Background/Purpose:

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.

Methods:

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.

Results:

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.

Conclusion:

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

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/cutaneous-vasculitis-as-a-paraneoplastic-syndrome/