Despite numerous reports of urticaria pigmentosa, the riddle of the mast cell and the etiology of this disease have not been solved. Paradoxically, although extensive lesions with systemic involvement have been described, the disease has, with few exceptions 1-9 run a benign course.10 The massive mast-cell infiltration is the sine qua non of the disease. However, some instances in which no mast cells were present have been recorded.11,12 Mast cells, which contain serotonin,13 are abundant in the skin of the rat and mouse. They are only occasionally present in the normal skin of human beings, but may be numerous in certain diseases and contain no measurable amount of serotonin.14,15 The number of these cells have been noted to diminish in urticated lesions,16 were not affected by urtication,17 and increased in number with urtication.18 The demonstration of the mast cell by aqueous fixatives has been both questioned19 and affirmed.20,21
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