The Mastocytosis Syndrome: Clinical and Biological Studies

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Abstract
Mastocytosis can produce a characteristic clinical syndrome. Flushing, tachycardia, and shock, gastrointestinal complaints, headache, and malaise occur in association with mast-cell infiltration of skin and frequently of liver, spleen, and bone. The symptoms appear to be induced by histamines and the characteristic increased urinary excretion of histamine has been correlated with symptoms in several patients. Histamine was formed by homogenates of human mastocytosis skin lesions which have been shown to contain a specific histidine decarboxylase. No evidence could be found for the occurrence or formation of serotonin by this tissue. Studies with carbon H14-labeled histidine and histamine eliminated the possibility that the histaminuria results from a block in the metabolism of histamine and indicated that increased excretion results from overproduction of histamine. Detection of the specific histamine metabolite 1,4-methyl-imidazole acetic acid (MeImAA) in the urine of patients also indicated histamine overproduction. Measurements of skin histamine content, when related to amounts excreted in the urine, suggest that some cases which clinically appear to be limited to skin actually may be examples of diffuse mastocytosis. The parallelism between the syndromes of mastocytosis and carcinoidosis is elaborated.
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