Gastrointestinal abnormalities and involvement in systemic mastocytosis

In 1869, Nettleship and Tay(159) described a 2-year-old child with hyperpig mented, urticarial skin lesions. Their report is credited as being the firs t description of urticaria pigmentosa. It was not until 1877(54) that mast cells were first described by Ehrlich, who found cells possessing cytoplasm ic granules that stained metachromatically with aniline dyes. He called the se cells "mastzellen" because they were distended with granules. Subsequent ly, in 1878, Sangster(191) described a patient with a pigmented, urticarial rash which he called "urticaria pigmentosa." In 1887,(226) Unna demonstrat ed the presence of mast cells in the skin lesions of affected patients. It was not until 50 years later, in 1933, that Touraine and co-workers(217) su ggested that the disease might involve internal organs and 16 years later, in 1949, that Ellis et al(55) established this fact on an autopsy study in a 1-year-old child. In this first description of systemic mastocytosis by E llis et al(55) the patient had urticaria pigmentosa from birth and develope d diarrhea with voluminous, foul-smelling stools. Autopsy revealed hepatome galy, splenomegaly, and lymphadenopathy, with microscopic infiltration by m ast cells of the bone marrow, liver, spleen, lymph node, kidney, and pancre as.(55) Therefore, in both the clinical description and histologically, the re was clear evidence for gastrointestinal involvement in the first establi shed case of systemic mastocytosis.(55) In 1936, Sezary(200) proposed the n ame mastocytosis for this generalized involvement by mast cells.