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

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

“…These findings are similar to those reported in other small series and case reports. 2,[5][6][7][8][9][10][11]13,14,[16][17][18][21][22][23]26,30,31 Malabsorption, which occurred in two patients in our series is well described in systemic mastocytosis, 2,5,7,9,10,16,23,30,31 occurring in 5-30% of patients. 30,32 While all patients in our series were women, the literature indicates an equal sex distribution for systemic mastocytosis in general 33 and for those cases with direct gastrointestinal involvement.…”

“…The literature shows considerable variation with respect to the presence and intensity of intestinal mast cell infiltrates in systemic mastocytosis. Several studies have reported an increase in lamina propria mast cells, 7,8,[11][12][13][14]16,22 while others have not. 3,4,6,9,15,20 Such variation may reflect genuine differences among patients but may also be the result of differences in selection criteria, sampling variability and stains used to highlight mast cells.…”

“…30,32 While all patients in our series were women, the literature indicates an equal sex distribution for systemic mastocytosis in general 33 and for those cases with direct gastrointestinal involvement. [5][6][7][8][9][10][11]13,[16][17][18][19][21][22][23]31 Endoscopically and grossly, the most frequent and distinctive finding in our series was that of mucosal nodularity (present in four of five patients). Such nodularity has been documented by others, both radiologically and endoscopically.…”

“…Such heavy eosinophil infiltrates in gastrointestinal biopsies from systemic mastocytosis patients have documented by others. 8,10,14,16,18 The use of special stains to highlight mast cells is invaluable in this setting as most other causes of mucosal eosinophil infiltrates (including eosinophilic gastroenteritis) are not associated with increased mast cells. 14 An exception is parasitic infestation that may be associated with a prominence of both eosinophils and mast cells.…”

Generalized Mastocytosis

“…Several authors assume that it is reticulosis [2,18,22,34]. In this respect immunological factors may be important.…”