Cutaneous vasculitis may be associated with
malignancies, and may behave as a
paraneoplastic syndrome. This association has been reported in a variable proportion of patients depending on population selection. We conducted the current study to assess the frequency, clinical features, treatment, and outcome of paraneoplastic
vasculitis in a large unselected series of 766 patients with
cutaneous vasculitis diagnosed at a single university hospital. Sixteen patients (10 men and 6 women; mean age ± standard deviation, 67.94 ± 14.20 yr; range, 40-85 yr) presenting with
cutaneous vasculitis were ultimately diagnosed as having an underlying
malignancy. They constituted 3.80% of the 421 adult patients. There were 9 hematologic and 7 solid underlying
malignancies. Skin lesions were the initial clinical presentation in all of them, and the median interval from the onset of
cutaneous vasculitis to the diagnosis of the
malignancy was 17 days (range, 8-50 d). The most frequent skin lesions were palpable
purpura (15 patients). Other clinical manifestations included constitutional syndrome (10 patients) and
arthralgia and/or
arthritis (4 cases). Hematologic
cytopenias (11 cases) as well as immature peripheral blood cells (6 cases) were frequently observed in the full blood cell count, especially in those with
vasculitis associated with
hematologic malignancies. Specific treatment for
vasculitis was prescribed in 10 patients; nonsteroidal antiinflammatory drugs (4 patients),
corticosteroids (3 patients),
chloroquine (1 patient),
antihistamines (1 patient), and
cyclophosphamide (1 patient). Ten patients died due to the
malignancy and 6 patients recovered following
malignancy therapy. Patients with paraneoplastic
vasculitis were older, more frequently had constitutional syndrome, and less frequently had organ damage due to the
vasculitis than the remaining patients with
cutaneous vasculitis. In summary, cutaneous paraneoplastic
vasculitis is an entity not uncommonly encountered by clinicians. The most common underlying
malignancy is generally hematologic. In these cases the presence of
cytopenias and immature cells may be red flags for the diagnosis of
cancer. In patients with paraneoplastic
cutaneous vasculitis, the prognosis depends on the underlying
neoplasia.