

The correct diagnosis and characterization of C1-inhibitor deficiency depends on both clinical observations and laboratory evaluation of complement in plasma. Rocket immunoelectrophoresis for C4d is a sensitive assay for C4 activation in plasma. We have evaluated the value of this assay in identifying patients with C1-inhibitor deficiency. C4 activation was assessed in the plasmas of 15 patients with hereditary angioedema, five patients with variant form of hereditary angioedema, and four patients with acquired C1-inhibitor deficiency. Control groups consisted of 27 patients with chronic idiopathic urticaria and/or angioedema and seven normal volunteers. C4 activation was detected in all 52 plasma samples collected from the 24 patients with C1-inhibitor deficiency. The degree of C4 activation increased during attacks of angioedema and decreased (but remained elevated) during treatment with attenuated androgens. The concentrations of C4, C2, and C1 inhibitor were also measured; however, none of these measurements identified all of the patients with C1-inhibitor deficiency. Thus, we conclude that the measurement of C4 activation is one of the best tests available to evaluate a patient for C1-inhibitor deficiency, and a normal result will exclude the diagnosis of C1-inhibitor deficiency.
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PMID:The value of rocket immunoelectrophoresis for C4 activation in the evaluation of patients with angioedema or C1-inhibitor deficiency. 378 76

We report two sisters who both have had severe attacks of urticaria and angioedema only when taking the contraceptive pill and during the latter half of pregnancy. Although they exhibited many features in common with hereditary angioedema (HAO), the C1 esterase inhibitor levels and other complement components were consistently normal, including levels measured during pregnancy and at the height of the eruption after oestrogen challenge. Aetiological factors in relation to other patterns and causes of urticaria are discussed.
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PMID:Recurrent angioedema: familial and oestrogen-induced. 380 12

A functional determination of C1 esterase inhibitor (C1 INH) can be easily performed with an amidolytic assay by monitoring the inactivation of plasma kallikrein. In patients with urticaria as well as in healthy donors kallikrein inactivation, determined as the ratio of kallikrein activity at t60/t15, was found to be 0.47 +/- 0.06; in patients with hereditary angioneurotic edema (HANE). However, it amounted to 0.80 +/- 0.06. There was a good correlation between the inactivation rate of kallikrein and the protein levels of C1 INH (r = -0.93, p less than 0.001). In patients with HANE, levels of plasma kallikrein were slightly decreased (mean = 0.37 +/- 0.11 U/ml, normal mean = 0.47 +/- 0.09 U/ml), but still sufficient for monitoring kallikrein inactivation. In the case of one patient with functionally inactive C1 INH protein, the inactivation of kallikrein was impaired as in the conventional form of the disease.
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PMID:A rapid method for functional determination of C1 esterase inhibitor in plasma. 617 50


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