Sixty-four consecutive patients with clinically or laboratory-supported definite multiple sclerosis (MS) were evaluated prospectively for evidence of primary Sjögren's syndrome (SS). This diagnosis was established when a patient had objective keratoconjunctivitis sicca, xerostomia, or both together with positive labial salivary gland biopsy. We found 2 patients (3.1%) with clinical evidence of primary SS. Whether this association is fortuitous or whether there is pathogenetic linkage between MS and primary SS remains to be established.

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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...

Background Urticarial vasculitis is an infrecuent subset of vasculitis described by McDuffy et al, in 1973, characterized clinically by urticarial skin lesions of more than 24 hours duration and histologically by vasculitis. Objectives Our aim was to evaluate the frequency, clinical and pathological features and treatment of UV from a large series of patients with cutaneous vasculitis. Methods Retrospective study of UV from a serie of 565 patients with cutaneous vasculitis of a University Hospital. For the diagnosis of UV, besides urticarial lesions persisting more than 24 hours, a skin biopsy showing necrotizing vasculitis of small vessels was required. Results UV was observed in 11 (1.95%) of 565 patients with cutaneous vasculitis. There were 4 men and 7 women, with a mean age of 52.18±19.39 years (range, 14 to 78 years). Precipitating factors and/or possible causes identified were upper respiratory tract infection (1 case), drugs (amoxicillin, 1 case) and a malignancy (megakaryocytic leukemia, 1 case). In addition to urticarial lesions, other features such as palpable purpura (5 patients), arthralgias (5 patients), arthritis (2 patients), abdominal pain (2 patients), and nephropathy (2 patients) were observed. Hypocomplementemia (low C4) was observed in only 1 of the 11 patients. Other Abnormal laboratory data observed were increase of ESR (5 cases), leukocytosis (3 cases), anemia (2 cases), positive ANA (1 case), and positive Rheumatoid Factor (1 case). Neither of the patients with positive ANA or Rheumatoid Factor developed systemic lupus erythematosus, rheumatoid arthritis or any other connective-tissue disease. The main histological findings were vascular and perivascular infiltration, endothelial swelling and fibrinoid necrosis. The cellular infiltrate was composed mainly of neutrophils, lymphocytes and eosinophils. The most common treatments were corticosteroids (6 cases), antihistaminic drugs (4 cases), chloroquine (4 cases), colchicine (2 cases), NSAIDs (1 case). Cytotoxic agents were not required in any of the patients. After a mean follow-up of 13±12 months (median 11 months) recurrences were observed in 4 patients. A patient died because of an underlying malignancy while the remaining had full recovery without complications. Conclusions UV is a rare subtype of cutaneous vasculitis. In addition to urticarial skin lesions, joint involvement was the most common clinical manifestation. Corticosteroids and antihistaminic drugs are the drugs more commonly used. The prognosis depends on the underlying disease but is usually good. References McDuffie FD, Sams WM, Malddonado JE, Andreini PH, Conn DL, Samayoa EA. Hypocomplementemia with cutaneous vasculitis and arthritis. Possible immune complex syndrome. Mayo Clin Proc 1973; 48:340-348, Disclosure of Interest None Declared