The coexistence of polycythemia vera with urticaria pigmentosa in a 62-year-old man is reported apparently for the second time. Review of literature indicates that mast cell disorder, manifested initially only in skin changes, may involve many systems. The possible mechanism responsible for the occurrence of the two disorders in the same individual is discussed. Administration of radioactive phosphorus had no effect on the mast cell infiltration of the skin, but resulted in a temporary suppression of erythropoiesis.

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