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)

A case of systemic mastocytosis is reported with an observation period of 20 years. During these two decades multiple manifestations of the disease appeared including urticaria pigmentosis, episodic histamine release, gastro-intestinal involvement and hepatosplenomegaly. The most extraordinary, and possibly unique phenomenon, has been the development of a massive proliferation of large mastocytomas mainly, but not exclusively, limited to the lower extremities. For different reasons (mechanical disability, bleeding, cosmesis), these tumors have required repeated admissions for surgical removal. The most successful technique has involved use of the ultrasonic scalpel.
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PMID:Systemic mastocytosis with extensive large cutaneous mastocytomas: surgical management. 393 76

H2-antagonists differ from the commonly applied antihistamines (H1-antagonists) by blocking a different spectrum of histamine-mediated pharmacologic reactions. Their effects on the skin as the target organ may be stronger, weaker, or even reverse. The main representative of this group of drugs is cimetidine. Other compounds are still in experimental stages. Some controversial effects were reported in urticaria, pruritus, atopic dermatitis, mastocytosis of the skin, and also in acne and psoriasis. With polyetiologic symptoms, as are manifested in cases of urticaria and pruritus, the efficacy of the drug may depend on the underlying disease. In acne and psoriasis, the clinical type and stage of the disease may also play a major role in the outcome of such studies. Experimental and clinical findings suggest that cimetidine has some immunomodulating effect in terms of influencing the delayed type skin hypersensitivity. The intake of cimetidine should be registered in patch testing. Application of H2-antagonists may be beneficial in diseases with reduced immune resistance (generalized mycotic infections). Serious group-specific side-effects of H2-antagonists are not yet known. Several side-effects have been reported following oral intake of cimetidine; however, their frequency seems rather low.
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PMID:[H2-antagonists and possibilities for their therapeutic use in dermatology]. 613 53

Mast cells with their stores of vasoactive and chemotactic mediators are central to the pathogenesis of allergic diseases. The cross-linking of receptorbound IgE molecules on the surface of mast cells initiates a complex chain of events, including calcium ion influx, phospholipid methylation and turnover and cyclic nucleotide metabolism, ultimately resulting in the release of mediators of immediate hypersensitivity. These mast cell mediators are important in smooth muscle reactivity, in the recruitment of eosinophilic and neutrophilic leukocytes and in the generation of secondary chemical mediators. Histologic evidence of mast cell degranulation, biochemical evidence of mast cell mediators in blood and tissues and clinical evidence of signs and symptoms reproducible by these mediators have strongly supported the crucial role of mast cells in asthma, urticaria, anaphylaxis, rhinitis and mastocytosis. Because of their unique location at host environment interfaces, mast cells may both participate in allergic diseases and promote homeostasis.
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PMID:Mast cells in allergic diseases and mastocytosis. 629 4

We report on 9 cases of systemic mastocytosis which underline the frequency and the potential severity of this disease. All patients had intercritical signs (usually urticaria). Seven patients had also typical crises with flush and vascular collapses are observed together, doctors should measure histamine blood level and perform correct biopsy.
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PMID:[Systemic mastocytosis: incidence and risks of vasomotor seizures]. 751 68

Cutaneous mastocytosis usually includes objective skin signs such as pigmented maculopapulae or skin infiltration. We report an unusual case of cutaneous mastocytosis without systemic involvement in a 9-month old infant. Clinical expression was entirely functional (pruritus, urticaria) with no permanent lesions. There were 22 times more mastocytes found in the skin biopsy than in similar biopsies obtained in healthy control subjects of the same age, which corresponds to the counts found in cutaneous mastocytosis. Diffuse cutaneous mastocytosis with no permanent lesions is an exceptional form of mastocytosis (3 cases reported in the literature). The long-term outcome is unknown. This syndrome should be distinguished from idiopathic anaphylaxis by quantification of the dermal mastocytes.
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PMID:[Diffuse cutaneous mastocytosis without permanent lesion]. 775 14

Urticaria and physical angioedema frequency is hard to evaluate in children. In this series, we keep 53/1000. Physical urticaria is found in 36% of the cases and concerns cholinergic urticaria, urticaria to cold, idiopathic dermographism and mastocytosis, and delayed urticaria to pressure. The authors insist on the interest of the etiologic research in these forms of infantile urticaria.
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PMID:[Physical urticaria and angioedema in children]. 826 43

Mastocytosis in children are very polymorphic, most often remaining isolated, without an associated visceral lesion. Beside the phenomenon of "urtication" characteristic, papulo-nodular lesions of urticaria and angio-oedema during the mastocytosis are rarely met. In contrast, congestive signs with pruritus, dermographism, congestive erythema and general signs are frequent.
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PMID:[Urticaria and angioedema caused by mastocytosis]. 826 47

We describe the clinicopathologic features of 10 patients with recurrent unexplained flushing. These patients were referred to the National Institutes of Health with a diagnosis of mastocytosis or idiopathic anaphylaxis. Both diagnoses were eliminated after evaluation. Patients reported attacks of flushing lasting 15 minutes to 2 days and associated with such symptoms as anxiety, chest tightness, paresthesia, slurred speech, weakness, and pruritus. Abdominal pain was a constant feature, often associated with cramping and an increase in stool frequency. Attacks witnessed by physicians consisted of an exaggerated blush response of the face and upper part of the chest, and were sometimes associated with tachycardia, mild hypertension, and tachypnea. Hives, angioedema, wheezing, and hypotension were not observed. Routine laboratory studies and 5-hydroxyindoleacetic acid, vanillylmandelic acid, and plasma histamine levels were normal. Plasma histamine levels did not elevate during attacks. When performed, results of bone marrow examinations, skin biopsies, and bone scans were normal. Psychiatric examinations frequently revealed somatization disorders. Patients had often been prescribed a wide variety of medications including antihistamines, nonsteroidal anti-inflammatory drugs, and steroids, with little or no benefit. Despite the benign nature of the clinical and laboratory findings, patients had undergone repeated, often invasive, examinations for several years. Whether such patients have a prominent flush response exaggerated through a somatization disorder or a relatively benign flushing disorder associated with putative mediator release remains to be determined. Recognition of this category of patients with unexplained flushing will avoid subjecting such patients to unwarranted repeated examinations, procedures, and inappropriate therapy.
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PMID:A clinicopathologic study of ten patients with recurrent unexplained flushing. 830 82

Oesophageal leiomyomatosis and idiopathic eosinophilic oesophagitis are both extremely rare. The former is a diffuse proliferation of smooth muscle in the muscularis propria, whilst the latter is an idiopathic inflammatory condition, thought to be associated with background atopy and characterized by an infiltrate of eosinophils throughout the full thickness of the oesophagus. However, two recent cases of oesophageal leiomyomatosis showed similar full thickness infiltration of the oesophageal wall by eosinophils and this inflammatory cell infiltrate was investigated in conjunction with one case of idiopathic eosinophilic oesophagitis. All three had a similar allergic profile characterized by CD45RO-positive primed T-lymphocytes, EG2-positive (i.e., activated) eosinophils, and tryptasepositive mast cells, together with gene expression for interleukin 4. Previous descriptions of leiomyomatosis describe an association with systemic mastocytosis and urticaria and the possibility that there is a common underlying allergic component to both disorders is raised.
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PMID:Full thickness eosinophilia in oesophageal leiomyomatosis and idiopathic eosinophilic oesophagitis. A common allergic inflammatory profile? 939 39

The skin represents a unique immunologic organ poised to protect the host from invading organisms and environmental antigens. The skin is also an important target for a variety of allergic and autoimmune responses. Mast cells are key to the pathogenesis of urticaria, angioedema, and mastocytosis. Atopic dermatitis is the consequence of an immunoregulatory abnormality resulting in a skin-directed T helper type 2 response. Allergic contact dermatitis is an example of classic delayed type hypersensitivity. Circulating autoantibodies against the epidermis are a key mechanism by which bullous skin diseases occur.
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PMID:Allergic and immunologic skin disorders. 939 53


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