Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with one year duration post-scabious nodules are reported. Lymphoid hyperplasia was found histologically and a malignant lymphoma was suspected. Six patients with pseudolymphomatous scabious nodules were found in the literature and of these only one had evidence of lymphoid follicle formation with germinal centres. Short lived and persistent scabious nodules are discussed and diagnosis with lymphomatous nodules, dermatofibroma and urticaria pigmentosa considered. A "Darier's sign" was elicited on rubbing the lesions which is probably related to the increased number of mast cells in the infiltrate and may explain the persistent pruritus. Histological criteria to separate cutaneous lymphoplasia from lymphoma are reviewed and other more common causes of lymphadenosis benigna cutis are considered. Cutaneous lymphoplasia in persistent scabious nodules is possibly an indirect evidence of the importance of immunological factors in scabies.
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PMID:[Persistent scabietic nodules with cutaneous lymphoplasia]. 103 87

The mast cell, equipped with enzymes, chemotactic factors, a vasoactive amine, an anticoagulant, and lipid-derived proinflammatory products, may be essential in tissue modeling as well as in defense. Its primarily perivascular location in skin and the mucosa of the respiratory tract and the gut assures its availability to counter parasites. By the same token, the mast cell is responsible for interactions with inhaled, ingested, and injected antigens that comprise IgE-mediated allergic reactions. Abnormally high numbers of mast cells in the skin, either localized or generalized, result in urticaria pigmentosa or generalized cutaneous mastocytosis, respectively. Tissue infiltration by excessive mast cells, primarily in gut, bone, liver, and spleen, results in systemic mastocytosis; this may be accompanied by myelodysplasia or lymphoma and may eventuate in mast cell leukemia. Until the etiology of mastocytosis is understood, the treatment is symptomatic: histamine antagonism by H1 +/- H2 blockade for flushing, itching, and gastric distress; cyclooxygenase inhibition to prevent prostaglandin D2 (PGD2)-induced hypotension when indicated; and oral cromolyn to prevent gastrointestinal symptoms and bone pain.
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PMID:Mast cell disease. 149 Jun 22

The onset of mastocytosis occurs between birth and 2 years of age in approximately 55% of all cases; an additional 10% develop the disease before the age of 15 years. Mastocytosis in these age groups differs in many respects from mastocytosis that has its onset in adulthood. The typical presentation of pediatric-onset mastocytosis consists of cutaneous manifestations: either a solitary mastocytoma, urticaria pigmentosa, or, less commonly, diffuse cutaneous mastocytosis. Particularly in infants, bullous eruptions may occur. Mastocytosis in infants and children may involve internal organs, including the bone marrow and the gastrointestinal tract, although such manifestations appear to be less common in children than in adults. Plasma histamine levels may be elevated in pediatric-onset mastocytosis. Treatment usually involves the use of H1 and H2 antihistamines to control itching and to control the hypersecretion of gastric acid that may occur. The prognosis for children with mast cell disease is variable; approximately half of the children with urticaria pigmentosa may experience resolution of lesions and symptoms by adolescence.
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PMID:Pediatric mastocytosis. 170 49

Systemic mast cell disease (SMCD) is a rare disease often associated with symptoms of general malaise, pruritus, diarrhea, vomiting, fever, urticaria pigmentosa, hepatosplenomegaly and lymphadenopathy. We reported a case of SMCD associated with cutaneous xanthoma and serum hyper IgE. Skin biopsy revealed xanthomas and diffuse infiltration of mast cells in the dermis. The association of SMCD with xanthoma was reported in the literature for only one case. The hyper IgE could be due to the defect of IgE receptors on the cell membrane of mast cells of dysfunction of T and/or B cell. Any of the treatment using H1 and H2 receptor blockade, disodium cromoglycate, adrenocorticosteroid or chemotherapy (VEPA) were not effective. The patient died of pulmonary edema and multiple organ failure 7 months after the diagnosis was established. The crush method for the cytological examination of bone marrow was considered more useful than smear method for the diagnosis of SMCD.
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PMID:[Systemic mast cell disease associated with cutaneous xanthomas and markedly elevated serum IgE]. 224 20

Neutrophil and eosinophil chemotactic activities (NCA and ECA) were measured in serum from twenty-two patients with urticaria pigmentosa or systemic mastocytosis. NCA was also measured after heating serum to 56 degrees C (heat-stable NCA). Although these factors were increased in about half of the patients there was no correlation with histamine release as estimated by the excretion of the main histamine metabolite methylimidazoleacetic acid (MelmAA) in urine. A significant increase in heat-stable NCA, however, was found in patients with pruritus and abnormal high values of MelmAA. It is concluded that only heat-stable NCA is a specific mast cell mediator, but that the heat-labile NCA and ECA are dependent on mast cells for their production by a different cell, tentatively identified as the macrophage.
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PMID:Serum levels of neutrophil and eosinophil chemotactic activities in mastocytosis. 247 24

The diagnosis of systemic mastocytosis without urticaria pigmentosa has been made with increasing frequency since modern methods of histamine assay have been used clinically. We examined the incidence of urticaria-angioedema and mastocytosis over a recent 12-month period. Of 490 new patients we saw, 52 had urticaria-angioedema, and ten had evidence of excess histamine +/- PGD2, with at least ten mast cells per high-power field on skin biopsy. The average age was approximately 35 years; the male:female ratio was 1:4 for urticaria-angioedema and 1:2 for mastocytosis. Symptoms of mastocytosis included flushing, abdominal cramping/diarrhea, syncope, urticaria-angioedema, pruritus, and headache. Symptoms have typically been prevented by a combination of H1 and H2 antagonists, with addition of a cyclo-oxygenase inhibitor in syncopal cases. Acute hypotension has responded to epinephrine.
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PMID:Mastocytosis: one year's experience. 287 59

We report on the clinical picture of urticaria pigmentosa adultorum and systemic mastocytosis. In four out of five patients, we proved systemic mastocytosis by means of a biopsy from the iliac crest, although these patients did not complain of systemic signs and symptoms. In all cases, the cutaneous changes and clinical symptoms could be improved by a four-week-heliotherapy in the North Sea summer climate; itching did not reappear but five months later. Heliotherapy is regarded as an useful alternative concerning the treatment of urticaria pigmentosa and cutaneous manifestation of systemic mastocytosis.
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PMID:[Urticaria pigmentosa adultorum and systemic mastocytosis--diagnostic principles and therapeutic possibilities]. 342 99

Topical corticosteroids are utilized in the treatment of a wide variety of skin diseases, primarily those involving an inflammatory component. Recent investigations have revealed that one of the effects of long-term usage of steroids is the depletion of skin mast cells. This led to the treatment of patients with urticaria pigmentosa with topical high potency corticosteroids for 6 weeks. At the end of treatment there was a marked reduction in tissue histamine and an absence of mast cells as well as a disappearance of pruritus and Darier's sign. Treated areas remained clinically improved for at least 9-12 months. Observations that corticosteroids profoundly affect mast cells in vivo provides a rationale to devise new treatment regimens for mast-cell-related diseases.
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PMID:Aggressive topical corticosteroid therapy: a novel approach to mast-cell-dependent cutaneous disorders. 367 48

Alpha-fluoromethylhistidine, alpha-FMH (L-641.575, Merck, Sharp and Dohme), a specific irreversible inhibitor of the mammalian histidine decarboxylase, was investigated for its effect on possible histamine symptoms in mastocytosis. Two patients were treated for 28 and 22 weeks respectively. The first patient had systemic mastocytosis and a severe malabsorption causing weight loss, excessive fecal fat losses and electrolyte disturbances, the main symptom being frequent diarrhoea. The second patient had mainly skin manifestations diagnosed as urticaria pigmentosa and the main symptom was pruritus. There were no side effects of the drug. The first patient produced formed stools after one week of treatment, concomitant with a decrease in plasma histamine and in urinary excretion of the main histamine metabolite tele-methylimidazoleacetic acid (MeImAA). In the second patient definite benefit of the treatment was observed after two months. However, changes in histamine parameters occurred earlier. Plasma histamine and urinary MeImAA were reduced after one week and in two or three weeks reached a steady-state level of about 25% of pretreatment values. The results indicate an effective inhibition of histamine synthesis in both patients but only the diarrhoea seemed to be causally related to the change in histamine metabolism.
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PMID:Treatment of two mastocytosis patients with a histidine decarboxylase inhibitor. 401 13

10 adult patients with symptomatic urticaria pigmentosa were treated with ketotifen versus placebo in a double-blind cross-over study. A highly significant reduction of the daily symptom scores for pruritus and whealing was found with 2 mg ketotifen daily per os, and, compared to oral disodium cromoglycate, ketotifen was much more effective. Tiredness was the only side effect noted in 40% of the patients, and a transient worsening of symptoms was noted after discontinuation of the drug.
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PMID:A double-blind cross-over study of the effect of ketotifen in urticaria pigmentosa. 634 Nov 2


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