FRI0228 Cutaneous vasculitis as a presenting manifestation of neoplasia:

Abstract

Background Cutaneous Leukocytoclastic Vasculitis (CLV) may be associated with malignancies, and sometimes it behaves as a paraneoplastic syndrome. This association has been reported in a variable proportion of CLV patients (from 0 to 8%) depending on population selection. Objectives Our aim was to assess the frequency and features of CLV associated to neoplasia in a wide and unselected series of CLV. Methods Study of CLV associated to neoplasia in a serie of 565 patients diagnosed as having CLV in the Rheumatology and Dermatology Divisions from a University Hospital. Results 11 out of 565 patients (1.95%) presenting with CLV were finally diagnosed as having an underlying malignancy (7 hematological/4 solid malignacies). In all of them, skin lesions were the first clinical manifestation. The median to the diagnosis of the malignancy from the onset of CLV was 17 days, (range 8–50). The most frequent skin lesions were palpable purpura (n=10), legs ulcers (n=2) and urticaria (n=1). Other manifestations were constitutional syndrome (n=7) and arthralgias and/or arthritis (n=3). There was no serious visceral vasculitic involvement. Cytopenia was frequently observed in the full blood cell count (n=9), especially in those cases of vasculitis associated to hematological malignancies (immature peripheral blood cells were observed in 4 cases). Immunological testing (ANA, Rheumatoid factor, C3, C4, and ANCA) were negative or within normal range in all cases. Six patients died due to the malignancy and 5 patients recovered following malignancy therapy. Table 1 CaseAge/sex*Main clinical featuresPeripheral blood smearSkin biopsyDiagnosis 140/FUrticarial lesions, fever, polyarthritisAnemia, immature cellsLeukocytoclastic VasculitisMegakaryocytic leukemia 252/FPalpable purpura, constitutional symptomsPancytopenia, immature cellsLeukocytoclastic VasculitisMyelodysplastic Syndrome 356/MPalpable purpura, constitutional symptoms, feverAnemia, leukopenia, immature cellsLeukocytoclastic VasculitisMyelodysplastic Syndrome 470/MPalpable purpura, constitutional symptoms, Polyarthritisanemia, leukopenia, immature cellsLeukocytoclastic VasculitisNon- Hodgkin Lymphoma 578/MPalpable purpura, constitutional symptoms, fever, arthralgias, abdominal painAnemiaLeukocytoclastic VasculitisWaldestrom’s Macroglobulinemia 683/MPalpable purpura, necrotic ulcer,constitutional symptomsAnemia, leukopeniaLeukocytoclastic VasculitisMyelodysplastic Syndrome 761/FPalpable purpura, hematuria,, polyneuopathyAnemiaLeukocytoclastic VasculitisWaldestrom’s Macroglobulinemia 882/MPalpable purpura,abdominal pain, fecal occult blood, hematuriaNormalLeukocytoclastic VasculitisOropharyngeal Squamous cell Carcinoma 949/FPalpable purpura, constitutional symptoms, fever,AnemiaLeukocytoclastic VasculitisInfiltrating Breast Carcinoma 1080/MPalpable purpura, constitutional symptomsAnemiaLeukocytoclastic VasculitisLung Adenocarcinoma 1185/FPalpable purpura, ulcersNormalLeukocytoclastic VasculitisBreast Carcinoma M: male; F: female. Conclusions CLV presenting as a paraneop...