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)

While the hereditary angioneurotic edema is a rare but serious disorder 10 to 25% of the population experience urticaria during a lifetime. Urticaria is for the patient a very impressive disease. He therefore has a great desire to know its cause. In acute urticaria this is usually possible since drugs or specific foods are the most common triggers. In chronic urticaria the search for a cause is much more difficult and successful only in 20 to 30% of cases. Over the past years it has been proven that about 30% of patients with chronic urticaria have antibodies against the high affinity Fc-receptor of mast cells. Thus a fraction of patients with chronic urticaria formerly often associated with psychosomatic illness suffer in fact from an autoimmune disorder.
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PMID:[From psychosomatic disorder to autoimmune disease--50 years urticaria and Quincke edema]. 981 26

Angiotensin-converting enzyme (ACE) inhibitor associated angioedema was detected in 39 subjects (17%) of 231 consecutive patients examined in the last 5 years at our out-patient clinic for symptoms of angioedema without urticaria. In these patients, angioedema was most commonly localized to the face. The duration of ACE-inhibitor treatment at the onset of angioedema ranged from 1 day to 8 years with a median of 6 months. The time elapsed between onset of angioedema and withdrawal of ACE-inhibitor ranged from 1 day to 10 years with a median of 10 months. Delayed diagnosis is explained by the unusual characteristics of this adverse reaction: angioedema may start years after beginning the treatment and then it recurs irregularly. In fact, ACE-inhibitors seem to facilitate angioedema in predisposed subjects, rather than causing it with an allergic or idiosyncratic mechanism. Thus, while Cl-inhibitor levels are usually normal in subjects developing ACE-inhibitor-dependent angioedema, we found that ACE-inhibitors caused angioedema in Cl-inhibitor-deficient patients. Because the main inactivator of bradykinin is kininase II, which is identical with ACE, it is believed that bradykinin mediates ACE-inhibitor-dependent angioedema. We had the possibility to examine the plasma bradykinin levels in one ACE-inhibitor-treated patient during an angioedema attack and we found very high levels, but we did not find an increase of break-down products of high-molecular-weight-kininogen as observed during acute attacks in hereditary angioedema. Bradykinin fell to normal levels during remission after withdrawal of the drug. These observations indicate that in ACE-inhibitor-induced angioedema, contrary to hereditary angioedema, the reduction of bradykinin catabolic rate plays a predominant role.
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PMID:Angioedema due to angiotensin-converting enzyme inhibitors. 1060 20

Congenital C1-inhibitor deficiency, or hereditary angioneurotic edema (HAE), is a rare autosomal dominant disease due to alterations in the C1 inhibitor gene that results in a deficiency of antigenic and/or functional C1-INH. Affected patients are heterozygous, and their deficiency is incomplete, many of them having up to 20% of the normal amount of the inhibitor. The disease is characterised by recurrent, circumscribed, non-pitting, and non-pruritic subepithelial swellings of sudden onset, which fade during the course of 48-72 hours, but can persist up to 1 week. Lesions can be solitary or multiple and primarily involve the extremities, larynx, face, and bowel wall. Bradykinin is believed to be the main, but certainly not the sole, mediator responsible for the bouts of edema in HAE. The diagnosis is suggested by family history, the lack of accompanying pruritus or urticaria, the presence of recurrent gastrointestinal attacks of colics, and episodes of laryngeal edema. Diminished C4 concentrations during symptomatic periods are highly suggestive for the diagnosis. Further laboratory diagnosis depends on demonstrating a deficiency of C1-INH antigen (type I) in most kindreds, but some kindreds have an antigenically intact but dysfunctional protein (type II) and require a functional assay to establish the diagnosis. Prophylactic administration of either attenuated androgens or protease inhibitors has proved useful in reducing frequency or severity of attacks. Infusions of a vapour-heated C1-INH concentrate are safe and effective means of both preventing and treating attacks. Nevertheless, this treatment is expensive and this extract is not readily available. It is emphasised that administration of angiotensin converting enzyme inhibitors is contraindicated in patients suffering from protease inhibitor deficiency states.
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PMID:Hereditary angioneurotic edema: review of the literature. 1078 4

Isolated angioedema, without urticaria or itching, occurs as a result of an inherited or acquired defect in C1 esterase inhibitor activity. Most cases of isolated angioedema are caused by one of two types of hereditary angioedema (HAE). We present a case of the much rarer type II HAE with abdominal pain as the sole presenting symptom. Hereditary angioedema should be suspected in young adults with episodic abdominal pain for which common causes have been excluded. A history of HAE or episodic abdominal pain in family members is not necessary for diagnosis.
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PMID:Hereditary angioedema as a cause of transient abdominal pain. 1174 47

The incidence of cutaneous effects of oral contraceptives (OCs) is estimated at 2.7-5%. Secondary effects directly attributable to the hormonal action of OCs include melasma, acne and hyperseborrhea, alopecia, and cutaneous lesions of vascular origin. Melasma or chloasma accounts for about 2/3 of all cutaneous side effects of OCs. It appears from 1 month-3 years after the start of OC use, its frequency increasing with dose and duration of use. Pigmentation appears to accentuate the symptoms in brunettes rather than predisposing them to melasma. Exposure to the sun plays a certain role, but use of a low dose OC and effective sun protection are not enough to reverse the pigmentation. These melasmas regress more slowly than after pregnancy and many remain definitive. The influence of OCs on acne is variable, with some OCs provoking sebaceous hypersecretion and some improving acne enough to be used for treatment. For the therapeutic effect to be observed, the estrogen dose must be sufficient to offset the androgenic effect of the progestin. Combined pills containing the strong antiandrogen cyproterone acetate should control acne if other, less androgenic progestins fail. Alopecia is a very rare effect of OCs and its appearance may even reflect simple coincidence. Vascular complications of combined OCs are dependent on estrogens and may include such manifestations as telangiectasias, angiomas, and livedo reticularis. Some secondary cutaneous effects are probably not due to a hormonal influence. They are less well known than the direct hormonal effects, and publications concerning the often detail isolated observations that are difficult to interpret. Reactions of hypersensitivity or allergy to combined OCs may include urticaria and eczema. A history of OC use should be sought in all women presenting with erythema nodosum and the OCs should be discontinued. Pruritus and jaundice may be observed in 1 OC user in 100,000. They indicate a cholestatic hepatitis for which estrogens are responsible. Most patients developing the condition have already had pruritus or jaundice during pregnancy; such a history contraindicates OC use. Several dermatological and systemic disorders are aggravated by OC use. Hereditary angioedema, herpes gestationis, porphyries, and systemic lupus erythematosus are exacerbated by OC use. The role of OCs in malignant melanomas remains controversial.
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PMID:[Dermatological complications caused by oral contraceptives]. 1234 76

Angioedema without an associated urticarial syndrome evokes a completely different differential diagnosis from urticaria. This review of the literature discusses hereditary angioedema as prototype of angioedema without urticaria. The review then establishes a differential diagnosis for angioedema, which includes allergic contact dermatitis, connective tissue disease, endocrine associations, parasitic disease, tumor masses, and miscellaneous causes for angioedema. Angioedema without urticaria is a distinct syndrome differing from chronic urticaria. The astute clinician should be familiar with the spectrum of disorders ranging from a functional or quantitative deficiency in C1-esterase inhibitor to a panoply of cutaneous and internal medical disorders. Angioedema without urticaria is a symptom in which there are many different disease mechanisms producing subcutaneous swelling recognizable as angioedema.
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PMID:Differential diagnosis of angioedema. 1247 44

A 6-year-old boy and a 3.5-year-old girl presented with unexplained episodes of angioedema without urticaria. Low serum C1 esterase inhibitor activity was found in both children. Family history revealed autosomal dominant inheritance in the girl. The boy had a negative family history for angioedema. C1 esterase inhibitor deficiency is a rare but serious condition that may cause oedema of the upper respiratory tract and death by asphyxiation. Episodes of angioedema occur spontaneously, usually subsiding within 48-72 h. Between episodes, the patients are symptom free. Treatment consists of substitution of synthetic C1 esterase inhibitor during episodes of edema carrying a risk of upper airway obstruction. In patients who have more than one episode of severe angioedema per month, daily treatment with tranexamic acid should be considered. Both of these patients were not receiving daily treatment.
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PMID:[Episodes of angioedema in children with C1 esterase inhibitor deficiency]. 1687 73

Monoclonal gammopathy is a condition characterized by the abnormal proliferation of a single clone of plasma cells, which produces a homogeneous monoclonal protein. It has been reported to occur in association with urticaria in the context of Schnitzler's syndrome and also has been observed to occur in angioedema with acquired C1 esterase inhibitor deficiency. We report 11 cases of monoclonal gammopathy presenting to practicing allergists (>2.5% of those screened) primarily in association with dermatologic disorders, i.e., urticaria, angioedema, and nonspecific dermatitis, but also with allergic respiratory disorders, i.e., allergic rhinitis, chronic sinusitis, and asthma. Most of the patients with dermatologic manifestations had respiratory disorders as well, three with chronic sinusitis. To our knowledge, these are the only such cases reported in patients with urticaria or angioedema in the absence of Schnitzler's syndrome or C1 inhibitor deficiency or in association with chronic sinusitis, allergic rhinitis, or asthma. Monoclonal gammopathy, angioedema, urticaria, allergic respiratory disorders, and sinusitis could be linked through antigenic stimulation as a trigger, either infectious, as in chronic sinusitis; self-antigens, as in autoimmunity; or the monoclonal gammopathy itself, causing idiotype-anti-idiotype immune complexes and inflammatory disease. The allergist, dermatologist, otolaryngologist, and primary care physician should all maintain a high index of suspicion for the occurrence of monoclonal gammopathy in the "allergic" population. Serum protein electrophoresis and/or serum immunofixation are useful screening tools. When monoclonal gammopathy is found, the presence of light chains in the urine should be assessed and the patient should be referred for prompt hematology-oncology evaluation with periodic monitoring for the development of plasma cell dyscrasias. Additional prospective study is necessary to determine the true prevalence of monoclonal gammopathy in the population presenting to the practicing allergist.
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PMID:Monoclonal gammopathy in association with allergic disorders of the skin and respiratory tract. 1672 32

Angioedema can be a symptom of anaphylaxis; it may be more hazardous that the circulatory collapse in otherwise healthy patients. Angioedema can be part of IgE- and histamine-mediated allergic reactions or part of NSAID-induced hypersensitivity with disturbances in arachidonic acid metabolism. If angioedema occurs without urticaria or other symptoms of anaphylaxis, it is usually mediated by increased bradykinin synthesis (HANE, EANE) or reduced metabolism (ACE inhibitors). These observations have led to new therapeutic approaches in HANE. Icatibant is a bradykinin-receptor-2 antagonist and blocks bradykinin-induced angioedema in HANE. How applicable this will be to ACE-inhibitor angioedema remains to be seen.
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PMID:[Angioedema]. 1800 29

Urticaria is a rash, that typically involves skin and mucosa, and is characterized by lesions known as hives or wheals. In some cases there is an involvement of deep dermis and subcutaneous tissue that causes a skin/mucosa manifestation called angioedema. Urticaria and angioedema are very often associated: urticaria-angioedema syndrome. The acute episodic form is the most prevalent in the pediatric population, and it is often a recurrent phenomenon (recurrent urticaria). Acute episodic urticaria it is usually triggered by viruses, allergic reactions to foods and drugs, contact with chemicals and irritants, or physical stimuli. In many instances it is not possible to identify a specific cause (idiopathic urticaria). Chronic urticaria is a condition that can be very disambling when severe. In children is caused by physical factors in 5-10% of cases. Other trigger factors are infections, foods, additives, aeroallergens and drugs. The causative factor for chronic urticaria is identified in about 20% of cases. About one-third of children with chronic urticaria have circulating functional autoantibodies against the high affinity IgE receptor or against IgE. (chronic urticaria with autoantibodies or "autoimmune" urticaria). It is not known why such antibodies are produced, or if the presence of these antibodies alter the course of the disease or influence the response to treatment. Urticaria and angioedema can be symptoms of systemic diseases (collagenopathies, endocrinopathies, tumors, hemolytic diseases, celiachia) or can be congenital (cold induced familiar urticaria, hereditary angioedema). The diagnosis is based on patient personal history and it is very important to spend time documenting this in detail. Different urticaria clinical features must guide the diagnostic work-up and there is no need to use the same blood tests for all cases of urticaria. The urticaria treatment includes identification of the triggering agent and its removal, reduction of aspecific factors that may contribute to the urticaria or can increase the itch, and use of anti-H1 antihistamines (and/or steroids for short periods if antihistamines are not effective). In some instances an anti-H2 antihistamine can be added to the anti-H1 antihistamines, even if the benefits of such practice are not clear. The antileucotriens can be beneficial in a small subgroup of patients with chronic urticaria. In case of chronic urticaria resistant to all the aforementioned treatments, cyclosporine and tacrolimus have been used with good success. When urticaria is associated to anaphylaxis, i.m epinephrine needs to be used, together with antihistamines and steroids (in addition to fluids and bronchodilatators if required).
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PMID:Urticaria and urticaria related skin condition/disease in children. 1870 Mar 29


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