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Query: UMLS:C0042109 (urticaria)
 6,569 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Paroxystic vasomotor skin manifestations are provoked by various etiologies. Widespread or generalized vasomotor skin manifestations may be induced by a physiological reaction (emotinal flushing), by a drug (vasodilator drugs, antabuse, antidiabetic, sulfonamides), by a discharge of histamine (urticaria, mastocytosis) or by an hypersecretion of serotonin (dumping-syndrome, carcinoid syndrome). They may be caused by an endocrinopathy (menopause, hyperthyroidism, hypoglycaemia, medullary thyroid carcinoma, pheochromocytoma, endocrine pancreas, carcinoma). More rarely vasomotor troubles happen in homocystinuria, inhalation of a toxic (trichlorethylen, calcic cyanamid) and exceptionally in some immunohaematologic diseases. Main localized vasomotor skin manifestations observed are dermographism, facial flushing (Sluder's syndrome, cluster headaches, Frey's syndrome, Riley-Day's syndrome) and acral syndromes (Raynaud's phenomenon, erythromelalgia).
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PMID:[Paroxystic vasomotor skin manifestations (author's transl)]. 8 21

The case is presented of a 3-year-old girl with urticaria and pressure dermographism. The condition began the age of one year. A skin biopsy confirmed the suspected diagnosis of diffuse mastocytosis. The identification of the form of mastocytosis without skin lesions and with dermographism and pressure urticaria as its only signs is important. It should be considered in any chronic urticaria appearing at birth or in an early age which is unresponsive to the usual symptomatic medication.
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PMID:Diffuse dermographic mastocytosis without visible skin lesions. 112 58

Four consecutive patients with systemic mastocytosis were studied. One patient had a malabsorption syndrome with only minor histological changes of the intestinal mucosa. Another patient with ulcer diathesis had a gastric secretory pattern resembling Zollinger-Ellison syndrome. Serum gastrin and histamine levels were consistently normal in all patients. Endoscopy of stomach and colon disclosed urticaria-like papulae either spontaneously or after topical provocation in all patients. No increase of mast cells was found in multiple mucosal biopsies. A markedly increased gastric tissue content of histamine was found, however, in the three patients studied. The findings suggest that urticaria-like lesions associated with a high tissue content of histamine may be more important that hyperhistaminaemia in causing the various gastrointestinal symptoms.
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PMID:Gastrointestinal involvement in systemic mastocytosis. 126 81

A woman born in 1917 presented with recurrent urticaria since childhood. In the course of her life she developed urticaria pigmentosa, followed by generalized mastocytosis involving the bones, gastro-intestinal tract, and liver. At the age of 71 years neurological symptoms of cranial nerves necessitated hospital admission. Within a month a concomitant conus medullaris syndrome caused sphincter dysfunction and sacral sensory disturbances. No cause or secondary abnormalities were found on myelography, CT and MRI of the brain and the spinal cord, and in the CSF.
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PMID:Generalized mastocytosis and neurological complications in a 71-year-old patient. 132 18

Thirty two cases of the association of mastocytosis and bone lesions were collected in a multicentre study. Five cases involved osteocondensation forms. However, most often (27 cases), there was osteoporosis (OP). The diagnosis was made in the absence of obvious risk factors, and thus often in men (2/3 of patients), when there was the association of pigmented urticaria and an excess of mast cells in bone biopsies. Laboratory, radiological and isotope scan findings are often non-specific, being identical to those encountered in common OP. The histomorphometric profile involves an association of decreased cancellous bone volume, increased area of resorption and decreased bone formation parameters. Progression to malignant mastocytosis occurs essentially in diffuse osteocondensation forms and is rare in OP types. Emphasis must be placed on the importance of qualitative study of bone marrow, using specific stains, since the diagnosis may be missed in the absence of typical skin lesions. Conversely, since a simple increase in mast cell count is possible during common OP, a search for mast cell nodules is important in order to establish the diagnosis with certitude.
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PMID:[Mastocytosis and bone manifestations. Results of the survey of the bone and phosphorus-calcium metabolism section of the French Society of Rheumatology]. 157 47

Adolescent patients are often affected by dermographic, cholinergic, cold or solar urticaria brought about by distinct physical stimulation. These physical forms of urticaria as well as cutaneous and systemic mastocytosis are due to the release of inflammatory mediators from the mast cells. As these diseases usually show characteristic cutaneous symptoms, they can easily be distinguished from other subtypes of urticarial reaction. In order to make the reader familiar with physical urticaria and mastocytosis, we describe the clinical features, pathogenic factors, and therapeutic possibilities of these disorders in adolescence.
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PMID:[Urticaria and mast cells in adolescence]. 169 47

Urticaria affects 15% to 20% of the population once or more during a lifetime. Chronic urticaria is a frequent recurrent eruption over a period greater than 6 weeks; the cause remains a mystery in more than 75% of cases. Urticaria and angioedema may be produced by immunologic or nonimmunologic means. Urticarial vasculitis, contact urticaria, mastocytosis, physical urticarias, dermatographism, cholinergic urticaria, localized heat urticaria, cold urticaria, aquagenic urticaria, and vibratory angioedema all require specific evaluation and treatment. Chronic idiopathic urticaria is usually controlled by antihistamines; depending on the circadian rhythm of the eruption, sedative or nonsedative antihistamines are prescribed. Some patients will require a combination of H1 and H2 antagonists, or even parenteral corticosteroids.
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PMID:Chronic urticaria. 197 Jun 97

We studied four patients (a mother, her two daughters, and her son) with bullous mastocytosis, or diffuse cutaneous mastocytosis, whose genetic inheritance suggested an autosomal dominant pattern. The clinical characteristics included extensive bullae, numerous urticaria, pruritus, flushing, and pseudolichenified skin over all body surfaces without systemic organ involvement. The histopathologic findings disclosed a pronounced accumulation of mast cells in the dermis. Electron microscopic studies of lesional skin obtained in infancy showed round or spindle-shaped mast cells with numerous fingerlike villous protrusions. The cytoplasmic granules varied in size and shape, and the appearance of degranulation was markedly noted. In the adult, most mast cells had markedly decreased numbers of granules and cytoplasmic villi. Some cells displayed degenerative or necrotic appearances. These findings correlated well with the clinical course of these cases, which improved spontaneously over time.
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PMID:The familial occurrence of bullous mastocytosis (diffuse cutaneous mastocytosis). 224 Dec 1

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

Mastocytosis is a disease characterized by an increase in the number of tissue mast cells and a concomitant increase in mast cell-derived mediators. To demonstrate the spectrum of skin disease in mastocytosis in the pediatric population, five children with mastocytosis and complaints of urticaria (4/5), bullae/vesicles (3/5), abdominal pain (3/5), flushing (2/5), headache (1/5), and bone pain (1/5) are reviewed. Confirmation of the diagnosis of cutaneous mastocytosis was obtained by histologic examination of a biopsy of lesional skin; however, mast cell numbers in lesional skin did not correlate with plasma histamine levels or the extent of cutaneous involvement. Mastocytosis is a diagnosis that must be recognized in the differential diagnosis of pediatric urticarial diseases.
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PMID:Mastocytosis in infants and children: recognition of patterns of skin disease. 292 86


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