Introduction. Urticarial vasculitis is a subtype of cutaneous leukocytoclastic vasculitis. The disease often accompanies systemic disorders, including hematologic malignancies, connective tissue diseases, and infectious diseases, or may be drug-induced.

Case report. A 69-year-old man with an established diagnosis of primary myelofibrosis was admitted to our department because of severe pruritus and cutaneous urticarial lesions. At the time of admission the patient was not receiving treatment for the underlying hematologic disease. Upon physical examination diffuse and confluent firm urticarial lesions were observed on the trunk and limbs. A skin biopsy revealed histological features of urticarial vasculitis. We initiated intravenous hydrocortisone up to 400 mg/day, methylprednisolone 500 mg in a single dose and prednisone orally 20 mg/day, with rapid resolution of skin lesions.

Conclusions. Urticarial vasculitis may accompany various systemic disorders, including primary myelofibrosis. To our knowledge this is the second case in the literature of urticarial vasculitis associated with myelofibrosis. Treatment with systemic glucocorticosteroids should be considered, as this method is usually beneficial.