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September 1972


Arch Dermatol. 1972;106(3):409-414. doi:10.1001/archderm.1972.01620120087026

Congenital Multiple Giant Mast Cell Tumors With Urticaria Pigmentosa: Malabsorption: First Reported Case. Presented by William B. Reed, MD, and, by invitation, Alonzo Y. Olsen, Jr., MD, and G. Robinson, MD.  This 16-year-old white girl was born with giant tumors over the scalp, forehead, neck, and trunk. Bullae developed soon after birth in these areas, associated with severe flushing. Severe vomiting was an early feature of her disorder. She occasionally developed generalized urticaria, particularly in the vulvar area. Laboratory examinations revealed mast cells in the bone marrow, and multiple biopsy specimens of the tumors have shown mastocytomas. In 1962, she had an iron deficiency anemia and, subsequently, developed a protuberant abdomen with loss of weight and lassitude. Her stools became soft, foul-smelling, and putty-colored. She continued to have episodes of vomiting, difficulty in breathing, urticarial attacks, and severe headaches. There has been some change in personality. At the age of