Familial Urticaria Pigmentosa With Giant Mast Cell Granules

摘要: Urticaria pigmentosa (UP) developed in two sisters infancy. The lesions both patients contained an extensive infiltration of large, tightly packed mast cells. number cells and the histamine content were increased also clinically normal skin one subject. A proportion lesional perilesional was found, by light microscopy, to contain giant cytoplasmic granules that, on electron noted reach a maximum size about 6 mum diameter have abnormal ultrastructure. Giant not seen other cell types, including neutrophils melanocytes. findings suggest that genetic factors are important determining this variant UP disease may be more widespread than eruption indicates.