Search

Background:Sjögren’s Syndrome (SS) is a chronic systemic autoimmune disease of unknown etiology, carrying the highest lymphoma risk among autoimmune diseases, with significant impact on mortality and morbidity of patients.Objectives:To describe: i) the clinical phenotype of SS, ii) the histologic type, stage, treatment options regarding lymphomas and iii) the prognosis of patients with SS related lymphoproliferative disorders.Methods:Eight hundred and fifteen consecutive SS patients’ records from a single center fulfilling the 2016 ACR/EULAR were reviewed retrospectively for the purpose of this study. One hundred twenty-one patients with a diagnosis of non-Hodgkin Lymphoma (NHL) were identified and enrolled in the study population. Cumulative clinical, laboratory and histologic data were recorded and overall survival as well as event free survival curves were constructed using the Kaplan-Meier method. An event was defined as a disease progression, lymphoma relapse, treatment failure, histologic transformation, development of a 2nd lymphoma or death from any cause.Results:From 121 pSS patients with lymphoma the most common histologic type encountered was MALT lymphoma (92/121, 76,0%) followed by DLBCL (11/121, 9.0%) and NMZL (8/119, 6.6%). The remaining 10 patients had various lymphomas of B (follicular, lymphoplasmacytic, chronic lymphocytic leukemia} and T cell origin (peripheral T cell lymphoma not otherwise specified, primary cutaneous T cell lymphoma, angioimmunoblastic t-cell lymphoma). Permanent salivary gland enlargement (66.1%, 80/121), palpable purpura (34,7% 42/121), peripheral nervous involvement (9,9%, 12/121), interstitial lung disease (8,2%, 10/121) presence of serum cryoglobulins (38,7%, 43/111) and C4 hypocomplementemia (69,8% 81/116) present at least 1 year before the development of lymphoma were the main pSS related features. The median age at lymphoma diagnosis was 58 years old (range 29-82) while MALT lymphomas developed earlier compared to DLBCL from pSS diagnosis (8 vs 3 OR= 3.84, 95%CI: 0.29 to 10.46; p=0.0266). The commonest biopsy proven extranodal sites included the labial minor salivary (43,8% patients) and parotid glands (30,5%) while 11% of patients had more than 1 extranodal sites affected. Bone marrow involvement was evident in 24,3% of patients (29/119) while nodal involvement in 35,5% (42/118). The majority of patients (65%) had limited disease (stage I or II). A watch and wait therapeutic policy was chosen in 40 patients while the rest received rituximab with or without chemotherapy. The 10-year survival and event free rates were 79% and 45,5% for MALT lymphomas, 40,9% and 24,2% for DLBCL and 46% and 31% for NMZL respectively (Figure 1). The Mantel-Cox log-rank comparison of the overall survival curves revealed a statistically significant difference (p=0.0016) among lymphoma subtypes.Figure 1.Overall and event free survival of SS-associated lymphoma patients. A. Kaplan-Meier overall survival analysis. B. A Kaplan-Meier event free survival analysis.Conclusion:This is the largest single center series of SS- associated lymphoma patients, providing a detailed description of SS and lymphoma related features, combined with a 10-year survival and event free curves for the first time in the literature.Disclosure of Interests:None declared.

Background:Primary Sjögren Syndrome (SS) is a slowly progressive systemic autoimmune disease complicated by lymphoma, with mucosa associated lymphoid tissue (MALT) type being the most common lymphoma form. Several predictors related to pSS associated lymphomas have been described, but there are no studies focusing on specific risk factors for the MALT histologic subtype.Objectives:To identify predictors at SS diagnosis for MALT lymphoma development in pSS patients using simple clinical features.Methods:From 815 SS patients of a single center fulfilling the 2016 ACR/EULAR criteria, those with subsequent development of MALT lymphoma according to the 2016 WHO classification were identified and matched in 1:2 ratio, with non-lymphoma SS control patients according to age, disease duration from SS diagnosis and gender. Lymphoma patients diagnosed within a year from SS diagnosis were excluded from the current study. Clinical, laboratory, histologic data as well as the ESSDAI scores at the time of SS diagnosis were recorded and compared between lymphoma and non-lymphoma patients. Independent lymphoma predictors were identified by a data driven Fast Correlation Based Feature selection (FCBF)/Logistic Regression (LR) algorithm.Results:A unified dataset of 57 MALT lymphoma patients and 114 non lymphoma controls along with 39 features/variables was generated. The median age of SS diagnosis and the disease duration from SS diagnosis to lymphoma diagnosis (lymphoma group) or last follow up (control group) was 50,5 years old (range 25-77) and 7 years (range 0- 30) for the control group and 50 years old (range 24-70) and 8 years (range 1 -30) for the lymphoma group, respectively. MALT lymphoma patients presented more frequently with palpable purpura (23,2% vs 5,3% p=0,001), cryoglobulinemia (30,2% vs 1,6% pConclusion:MALT is the predominant pSS related lymphoproliferative histologic type, associated with systemic disease activity and vasculitic manifestations at SS diagnosis. Cryoglobulinemia and ESSDAI score were proven independent risk factors for MALT lymphoma development.Table 1.An FCBF-based multivariable logistic regression analysis results for investigating risk factors for MALT lymphoma developmentProminent featureRegression coefficientOdds ratiop-valueCI lowCI upperCryoglobulinemia1.675.3420.033*1.1824.327Total ESSDAI at diagnosis0.281.3181.2081.439Kidney involvement0.071.0690.50.1010.351•< 0.05 (95% confidence interval). The rest of the features that participated in the analysis include the following: Palpable purpura, Low C4, Salivary gland enlargement, Lacrimal gland enlargement, ANA Titers, RF, Focus score at Sjögren diagnosis, PNS involvement, Anti-La, Disease duration from SS onset to SS diagnosis, Neutrophils•AUC=0.78Disclosure of Interests:None declared

ObjectivesWe determined the temporal association between clinical and serological disease manifestations and development of cutaneous small vessel vasculitis in a large prospective multiethnic cohort.MethodsPatients with SLE diagnosed according to the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria or the revised classification criteria as defined by the American College of Rheumatology (ACR) were enrolled in the Hopkins Lupus Cohort. Cutaneous small vessel vasculitis was determined as a component of the Systemic Lupus Erythematosus Disease Activity Index. SLE-associated cutaneous small vessel vasculitis lesions were reported clinically. They presented as punctate lesions, palpable purpura, tender erythematous plaques or macules with or without necrosis. No histopathological diagnosis was pursued to confirm the diagnosis of vasculitis or to differentiate it from other causes of digital lesions in patients with SLE. Disease manifestations that preceded the first occurrence of cutaneous small vessel vasculitis lesions were analysed using Kaplan-Meier. Cox regression analysis was used to assess the relationship between baseline clinical and immunological manifestations and the development of cutaneous small vessel vasculitis. We adjusted for gender, race and age at SLE diagnosis.ResultsA total of 2580 patients were studied: 52.4% were Caucasian and 39.4% were African-American. The mean age of the cohort was 45.5±14.5 years. The mean years of cohort follow-up was 7.9±7.6. Cutaneous small vessel vasculitis was observed in 449 (17.3%). The mean time to cutaneous vasculitis after SLE diagnosis was 4.78 years (95% CI 3.96 to 5.60). At least 159 (35%) patients had recurrences of cutaneous vasculitis lesions. Discoid rash, Raynaud’s phenomenon, myositis, anaemia, Coombs’ positivity, leucopenia, anti-Smith and anti-RNP (Ribonucleoprotein) were significantly associated with the development of cutaneous vasculitis. The SLICC/ACR Damage Index score was higher in patients with cutaneous vasculitis compared with those without cutaneous vasculitis.ConclusionsCutaneous vasculitis is frequent (17.3%) and often recurrent (35%). African-Americans are at higher risk of developing cutaneous small vessel vasculitis than Caucasians. Clinical presentations such as myositis and haematological manifestations are predictors of cutaneous vasculitis development. The presence of cutaneous vasculitis is associated with increased organ damage.

Background:Sjögren’s syndrome (SS) is characterized by B cell hyperactivity reflected by hypergammaglobulinemia as well as a plethora of autoantibodies including antinuclear antibodies (ANA), anti-Ro/SSA, anti-La/SSB and rheumatoid factors (RF). Previous studies have focused on the phenotype of single positive (ANA or anti-Ro/SSA or anti-La/SSB) or double positive (anti-Ro/SSA and anti-La/SSB positive) SS patients, showing differences regarding the age of diagnosis, sicca manifestations and specific extraglandular manifestations. To our knowledge, no study has ever explored the clinical spectrum of triple seronegative (anti-Ro/SSA + anti-La/SSB + RF negative) and quadruple seronegative (ANA +anti-Ro/SSA + anti-La/SSB + RF negative) SS patients.Objectives:To study the differences in the clinical phenotype of triple and quadruple seronegative (SS) patients in a large cohort of well characterized patients, after comparison with anti-Ro/SSA positive patients.Methods:From a total cohort of 1723 consecutive SS patients who fulfill the 2016 EULAR/ACR criteria and are followed up in 4 clinical centers ([Universities of Pisa and Athens, Harokopio and Ioannina, (PAHI)], those who have been found triple or quadruple seronegative were identified and compared with matched anti-Ro/SSA positive SS patients according to age of SS onset, disease duration and gender, in 1:1 and 1:2 ratio respectively. Glandular (dry mouth, dry eyes, parotid gland enlargement) and extra-glandular manifestations (Raynaud’s phenomenon, chronic fatigue arthralgias/myalgias, arthritis, palpable purpura, liver involvement, kidney involvement, lung involvement, neurologic involvement, long standing lymphadenopathy and lymphoma) were compared between the 2 seronegative groups and the anti-Ro/SSA positive control group. Statistical analysis for categorical variables was performed by Fisher exact or chi-square tests and for continuous variables with t test or Mann-Whitney accordingly.Results:Two hundred and four SS patients (11,8%) were identified as triple negatives and 53 (3,0%) as quadruple, with a median disease duration of 6 years (range: 0-41) and 5 years (range: 0-32) respectively. The matched anti-Ro/SSA controls were 204 for the triple and 103 for the quadruple negatives. Triple negatives had lower frequency of monoclonal gammopathy (5,5% vs 12,1% p=0,04), low C4 serum levels (23% vs 36%, p=0,009) and lymphoma (3,4% vs 9,8%, OR= 3,06, 95% CI =1,27-7,85) while quadruple negatives exhibited higher prevalence of dry eyes (100% vs 90%) and lower prevalence of long standing lymphadenopathy (2,7% vs 19,5%, p=0,001) and lymphoma (0% vs 15%, p=0,001) compared to anti-Ro/SSA matched controls.Conclusion:Combined seronegativity account for more than 10% of SS population and is associated with lower prevalence of lymphoma compared to anti-Ro/SSA positive patients.Disclosure of Interests:Loukas Chatzis: None declared, Vasileios Pezoulas: None declared, Francesco Ferro: None declared, Valentina Donati: None declared, Aliki Venetsanopoulou: None declared, Evangelia Zampeli: None declared, Maria Mavromati: None declared, Paraskevi Voulgari: None declared, Clio Mavragani: None declared, Dimitris Fotiadis: None declared, Fotini Skopouli: None declared, Salvatore De Vita Consultant of: Roche, Human Genome Science, Glaxo Smith Kline and Novartis, Chiara Baldini: None declared, Haralampos M. Moutsopoulos: None declared, Athanasios Tzioufas: None declared, Andreas Goules: None declared

Background:Rheumatoid arthritis (RA) is an autoimmune disease characterized by erosive synovitis (causing severe joint damage) and systemic damage. Rheumatoid vasculitis (RV) is an extra-articular manifestation of rare but serious rheumatoid disease that involves the damage of small and medium sized vessels.Objectives:The purpose of the study was to evaluate the incidence of damages occurred on cutaneous level in patients diagnosed with RA and to detect capillaroscopic microangiopathy changes in the activity of rheumatoid disease.Methods:101 patients were included in the study, all were diagnosed with RA according to the ACR/EULAR 2010 criteria. RV diagnosis was based on the clinical examination. The capillaroscopic evaluation was performed with a 3.0 VideoCap device at a 200x magnification (200x magnification contact lenses). Capillaroscopic examination was performed on fingers II-V of both hands, being considered pathological if the capillaroscopic changes are present in at least two fingers. The activity of disease was calculated using DAS 28 ESR score.Results:18% of examined patients was male and 82% female, with the mean age of 56,2years±10.16SD, and the mean duration of the disease in years of illness was 8.65±5.31SD. 3 patients had periungual vasculitis with cutaneous ulceration and gangrene, 12 patients had palpable purpura. 29% of the patients had vasospastic skin changes. The activity score of RA was associated with the capillaroscopic changes in peripheral microangiopathy p-0.037. Capillaries images were heterogenous in aspects and distribution, tortuous capillaries, microhemorrhages, giant/dilated capillaries, avascular areas were observed.Conclusion:Vascular microangiopathy evidenced by capillaroscopic examination is present in patients with cutaneous vasculitis having a medium, severe activity of rheumatoid diseases.References:[1]Cutolo M, Paolino S, Smith V. Nailfold capillaroscopy in rheumatology: ready for daily use but care in terminology. Clinical Rheumatology 2019; 38:2293-2297http://doi.org/10/s10067-019-04716-w[2]Lambova N, Müller-Ladner U. Capillaroscopic pattern in inflammatory arthritis. Microvascular Research 2012; 83:318-322Dilated, giant capillaries, microhemorrhage bleedings, avascular areaReduced blood flow (observed in dynamics)Disclosure of Interests:Mirela Parvu Speakers bureau: Not for this subject, theme, Anca Cozos Speakers bureau: Not for this subject, theme, Anna-Iuliana Biro Speakers bureau: Not for this subject, theme, Horatiu Valeriu Popoviciu Speakers bureau: Not for this subject, theme

Background:Purpura are lesions that occur after bleeding on the skin, mucous or serosal surfaces. Purpura can be classified into 2 subgroups; which are linked to thrombocytopenia and non-trombocytopenic purpura. While thrombocytopenic purpura often occurs due to a hematological disease; in non-trombocytopenic purpura etiological causes are very variable, and systemic vasculitis has an important place among them.Objectives:The demographic features of the patients applying with purpura and the underlying causes and diseases were aimed to be revealed.Methods:44 consecutive patients (22 women, 22 men) who were admitted to the hospital in the last 6 months, due to purpura were evaluated. Average age of patients was 49.6 ± 19.6 years. Patients were questioned about a recent infection, drug use, concomitant or underlying diseases. The serological tests and other laboratory tests for etiology were performed and biopsy was taken from the skin lesions which are appropriate.Results:While 4 (9%) patients had widespread purpura on the trunk-upper-lower extremities, in 22 (50%) patients purpuric lesions were limited only to the lower limb. The period between the onset of the first symptom and the admission to the hospital was longer than 4 weeks in 35 (79.5%) patients, and shorter than 1 week in 9 (20.5%) patients. 24 (54%) patients had an anamnesis of infection 2-3 weeks before purpura, and 20 (45%) patients had an anamnesis of drug use. The most common accompanying symptom was abdominal pain and was present in 15 (34%) patients. Biopsy was performed from the skin lesion in 37 patients. Histopathological examination of all was compatible with leukocytoclastic vasculitis. In indirect immunofluorescence staining, 17 were found to be IgA positive. 2 (4.5%) patients were diagnosed PR3-ANCA positive granulomatosis with polyangiitis. 1 patient had Hepatitis B virus infection was detected in 1 patient (2.2%), HIV infection was detected in 1 patient (2.2%) and malignancy was detected in 1 patient (2.2%).Conclusion:In our study, the most common reason was found as IgA vasculitis in patients presenting with palpable purpura. Although vasculitic involvement was limited to the skin in most patients, organ-threatening systemic vasculitis was detected in a few patients. Patients applying with Purpura should be questioned for infection and drug use, should be examined for underlying diseases including systemic vasculitis, and closely monitored for organ involvement.Disclosure of Interests:None declared

A 62-year-old man presented with excruciating joint pains, back stiffness and numbness of his hands and feet. Over the past 18 months, he had experienced similar episodes for which the diagnoses of bilateral carpal tunnel syndrome and lateral epicondylitis had been made. Physical examination revealed polyarticular arthritis affecting the shoulders, wrists and right knee. Palpable purpura overlying the calves and ankles was present. Laboratory tests showed markedly elevated erythrocyte sedimentation rate and C-reactive protein in the setting of negative blood and urine cultures. Rheumatoid factor and antinuclear antibodies were negative. Chest CT demonstrated bilateral pulmonary infiltrates. A punch biopsy of the rash showed leukocytoclastic vasculitis. Anti-proteinase-3 titers returned strongly positive. A diagnosis of granulomatosis with polyangiitis was made. Treatment with high-dose steroids, followed by rituximab resulted in normalisation of inflammatory markers with subsequent resolution of joint pains, rash and pulmonary infiltrates and improvement of neuropathic symptoms.

We present a rare case of a 4-year-old boy with newly diagnosed Henöch-Schonlein purpura (HSP) affecting the scrotum and penis. The patient presented to the emergency department with palpable purpura symmetrically distributed over the lower limbs. This was associated with arthritis of the right knee, abdominal pain and scrotal swelling. These symptoms were preceded by an upper respiratory tract infection (URTI). The patient was initially treated with empirical oral antibiotics for epididymitis and was discharged. He subsequently re-presented 12 days later with penile swelling, erythema and tenderness. An ultrasound scan of the penis revealed grossly oedematous subcutaneous tissue with normal penile architecture. His symptoms resolved spontaneously and the patient remains under close follow-up by the paediatric team for further sequelae of HSP.

Henoch–Schonlein purpura (HSP) is the commonest vasculitis of childhood. The first description of this disorder was probably that of a young boy with “bloody points” over the shins of his legs, abdominal pain, blood in the stools and urine and painful subcutaneous oedema, described by William Heberden in 1801. In 1837 Johann Schonlein described the association of purpura and joint pain as “Peliosis rheumatica”. Eduard Henoch, Schonlein’s former student, noted gastrointestinal involvement in association with purpura and arthritis in 1868 and subsequently he recorded renal involvement too.1 Until recently the 1990 American College of Rheumatology criteria for HSP were the most commonly used criteria for diagnosis.2 This classification was modified but has now been superseded by the European League Against Rheumatism (EULAR) and Paediatric Rheumatology European Society (PReS) consensus criteria for the classification of childhood vasculitis. The group reached consensus that the classification of HSP should be the finding of palpable purpura in the presence of one of either diffuse abdominal pain, a biopsy showing predominant IgA deposition, arthritis or arthralgia and/or renal involvement (any haematuria and/or proteinuria) (table 1).3 View this table: Table 1 Classification criteria for Henoch–Schonlein purpura3 Henoch–Schonlein purpura is found in all age groups, from the age of a few months to late adulthood but it is more common in young children, with over 50% under 5 years and over 75% under 10 years. The clinical features of HSP are often atypical at extremes of age and the illness is more severe in adults while children under 2 years old are less likely than older children to have nephritis or abdominal complications. The reported incidence of HSP varies from 10–20.4 per 100 000 children.4–7 It is highest in younger children with 22.1/100 000 in the under 14 years and 70.3/100 000 in the 4–7 year age group being reported in one series …

A 59-year-old male presented with methicillin-resistantStaphylococcus aureus bacteraemia from a prostatic abscess and was treated with vancomycin. Two weeks into his treatment course, he developed severe joint pains, abdominal pain with bloody, mucinous stools and a diffuse palpable purpuric rash on his extremities. Biopsy of the rash showed IgA immune-complex deposition consistent with Henoch-Schönlein purpura. After treatment with glucocorticoids, his symptoms resolved completely. Vancomycin is an extremely commonly used antibiotic with certain well-known adverse effects. Henoch-Schönlein purpura, a vasculitis involving abdominal pain, arthralgias and palpable purpura, is a much less common side effect, as seen in this patient. Given that vancomycin is widely used internationally, clinicians should be aware of the risks entailed by its use.

Dabigatran is a non-vitamin K antagonist oral anticoagulant that has been approved for atrial fibrillation and prevention of venous thromboembolism. Its use has been increasing in the USA since serum drug levels do not need monitoring. To date, no significant skin side effects have been reported other than 4 cases of non-specific skin lesion and 2 cases of leukocytoclastic vasculitis (LCV), which is a small vessel inflammatory disease that presents as palpable purpura in lower extremities. We present a man aged 57 years with chronic deep vein thrombosis who developed palpable purpura, petechiae, swelling in lower extremities, torso and distal upper extremities on the third day after dabigatran initiation. The present case highlights the potential risk for LCV with dabigatran use and provides insight into its management.

Background There has been much research aimed at elucidating key biological and clinical features which predict severity of disease and development of lymphoma in patients with primary Sjogren9s syndrome (pSS). Key biological predictors identified include the presence of leukopenia or lymphopenia, hypocomplementaemia, cryoglobulinaemia, monoclonal gammopathy; key clinical predictors include younger age at time of symptom onset or diagnosis, peripheral neuropathy, salivary gland enlargement and skin involvement (particularly palpable purpura) [1–4]. The UK Primary Sjogren9s Syndrome Registry (UKPSSR) is a MRC funded patient cohort, devised to facilitate research into pSS to improve understanding of the condition. Objectives Ascertain the prevalence of putative clinical predictors amongst UKPSSR patients. Explore the relationship between demographics, clinical parameters, disease severity and lymphoma development. Methods Patients were identified on the UKPSSR (n=868), those with insufficient data were excluded from analysis (n=36). Frequency of the predictors was calculated for remaining 832 patients. Patients were assigned a value for each predictor: 1 for present, 0 for absent. These values were then added to provide the prevalence of each predictor within the cohort, and a cumulative score for each patient. Each patient had 2 cumulative scores: one for disease severity and risk of death, the other for risk of lymphoma. Patients with a recorded age of pSS symptom onset (n=739) were analysed to investigate the relationship between age of onset and development of lymphoma. SPSS used for statistical analysis. Results Pearson Chi-squared analysis found a statistically significant (p=0.004) increased incidence of lymphoma in patients presenting with symptoms of pSS before the age of 35. Predictors of lymphoma development were more common in patients whose symptoms started between age 20–34 (p=0.05). Patients with diagnosed lymphoma had a higher cumulative lymphoma risk score than patients who did not have lymphoma (p=0.05). Predictors of severe disease were found to be more common in patients whose symptoms began before age 35 (p=0.05). Conclusions This analysis corroborates the findings of previous research showing an increased lymphoma prevalence in patients presenting with pSS at a younger age [4]. It also finds a statistically significant link between poor prognostic features and development of lymphoma as described in the literature [2,3]. Further statistical analysis will aim to establish the link between each predictor and the development of systemic manifestations of pSS, baseline and cumulative ESSDAI scores and lymphoma. References Brito-Zeron, P., et al., Systemic activity and mortality in primary Sjogren syndrome. Ann Rheum Dis. Luciano, N., et al., One year in review 2015: Sjogren9s syndrome. Clin Exp Rheumatol. Nocturne, G. and X. Mariette, Sjogren syndrome-associated lymphomas. BJH. Ramos-Casals, M., et al., Systemic involvement in primary Sjogren9s syndrome evaluated by the EULAR-SS disease activity index. Rheumatology. Disclosure of Interest None declared

Background Henoch-Scholein purpura (HSP) is a generalised vasculitis characterised by various combinations of skin, joint, gastrointestinal and renal involvement. The diagnosis HSP is based on clinical and histological features. HSP can occur at any age, but is most common in childhood. The etiology remains unknown, although it is quite common upper respiratory tract (URT) infections as a precedent. Objectives To describe the prevalence of the clinical manifestations in adults and children at the time of diagnosis of HSP. Methods A retrospective study of the case records of all admitted patients identified by the HSP diagnosis in the Hospital Universitario Severo Ochoa (Madrid, Spain) between January 1976 and June 2015 was undertaken. Classification criteria proposed by Michel et al were used for the diagnosis - including age Continuous data were described as mean and standard deviation (mean, SD), or median and interquartile range (IQR) and categorical variables as percentages. Means and incidence rates were compared using the Student t test. To compare categorical data we performed the chi-square test. Results We identified 51 patients, mainly male (64.7%), with a median age at the onset of symptoms of 18 (IQR 13–41) years old. Thirty nine were younger than 20 years old. In 51% a history of URT infection before the onset of HSP was found, particularly in children (56.4%), without statistical significance (p=0.19). Palpable purpura was the initial manifestation of the disease in all patients. Differences in the distribution of cases by joint and abdominal manifestations between adults and children were not statistically significant. Joint involvement was presented in 66.7% (34 patients), where arthritis was recorded up to 41.2%, mostly children having arthritis in 84.21% (p=0.5). Abdominal manifestations were presented in 60.8% (47% adults and 13% children) (p=0.8). Renal manifestations were hardly recorded at onset, whereas up to 66.7% had no renal involvement described (31 children and 3 adults) (p=0.001). Hematuria and proteinuria were observed in 3.9% and 2% of the study population respectively, while the association of both renal manifestations was observed in 21.6%. Conclusions Although the simple size is small, our study shows several differences at the onset of HSP between adults and children. The disease is observed predominantly in childhood, where joint involvement was frequent. Renal involvement, generally manifested as hematuria either isolated or in association with proteinuria, constitutes the most serious feature of HSP in adulthood. References Michel BA, Hunder GG, Bloch DA, Calabrese LH. Hypersensitivity vasculitis and Henoch-Schonlein purpura: A comparison between the 2 disorders. J Rheumatol 19: 721–8, 1992 Disclosure of Interest None declared

Background Sjogren9s syndrome (SS) is an autoimmune disease associated with focal lymphocytic infiltration in the epithelium of exocrine glands, and may develop systemic manifestations in about 80% of the patients. Typical antibodies present in SS are antinuclear antibody (ANA), rheumatoid factor, SSA/Ro and SSB/La. SS classification criteria includes SSA/Ro and SSB/La, and their presence is associated with extra-glandular manifestations. The prevalence of SSA/Ro in SS is 30–70%, and is related to increased frequency of dermatologic symptoms, scleritis and interstitial lung disease. The relationship between SSB/La and clinical manifestations has not been previously well established, though it can be found in 5–50% of SS, and may co-exist with SSA/Ro half of the times. SSB/La is found isolated in a very low percentages of SS. Objectives To evaluate the correlation between the presence of SSB/La antibody with distinct clinical, demographic and serological characteristics of SS. Methods Adult patients evaluated in the Rheumatology Section (Buenos Aires British Hospital) who fulfilled American European Consensus Group (AECG) SS criteria were included. Three groups were compared according to the antibodies present: 1. Positive SSA/Ro and negative SSB/La 2. Both positive 3. Negative SSA/Ro and positive SSB/La. Mann-Whitney and Fisher tests were used according to variables; those with p Results Among 195 patients with SS, 93.8% (n=183) were female. Mean age was 54.7 years and the mean total disease duration was 68.5 months. We excluded from analysis 16/8.2% patients due to missing data, and 29/14.9% patients that were negative for both antibodies. Two groups were finally compared (Group 3 excluded due to low prevalence, n=3/1.5%): 80/41% patients with positive SSA/Ro and negative SSB/La; and 67/34.3% positive SSA/Ro and SSB/La. In the univariate analysis, positive SSA/Ro and SSB/La was statistically found to be associated with recurrent sialoadenitis and palpable purpura. Serological findings associated with this group included positive rheumatoid factor, hypocomplementemia and hypergammaglobulinemia. Poor prognosis features such as palpable purpura (OR 9.5 IC 95% 1.1–79 p 0.037), recurrent sialoadenitis (OR 3.1 IC 95% 1.2–7.7 p 0.011), hypocomplementemia (OR 3.6 IC 95% 1.4–9.4 P 0.0075) and hypergammaglobulinemia (OR 2.7 IC 95% 1.2–6.1 p 0.01) were also statistically significant in the multivariate analysis. Cryoglobulinemia and leucopenia were not found to be associated in both of the analysis. Conclusions In our population, the presence of SSB/La in patients with positive SSA/Ro was related to recurrent sialoadenitis, palpable purpura, hypocomplementemia, hypergammaglobulinemia and reumathoid factor. Although previous studies are contradictory, our study might justify the systematic search of this antibody due to its clinical relevance and link to poor prognosis factors in SS. Disclosure of Interest None declared

A 23-year-old previously healthy man was in his usual state of good health until 5 days prior to admission when he noted fever of 38.0°C, cough, rhinorrhoea and sore throat. Two days prior to admission, he developed a symmetrical painful rash over the lower extremities, and bilateral arthralgia in his knees and ankles. Over the ensuing 48 h, palpable purpura developed over the bilateral buttocks, lower legs and thighs (figure 1). He subsequently presented to our outpatient clinic and was admitted for inability to walk due to severe bilateral knee and ankle pain. Swelling in the knees and ankles was not noted. Owing to the history of prior …

Background Rheumatoid vasculitis is an extra articular manifestation of rheumatoid arthritis (RA), that occurs during the course of long standing disease. It is associated with a poor prognosis Objectives To describe the clinical characteristics, incidence, treatment, and outcome of patients diagnosed with rheumatoid vasculitis (RV) at a Rheumatology service of a University hospital during the last 30 years Methods Retrospective study (1984-2014). Location: University hospital. Referral area: 850.000 inhabitants. Medical records of patients with diagnosis of RA were reviewed, from the Rheumatology service data base. Inclusion criteria: patients with RV with histopathological confirmation of vasculitis or high suspicion based on clinical or radiological (arteriography) findings (according to Scott and Bacon criteria) Results A total of 18 patients were included: 6 (33.3%) females and 12 (66.7%) males, mean age of 10.13±66.5 (49-83 years). The clinical characteristics of patients are listed in Table 1. The most common clinical presentation was skin involvement with palpable purpura and digital ulcers. Other common manifestations were multineuritis and systemic involvement. Heart, eyes, or pulmonary involvements secondary to RV were absent. Laboratory tests at diagnosis of RV, showed an increased of acute phase reactants and anemia. All patients had a positive rheumatoid factor, citrullinated antibodies were determined only in five (27.8%) cases, being positive in all of them.Histological confirmation was possible in 8 (44.4%) cases, in the other 10 (55.6%) cases the diagnosis was made with clinical or radiological findings. Most patients were treated with pulse steroids associated with other immunosuppressive drugs. Seven (38.9%) patients died. Three of them (16.7%) secondary to vasculitis, and 4 (22.2%) of other causes.The incidence of rheumatoid vasculitis has decreased considerably in the last decade. Just 4 cases were diagnosed during the last 10 years. Conclusions RV is a rare complication of RA. Cutaneous and neurological involvement is more common. It is more frequent in male smokers with seropositive and nodular RA. Histological diagnosis is difficult. Currently the incidence is decreasing, however mortality is still high. Disclosure of Interest None declared

Cutaneous small vessel vasculitis is a severe neutrophilic vascular inflammation mediated by immune complexes that involves the dermal postcapillary venules. Neoplasms represent 2–5% of all causes of secondary cutaneous vasculitis. We present a case of a 52-year-old man who was admitted due to a 10-day history of respiratory symptoms and myalgia. From the third day of symptoms onwards, the patient noticed the appearance of cutaneous lesions in the lower limbs with palpable purpura and erythematous papules. Additionally, he reported of asthenia, anorexia and weight loss during the prior month. Chest radiography showed an enlarged mediastinum and thoracoabdominal-pelvic CT scan revealed a bulky left hilar mass. Biopsy of the left superior lobar bronchus’ mucosa allowed for the histological diagnosis of diffuse large B-cell lymphoma (DLBL) of the thymus. The patient received symptomatic treatment with improvement of the purpuric lesions and a multiagent chemotherapy regimen was initiated.

Henoch-Schonlein purpura (HSP) is an immune-mediated vasculitis associated with IgA deposition within affected organs. It is the most common vasculitis in children; >90% of reported cases occurred in children

Background Hypersensitivity vasculitis (HV) and Henoch-Schonlein purpura (HSP) are the most common entities included within the category of cutaneous vasculitis (CV). Palpable purpura and histological changes characterized by the presence of leukocytoclastic vasculitis are common in both conditions. Because of that, considerable overlap between them is often seen. It is especially true when the CV occurs in adults. Objectives Our aim was to assess the main clinical differences between HV and HSP in a wide and unselected series of adults with CV from a defined population. Methods We reviewed the clinical records of 297 consecutive adults (age>20years) seen at a single center between January 1975 and December 2012 that were classified as having HSP or HV according to the criteria proposed by Michel et al.1. Results Based on the inclusion criteria 102 adult patients (71 men/31 women) were classified as HSP and 195 (104 men/91 women) as HV. The mean age was similar in both groups (55.8±16.5 years in HSP and 56.8±18.3 years in HV). Precipitating events, usually an upper respiratory tract infection and/or drug intake, were more frequently observed in HV. Both at the beginning of the disease and when the CV was established clinical manifestations were more frequent in patients with HSP than in those with HV. It was the case for gastrointestinal (57.4% vs. 6.8%; p

Essential mixed cryoglobulinaemia or type II cryoglobulinaemia is an important extrahepatic manifestation of chronic hepatitis C. Cryoglobulinaemia results in the deposition of immune complexes in small or medium-sized blood vessels leading to palpable purpura, arthralgia, renal disease and peripheral neuropathy. Posterior reversible encephalopathy syndrome (PRES) is a distinct phenomenon characterised by vasogenic oedema in the posterior circulation of brain. Cryoglobulinaemic vasculitis leading to spontaneous intracranial haemorrhage and PRES syndrome is rarely reported in the medical literature. In this report, we present an unusual case of spontaneous intracranial haemorrhage and PRES secondary to hepatitis C-associated cryoglobulinaemia presenting as right dense hemiplegia. Prompt institution of plasmapheresis resulted in successful resolution of symptoms in our patient, followed by full neurological recovery. To the best of our knowledge, this case describes the first successful use of plasmapheresis in alleviating neurological complications resulting from cryoglobulinaemic vasculitis and PRES secondary to chronic hepatitis C.

Cryoglobulinaemic vasculitis is characterised by immunoglobulin deposition at low temperatures. The most common manifestations are cutaneous involvement, arthralgias, Raynaud's phenomenon, peripheral neuropathy and renal disease. Myopathy is unusual and only a few cases have been reported. Here, we present a 31-year-old woman who developed progressive muscle weakness involving upper and lower extremities, dysphagia, paraesthesias and palpable purpura. Diagnostic studies revealed elevated creatine kinase, diffuse myopathic and sensorimotor axonal neuropathy on electromyography and nerve conduction studies, and inflammatory myopathy on muscle biospsy. Cryoglobulin levels were elevated on two occasions. She responded favourably to cyclophosphamide and high-dose corticosteroids. Cyclophosphamide was continued for 1 year followed by methotrexate. Prednisone was gradually tapered and discontinued 1 year later. She remained in clinical remission after 4 years of follow-up. This case suggests that cryoglobulinaemia should be considered in the differential diagnosis of a patient presenting with inflammatory myopathy.

Background Previously we reported that salivary gland ultrasonography (SGUS) using a simplified score 1,2 is a specific but not a sensitive diagnostic method in primary Sjogren’s syndrome (pSS). The purpose of the present work is to analyse intra-observer validity and association with lymphoma risk factors. We have investigated 160 individuals, of which 30 have been studied at two time-points by the same investigator. Objectives To evaluate association with other lymphoma risk factors and intra-observer validity of SGUS in pSS. Methods SGUS was performed scoring parenchymal inhomogeneity as 0 = completely normal, 1 = slight abnormalities/few hypoechogenic areas, 2 = multiple hypoechogenic areas or 3 = many or confluent hypoechogenic lesions 1 . Assessment of EULAR SS Disease Activity Index (ESSDAI), presence of autoantibodies, immunoglobulin levels, complement levels, lymphocyte subtypes, parotid swelling and palpable purpura were assessed in pSS patients. Salivary gland biopsies were evaluated with regard to focal sialadenitis and germinal centre (GC)-like structures by routine staining and light microscopy, when available. Results Salivary glands of 105 pSS patients fulfilling the 2002 American European Consensus Criteria and of 56 controls (healthy n=10, sicca syndrome n=17, other rheumatic diseases 29) were studied. At first examination 54% of the pSS patients and none of the controls showed the characteristic pattern of multiple or confluent hypoechogenic rounded lesions typical for pSS, (p Patients with SS-typical US pattern had significantly higher ESSDAI score (8.6 vs. 3.3) and IgG levels (22.2 vs.14.9g/l) (both p In 30 individuals repeated SGUS was performed with a median 12 (range 2-23) months interval. Scores from the two investigations were highly correlated. Kappa: 0.54. Only 5 patients newly acquired the classical pSS pattern, mainly patients with short disease duration at baseline. Conclusions SGUS is a non-radiating bedside investigation with high specificity for pSS. Patients with these lesions have higher disease activity and more often other risk markers for lymphoma development. Given the fact that pSS usually is characterized by a stable disease state and not treated with potent medication, the high consensus between the repeated investigations signals high intra-observer reliability of the method. References Hocevar A et al. Ultrasonographic changes of major salivary glands in primary Sjogren’s syndrome. Diagnostic value of a novel scoring system. Rheumatology (Oxford) 2005;44:768-72. Theander E et al. Diagnostic utility and prognostic value of parotid gland ultrasonography in primary Sjogren’s Syndrome: The classical hypoechogenic pattern of parenchymal abnormality is highly specific, associated with higher disease activity and systemic disease. ARD 2012 June S 3 : 16,904 Disclosure of Interest None Declared

Background Vasculitides are generally syndromes of unknown etiology. Although drugs are possible precipitating factors, there are few published series on clinical characterization, and the type of drugs involved. Objectives Our aim was to study a wide series of unselected drug-induced cutaneous vasculitis. Methods Retrospective study of patients diagnosed consecutively in the same Universitary hospital as having cutaneous vasculitis due to previous drug intake. Intake of this drug must be new in polymedicated patients and occur in the week before the onset of the vasculitis. Results According to the above criteria, we found 232 patients (129 men/103 women, mean age 35.9±1.8 years) with cutaneous vasculitis secondary to a new drug in the previous week. The drugs most frequently involved were: s-lactam antibiotics [penicillin (80 cases, 34.5%), cephalosporins (12 cases, 5.2%)], quinolones (11 cases, 4.7%), anti-inflammatory [diclofenac (4 cases, 1.7%), ibuprofen (3 cases, 1.3%), aceclofenac (1 case, 0.4%), ketoprofen (1 case, 0.4%) and other NSAIDs (14 cases, 7.75 %)], diuretics (2 patients, 0.9%). The most common clinical manifestations of 232 cases were a) skin (100%) as palpable purpura (83.2%), erythema (3.0%), bullous (3.4%), ulcers (2.2 %), urticaria (3.0%) and other (5.2%). b) Digestive (88 cases, 37.5%), abdominal pain (34.1%), nausea and vomiting (12.1%) and gastrointestinal bleeding (melena, hematochezia, or positive stool Guaiac test) (7.7%). c) joint symptoms (117 cases, 50%), arthritis (14.6%). d) Fever (56 cases, 23.3%). e) nephropathy (80 cases, 33.6%), 29 of them associated with renal insufficiency. The main laboratory data were ESR (41.0%), leukocytosis (24.6%), anemia (18.1%), positive rheumatoid factor (17.9% of 123 cases tested), positive ANA (21.1% of 128 cases tested), low C3 and / or C4 (4.4% of 128 cases tested), cryoglobulin positive (26.4% of 72 cases tested) and ANCA positive (4.8% of 62 cases tested). In case of positive immunological tests they were at low titers and no patient in the folow-up developed a connective tissue disease or a systemic necrotizing vasculitis. Besides precipitating drug suspension and rest, in 102 patients (43.9%) was used the following pharmacological treatment: corticosteroids (67 cases, 28.9%), azathioprine (1 case), cyclophosphamide (4). After a mean follow up of 25.4±3.4 months, relapses were observed in 18.1% of patients, chronic persistent microhematuria (4 cases) and mild (7 cases). Conclusions Drugs mainly produce cutaneous vasculitis, although it may have systemic involvement it is usually mild. The final outcome is benign. Acknowledgements This study was supported by grants from “Fondo de Investigaciones Sanitarias”PI12/00193 (Spain). This work was also partially supported by RETICS Program, RD08/0075 (RIER) from “Instituto de Salud Carlos III” (ISCIII) (Spain). Disclosure of Interest None Declared

Background Cutaneous vasculitis (CV) can be a clinical manifestation of a severe infection. In such cases a delayed diagnosis or the use of immunosuppressive therapy may impair the prognosis. Objectives Our objective was to determine the frequency, subtypes of major infections and clinical characteristics of CV associated to severe infections. Methods Study of a large series of 817 unselected patients with CV seen in the same hospital. A CV was considered as secondary to infection when it was associated to a severe infection and antibiotics were needed for the resolution of the vasculitis. The diagnosis of CV required histological confirmation. Results In 27 (22 men/5 women) (mean age±SD; 52.5±17.6 years) of 817 patients (3.3%) the CV was associated to a major underlying infection: pneumonia (8 cases), endocarditis (6 cases), meningitis (4 cases), abdominal infection (3 cases), septic arthritis (2 cases), septicemia (2 cases), septic bursitis (1 case), and 1 urinary tract infection (1 case). In all cases, the vasculitic skin lesions were observed at the beginning of the clinical syndrome. The types of skin lesions were palpable purpura (26 patients) and erythematous lesions (1 case). The most common location was the lower limbs. The mean duration of skin lesions was 15±6.9 days. In addition to the specific clinical manifestation of each infection, other clinical manifestations were fever (21 patients) and constitutional syndrome (5 patients). There was not severe visceral vasculitic involvement in any case. Laboratory findings showed, leukocytosis (27 patients), elevated ESR (18 patients), anemia (16 cases), mild hematuria (8 cases), positive rheumatoid factor (6 patients), positive ANA (5 patients), decreased C3 (2 patients), and circulating cryoglobulins (3 cases). None of the 27 patients developed a connective tissue disease in the follow-up. All the patients were treated with antibiotics, and 7 required the use of low-dose corticosteroids for the resolution of the skin lesions. There was a significant delay in the diagnosis of 2 patients with endocarditis of 32 and 45 days, respectively. Two patients died due to complications of the infection, the remaining, recovered completely. Conclusions Although uncommon, CV may be a clinical manifestation of a serious infection. In this case it may be a red flag that may help the clinician to set up an early diagnosis and treatment of a severe infection. Acknowledgements This study was supported by grants from “Fondo de Investigaciones Sanitarias” PI12/00193 (Spain). This work was also partially supported by RETICS Program, RD08/0075 (RIER) from “Instituto de Salud Carlos III” (ISCIII) (Spain). Disclosure of Interest None Declared

Background Adult Henoch-Schonlein purpura (HSP) has a significantly higher frequency of gastrointestinal (GI) bleeding, compared with childhood HSP. However, the clinical characteristics of GI bleedings in adult HSP are still largely unknown, since the small number of studies performed included case reports and a few case series. Objectives To investigate clinical manifestations, and endoscopic features of GI lesions in adult HSP patients with GI bleeding. Methods Twenty-four adult HSP patients, who had evidence of GI bleeding and underwent both upper GI endoscopy and colonoscopy, were included for this study. The controls were twenty-seven adult HSP patients with no evidence of GI bleeding. Results Patients with GI bleeding showed a significantly higher frequency of palpable purpura on the upper extremities ( p

Objectives To investigate pregnancy and fetal outcome in patients with an established diagnosis of primary Sjogren’s syndrome (pSS) Methods The clinical charts of 1073 women with pSS from four rheumatology centres were retrospectively evaluated. When a pregnancy has occurred after pSS diagnosis, the patient was personally interviewed to obtain more detailed information regarding obstetric history; obstetric clinical charts were reviewed as well. Results Patients’ mean age was 59 yr (17-89), mean age at diagnosis 51,4 yr; 138/1073 (12,8%) were diagnosed before 35 yr. Thirty-five women (31 with anti-SSA/Ro and/or anti-SSA/La antibodies) with an established diagnosis of pSS had 44 pregnancies which ended with the delivery of 39 newborns. Two miscarriages, 2 fetal death and one induced abortion were recorded. Mean age at the latest pregnancy was 34,6 yr (range 29-44), mean number of pregnancy 1,25 (1-3); 17/39 cesarean sections were performed, mean pregnancy length was 38,5 week (range 32-43) with 6 preterm delivery. The mean Apgar score at 5 minute was 8,9 (range 5-10), mean birth weight was 2934 mg (range 826-4060). Congenital heart block (CHB) occurred in 2 newborns of 31 mothers with anti-SSA and/or SSB antibodies (6,45%), with fatal outcome. Other 2 infants had cardial incontinence and a mild interatrial defect, respectively. During pregnancy one patient presented thrombocytopenia and another palpable purpura. In 4/39 pregnancies (10,2%) a flare of disease activity was observed (arthralgia/arthritis and central nervous system involvement) within a year from delivery. Conclusions Even if pSS generally starts after menopause, it can appear during the childbearing age. pSS can have successful pregnancies, which might be followed by a mild relapse. CHB is a fearful complication for women with anti-SSA/Ro and or anti-SSB/La antibodies. Disclosure of Interest None Declared

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. Conclusions CLV presenting as a paraneoplastic syndrome is a rare condition. The most common underlying malignancy is generally hematological. The prognosis depends on the underlying neoplasia. Disclosure of Interest None Declared

Background The clinical presentation of HSP is more severe among adults and tends to be atypical: higher rate of severe and atypical GI problems and delayed renal complications. Management in particular of refractory HSP remains controversial and results so far have been conflicting. We present a case of adult HSP with intractable GI involvement treated with repeat courses of Rituximab over the last 9 years. Efficacy of Rutiximab has been reported in 3 children1 and 2 adult cases of refractory HSP but in all cases remission was achieved with 1- to 2 cycles of Rituximab. Our case is the first case reporting on the 9 year experience with RTX used as a remission maintenance therapy in HSP. Our case alongside with other reports suggests the use of RTX in non-ANCA vasculitis and highlights the need for studies of RTX as remission maintenance therapy in small vessel vasculitis and its long term safety profile. Objectives A 61 year old female patient was referred to our centre in 2001 with progressive gut vasculitis and leucocytoclastic skin lesions, preceded by an 18 month history of abdominal pain, nausea, vomiting, haematochezia and palpable purpura. She also sustained right iliofemoral artery thrombosis and was fully anticoagulated. The diagnosis of HSP was confirmed by a skin and small bowel biopsy showing immune complex deposition, strongly stained for IgA. In 2002 she completed six cycles of Cyclophosphamide and was started on maintenance therapy with Azathioprine. However, she relapsed with severe GI symptoms and was treated with further eight cycles of Cyclophosphamide. Despite being heavily immunosupressed she continued to experience recurrent episodes of abdominal pain and haematochezia requiring treatment with intravenous methylprednisolone. In 2002 she received her first cycle of 500 mg RTX two weeks apart, resulting in clinical remission and was followed by Methorexate as a maintenance therapy. 11 months post RTX therapy she experienced further relapse of GI and skin symptoms. She has received repeat cycles since with a mean duration of 14 months in between the retreatment. In 2008 the patient developed severe haematemesis accompanied by acute right limb ischaemia requiring right tibial embolectomy. Subsequent endoscopy revealed presence of vasculitic lesions in the stomach. Again she responded well to RTX and stayed in remission for a year. Last relapse of severe GI symptoms happened in 2011 after 2 years of remission. Retreatment with RTX is usually given at onset of recurrent symptoms. Results IgA plays a central role in pathogenesis of HSP and inhibition of IgA production might be a good approach to treat HSP. Although RXT should not deplete immunoglobulin secreting plasma cells, given its efficacy of Bcell depletion in other Bcell mediated disorder suggests use in refractory HSP. In our case the patient continues to be treated on demand with RTX therapy but the timing of retreatment remains unclear and this question expands to other type of small vessel vasculitis. It also remains unclear which remission maintenance treatment patient should receive following successful first remission induction with RTX to prevent relapses. Conclusions RTX can be considered in cases of refractory HSP References K Donnithorne et al. RTX for severe refractory chronic HSP. Disclosure of Interest None Declared

Fournier's gangrene is a form of rapidly progressive necrotising fasciitis involving the soft tissues of the male genitalia. The treatment for this urologic emergency is immediate surgical debridement. Misdiagnosis of Fournier's may lead to unnecessary surgical interventions, making careful recognition of systemic signs of illness crucial. The authors present a case of a 48-year-old patient who presented emergently with massive scrotal oedema, erythema and pain suspicious for Fournier's gangrene and systemic signs of illness, including palpable purpura and leukocytoclastic vasculitis on biopsy.

A 53 year old, afebrile woman presented to the dermatology clinic with a two week history of petechial rash on her lower extremities that had progressed up to her arms. In addition, she also had pain in the wrists, knees, and elbows. Approximately five months earlier, she had been diagnosed with group A streptococcal pharyngeal infection, but she had not been treated with antibiotics. She was currently taking salbutamol, famciclovir, levothyroxine, lovastatin, and varenicline. A systems review was unremarkable and she had no reported haematuria, abdominal pain, or bloody stools. A physical examination found multiple petechial macules, papules, and purpuric plaques, which were more numerous on the legs than on the arms (figure[F1]). A complete blood count and coagulation studies were normal. Urinalysis showed haematuria and proteinuria. A skin biopsy of a petechial papule revealed leucocytoclastic vasculitis, with a granular IgA reactivity around the blood vessels in the papillary dermis. ### Short answers ### Long answers #### 1 Investigations Cutaneous palpable purpura may signify small vessel vasculitis, which has many possible causes (box 1).1 2 Histological findings of leucocytoclastic vasculitis include fibrin deposition in the vessel walls, red blood cell …

Cutaneous vasculitis is frequently located on the lower limbs. We describe a patient who developed palpable purpura affecting the penis as the presenting sign of more widespread lesions of Henoch-Schönlein purpura.

Cryoglobulins are immunoglobulins which precipitate at reduced temperature and that redissolve by warming the serum sample to 37 °C. Mixed cryoglobulinaemia may manifest clinically as skin, articular, renal, and peripheral nerve complications.1 To ensure optimal detection, blood samples must be obtained and preserved at 37 °C. We report on a patient whose clinical presentation was suggestive of cryoglobulinaemia. Because cryoglobulins had been either undetectable or found at very low levels for several years despite repeated careful blood sample examinations with conventional assays, we initially thought he had Henoch-Schonlein purpura. The recent use of a modified assay finally led us to diagnose mixed cryoglobulinaemia. Included below is a description of the method used for cryoglobulin detection, emphasising the importance of in vitro calcium concentration. A 52 year old man with multiple lipoma had a 20 year history of polyarthralgias affecting elbows, wrists, hands, knees, and feet, a 10 year history of Raynaud's disease affecting the hands and feet, and a seven year history of palpable purpura. His sister had coeliac disease. In June 1996 he developed attacks of abdominal pain concomitantly with arthralgias and palpable purpura of both legs. Serum creatinine was 95 μmol/l. Gammaglobulins were low (4.2 g/l) on serum protein electrophoresis. Serum concentrations of immunoglobulins were 4.49 g/l for IgG (normal range 6.42–11.92), 1.84 g/l for IgM (normal range 0.52–1.47), and 2.51 g/l for IgA (normal range 1.05–3.36). Tests for rheumatoid factors, including the Rose-Waaler test (Sanofi Pasteur, Marnes La Coquette, France), were positive (table 1), but other serum autoantibodies remained negative, including antinuclear, anti-DNA, and antineutrophil cytoplasmic antibodies. Complement concentrations were notably down, both …