Urticaria pigmentosa complicated by polycythemia vera. Report of a case.

U NTIL ABOUT 15 years ago an abnormal proliferation of tissue mast cells was observed only as a cutaneous manifestation. Described originally by Nettleship in 1869, this was named " imrticaria pigmentosa " about 20 years later by Sangster.1'2 The natural course of this disease is highly variable,3 since the cutaneous manifestations represent only one stage of a systemic disease which may not only become generalized, but also initiate changes resulting from biochemical properties of the mast cells. This paper presents the second4 reported case of urticaria pigmentosa complicated by polycythemia vera in an adult. A 62-year-old man entered the hospital because of sudden onset of painful swelling of right leg. Twenty-five years previously he had had acute glomerulonephritis. Seven years ago he developed a nonitching skin rash diagnosed by biopsy as urticaria pigmentosa. Five years ago a right inguinal herniotomy and the removal of an intradermal nevus of the neck had been done. At that time, the peripheral blood film was within normal limits. No history of other skin rashes or allergic disorders was obtained. In the family history one sister had died of cancer, and one granddaughter developed urticaria pigmentosa soon after birth and still has it at age 5. On physical examination, numerous red-brown mactiles and slightly raised papules were found scattered on the trunk and extremities which, when scratched, produced an itching sensation and areas of erythematous halos. The liver and spleen were not enlarged. The right leg was cyanotic and cool distally from the knee and tender on deep palpation of the calf region. The urine showed 2+ albumin and occasional granular casts/high power field in the sediment. WBC was 27,200/cu. mm. on admission, fluctuating between 16,000 and 26,000 on numerous occasions, with an average of 72 per cent neutrophils, relative lymphocyto-penia, and definite monocytosis between 9 and 21 per cent.