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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case is described of a 42-year-old man with urticaria pigmentosa and documented systemic mastocytosis who had 2 episodes of loss of consciousness, headache, nasal stuffiness and faecal incontinence, following a swim in a river. Under controlled conditions the patient was subjected to immersion of the right hand to icewater for a period of 2 minutes, followed by 2 minutes of strenuous exercise. Similar symptoms involving change in conscious state were evoked with accompanying rises in plasma histamine levels. These findings suggest that histamine is at least one of the major mediators of the change in conscious state that is reported rarely in these patients and draws attention to the neurological facets of this rate disorder.
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PMID:Urticaria pigmentosa---change in conscious state associated with rise in plasma histamine levels. 55 Sep 41

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

A patient with urticaria pigmentosa and systemic mastocytosis developed hypotension following indomethacin administration. He then developed further episodes not related to indomethacin. Based upon the experience of others with the management of patients with systemic mastocytosis who showed exceptional reaction to cyclooxygenase inhibition, it was decided to treat him with H1 and H2 blockade followed by aspirin, another cyclooxygenase inhibitor. The procedure was carried out under careful observation with cardiac monitoring. After 160 mg of aspirin, he developed hypotension, tachycardia, and flushing accompanied by difficulty of breathing and headache. A vasoconstrictor drug (levarterenol) was administered. The patient's symptoms subsided, and after 1 hour aspirin was again administered, this time with no side effects. The dosage was increased to 975 mg every 6 hours, and he has had no further hypotensive episodes on this regime for 2 years. Cyclooxygenase inhibition, combined with H1 and H2 blockade, is an effective treatment for this condition, but for these patients initiation of aspirin therapy should be carried out with extreme care.
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PMID:Systemic mastocytosis: management of an unusual case with histamine (H1 and H2) antagonists and cyclooxygenase inhibition. 288 Jun 86

A patient reported that she developed various shock-like symptoms upon intake of alcohol, acetylsalicylic acid, or after injection of contrast medium. After provocation with alcohol applied orally and following endoscopy flush, tachycardia, and a severe headache followed immediately by painful diarrhea were observed. According to our diagnosis the patient had urticaria pigmentosa involving the bone marrow and an enlarged liver and spleen, respectively. The suspected intestinal mastocytosis was confirmed histologically by a biopsy from the jejunum. It was concluded that the symptoms were the result of a direct activation of the accumulated intestinal mast cells. The differential diagnosis of mastocytosis as opposed to allergic gastroenteritis, sprue, and carcinoid is discussed.
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PMID:[Mastocytosis simulating a food allergy]. 686 85

Mastocytosis is a rare disease of mast-cell proliferation with involvement of the reticuloendothelial systems including skin, bone, gastrointestinal tract, liver, lungs, spleen, and lymph nodes. Systemic mastocytosis is characterized by a combination of symptoms that relate to the mast cells' release of vasoactive substances, such as histamine. These symptoms include urticaria pigmentosa, flushing, syncope with hypotension, headaches, nausea, vomiting, diarrhea, and occasional bronchospasm. The diagnosis of mastocytosis is typically based on the presence of the characteristic extraosseus manifestations. A well recognized roentgenographic feature seen in 70-75% of patients with mastocytosis is diffuse osteolysis and osteosclerosis, affecting primarily the axial skeleton and the ends of the long bones. Rarely, the bony involvement consists of generalized osteoporosis, which may lead to pathologic fracture, or solitary lesions (mastocytomas) which may cause symptoms of localized pain. Four patients with previously diagnosed systemic mastocytosis had unusual skeletal lesions. Clinical and laboratory evaluation of these patients eventually led to the correct diagnosis of systemic mastocytosis. We report these four cases to emphasize the need for thorough evaluation of unusual musculoskeletal findings in association with extraosseus symptoms that are characteristic of mastocytosis. Knowledge of a wide differential diagnosis of unusual skeletal lesions should include systemic mastosytosis.
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PMID:Mastocytosis presenting as a skeletal disorder. 912 84

Systemic mastocytosis (SM), as opposed to cutaneous-only mastocytosis, implies the presence of neoplastic mast cell infiltration in extracutaneous tissue. Mast cell disease in adults is often systemic and often involves the bone marrow. Typical clinical and laboratory features of SM include urticaria pigmentosa, mast cell mediator symptoms (eg, headache, flushing, lightheadedness, urticaria and pruritus, nausea, diarrhea, abdominal pain, and vasodilatory shock), bone pain (eg, osteoporosis, lytic bone lesions, and fractures), hepatosplenomegaly, cytopenia, eosinophilia, elevated serum tryptase and histamine, and bone marrow fibrosis and angiogenesis. SM may be indolent (no evidence of organ dysfunction), aggressive (presence of organ dysfunction), associated with another often chronic myeloid hematologic disease (SM-AHD), or present as mast cell leukemia or sarcoma. Mast cell-mediator symptoms are treated with histamine antagonists and cromolyn sodium. Indolent SM does not require cytoreductive therapy. Aggressive SM and SM-AHD are managed based on their molecular profile. Recent information suggests that FIP1-like-1-platelet-derived growth factor receptor-alpha(+) SM responds well to imatinib mesylate, whereas interferon-alpha should be considered as a first-line treatment in all of the other cases, including patients with Asp816Val(+) SM. Cladribine has been shown to be effective in patients who develop resistance to interferon treatment.
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PMID:Systemic mastocytosis: current concepts and treatment advances. 1508 68

Mastocytosis has a bimodal distribution often presenting in children from birth to 2 years of age and in those over the age of 15. Pediatric mastocytosis is due to the effects of mast-cell degranulation enzymes such as histamine and tryptase causing the presentation of pruritis, flushing, vesicles, abdominal and bone pain, or headache. Three different forms of mastocytosis can occur: urticaria pigmentosa, diffuse cutaneous, and solitary mastocytoma. Systemic symptoms are typically a result of mast-cell mediator release but do not prove systemic mast-cell hyperplasia. In this review, we present several research studies related to pediatric mast-cell disorders, and discuss several cases of pediatric mastocytosis, acute myeloid leukemia, pathophysiology, genetic studies, and treatment.
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PMID:Pediatric Mastocytosis: A Review of the Literature. 2438 17

Although the prognosis of maculopapular cutaneous mastocytosis (MPCM), also referred to as urticaria pigmentosa, is often benign, clinicians lack evidence to reliably predict those at risk of associated systemic manifestations. We sought to elucidate clinical markers of disease severity to provide better treatment and prognostic information for individuals with MPCM. A retrospective chart review querying characteristics of children diagnosed with MPCM in the Emory Dermatology Clinic was performed. Follow-up was obtained through a clinical encounter or telephone interview. Linear regression was used to determine predictors of the number of MPCM-related systemic symptoms. Of 67 subjects, 57% were male, and the mean age of onset was 4.5 months. The maximum number of MPCM lesions was 1 to 10 in 16%, 11 to 30 in 33%, 31 to 50 in 25%, 51 to 100 in 6%, and more than 100 in 20% of subjects. For their MPCM lesions, 46% of subjects reported itching, 34% flushing, and 25% blistering. Reported systemic symptoms included diarrhea (22%), abdominal pain (15%), wheezing or dyspnea (13%), vomiting (10%), bone pain (10%), headaches (8%), cough (10%), rhinorrhea (8%), irritability (6%), and anaphylaxis (1.5%). In a multivariate linear regression analysis, the maximum number of MPCM lesions (p = 0.02) and the number of skin symptoms (p < 0.01) were statistically significant predictors of the number of systemic symptoms, controlling for age of onset, body sites involved, and sex. The correlation between cutaneous findings and symptomatology could aid clinicians in identifying individuals with MPCM who might warrant systemic evaluation and therapy.
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PMID:Severity of cutaneous findings predict the presence of systemic symptoms in pediatric maculopapular cutaneous mastocytosis. 2461 40