Urticaria Pigmentosa as a Systemic Disease

Gastrointestinal involvement in systemic mastocytosis.

“…Oedema, thickening of the Kerckring folds, and a peculiar nodular mucosal pattern of stomach and small intestine have been demonstrated radiologically in SM (Janower, 1962;Remy, 1962;Bank and Marks, 1963;Bloom, 1965;Jarnum et al, 1967;Clemett et al, 1968;Schongut et al, 1968;Debray et al, 1973). Endoscopically, urticaria-like lesions have been visualized in stomach, duodenum (Clemett et al, 1968;Debray et al, 1973), or in the rectum (Berlin, 1955). Such changes were found also in the four patients of the present series, in some of them only after topical provocation.…”

“…But during administration of brocresine, a specific inhibitor of histidine decarboxylase, the urinary histamine excretion diminished significantly, whereas no significant decrease of the basal acid output occurred. Gastric acid output in the few patients studied was found to be normal or decreased in the majority of patients with urticaria pigmentosa or SM (Jeanselme and Touraine, 1919;Ellis, 1949;Berlin, 1955;Zak et al, 1957;Remy, 1962;Szweda et al, 1962;Bank and Marks, 1963;Jarnum and Zachariae, 1967;Broitman et al, 1970). In the present series, the gastric acid secretion was normal or depressed in two patients (Table 3).…”

Mastocytosis (urticaria pigmentosa) of skin, stomach, and gut with malabsorption.

“…The histological site of histamine in the stomach (Feldberg and Harris, 1953) indicates the presence of mast cell histamine as well as non-mast cell histamine. Although it has been accepted lately that urticaria pigmentosa is not strictly limited to skin (Berlin, 1955;Reilly, Shintani, and Goodman, 1955;Asboe-Hansen, 1960), and some cases have been reported with gastrointestinal symptoms (Berlin, 1955;Brodeur and Gardner, 1956;Reilly et al, 1955;Zak, Covey, and Snodgrass, 1957), no determinations showing increased gastric and jejunal histamine seem to have been published previously.…”

A case of mastocytosis involving bone

“…It is well known that the histologic diagnosis of mastocytosis can be missed when using only conventional staining methods in the histologic study (Berlin 1955). We recommend that clinically diffuse cases, and especially those with marrow fibrosis, be studied using mast cell stains.…”