Session Title: Vasculitis
Session Type: Abstract Submissions (ACR)
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. Our aim was to assess the frequency and features of CLV associated to neoplasia in a wide and unselected series of CLV.
Study of CV associated to neoplasia in a series of 877 patients diagnosed as having CLV in the Rheumatology and Dermatology Divisions from a University Hospital.
16 out of 877 patients (1.82 %) presenting with CLV were finally diagnosed as having an underlying malignancy. There were 9 hematological and 7 solid malignacies. In all of them, skin lesions were the first clinical manifestation and the median interval to the diagnosis of the malignancy from the onset of CLV was 17 days (range 8 –50 days). The most frequent skin lesions were palpable purpura (13 patients), legs ulcers (2 patients), urticaria (2 patient) and erythema (1 patient). Other manifestations were constitutional syndrome (10 patients) and arthralgias and/ or arthritis (6 cases). There was no serious visceral vasculitic involvement. Cytopenia was frequently observed in the full blood cell count (11 cases), especially in those cases of vasculitis associated to hematological malignancies. Immature peripheral blood cells were observed in 9 cases. Immunological testing (ANA, Rheumatoid factor, C3, C4, and ANCA) were negative or within normal range in all cases. 10 patients died due to the malignancy and 6 patients recovered following malignancy therapy.
CLV presenting as a paraneoplastic syndrome is a rare condition. The most common underlying malignancy is generally hematological. The prognosis depends on the underlying neplasia.
|Case||Age/sex||Main clinical features||Peripheral blood smear||Skin biopsy||Diagnosis|
|1||40/F||Urticarial lesions, fever, polyarthritis||Anemia, immature cells||Leukocytoclastic Vasculitis||Megakaryocytic leukemia|
|2||52/F||Palpable purpura, constitutional symptoms||Pancytopenia, immature cells||Leukocytoclastic Vasculitis||Myelodysplastic Syndrome|
|3||56/M||Palpable purpura, constitutional symptoms, fever||Anemia, leukopenia, immature cells||Leukocytoclastic Vasculitis||Myelodysplastic Syndrome|
|4||70/M||Palpable purpura, constitutional symptoms, , polyarthritis||anemia, leukopenia, immature cells||Leukocytoclastic Vasculitis||Non- Hodgkin Lymphoma|
|5||78/M||Palpable purpura, constitutional symptoms, fever, arthralgias, abdominal pain||Anemia||Leukocytoclastic Vasculitis||Waldestrom´s Macroglobulinemia|
|6||83/M||Palpable purpura, necrotic ulcer,constitutional symptoms||Anemia, leukopenia||Leukocytoclastic Vasculitis||Myelodysplastic Syndrome|
|7||61/F||Palpable purpura, hematuria, polyneuopathy||Anemia||Leukocytoclastic Vasculitis||Waldestrom´s Macroglobulinemia|
|8||76/M||Palpable purpura, urticarial lesions, constitutional syptoms, fever||Pancytopenia, immature cells||Leukocytoclastic Vasculitis||Hairy cell leukemia|
|9||81/F||Palpable purpura, erythema, constitutional symptoms, fever||Anemia, immature cells||Leukocytoclastic Vasculitis||Mantle cell lymphoma|
|10||82/M||Palpable purpura,abdominal pain, fecal occult blood, hematuria||Normal||Leukocytoclastic Vasculitis||Oropharyngeal Squamous cell Carcinoma|
|11||49/F||Palpable purpura, constitutional symptoms, fever||Anemia||Leukocytoclastic Vasculitis||Infiltrating Breast Carcinoma|
|12||80/M||Palpable purpura, constitutional symptoms||Anemia||Leukocytoclastic Vasculitis||Lung Adenocarcinoma|
|13||85/F||Palpable purpura, ulcers||Normal||Leukocytoclastic Vasculitis||Breast Carcinoma|
|14||53/M||Palpable purpura, arthralgias||Normal||Leukocytoclastic Vasculitis||Pyriform sinus squamous cell carcinoma|
|15||71/M||Palpable purpura, constitutional syptoms||Normal||Leukocytoclastic Vasculitis||Bladder carcinoma|
|16||70/M||Palpable purpura||Normal||Leukocytoclastic Vasculitis||Glottic squamous cell carcinoma|
F. Ortiz Sanjuan,
M. A. Gonzalez-Lopez,
M. A. Gonzalez-Gay,
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/cutaneous-vasculitis-as-a-paraneoplastic-syndrome/