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Competing Interests: Competing interests: None declared.

Introduction: Rheumatoid purpura is the most common vasculitis in children, and its renal involvement determines the prognosis. To date, no national protocol exists for its management. A protocol was drafted for the French Grand Ouest inter-region in 2011 in order to standardize practices., Objectives: The main objective is to evaluate renal sequelae with a median follow-up of 2 years since the implementation of this protocol. The secondary objectives are to evaluate the different therapeutic and diagnostic management., Method: Inclusion of all children from 2006 to 2018 with nephropathy due to rheumatoid purpura followed in the university hospitals of Rennes, Nantes, Tours, Angers and Brest., Results: 169 patients were included, of whom 104 were treated accroding to protocol and 65 differently. Sequels at 2-year follow-up concerned 27.0% of patients with no significant difference according to whether or not the protocol was followed. A significant decrease of 26.1% in the number of renal biopsies was observed in the group that followed the protocol. The latter was performed with a median delay of less than 30 days., Conclusion: The protocol allowed a standardization of practices without deleterious consequences at 2 years of follow-up and a decrease in renal biopsy punctures. It is in agreement with the recommendations of KDIGO (Kidney Disease Improving Global Outcomes) and European experts. On the other hand, in view of recent studies and the physiopathology, immunosuppressive drugs other than corticosteroids could be introduced earlier in severe forms.

Renal involvement represents the major long-term morbidity associated with IgA vasculitis (IgAV). Our aim was to evaluate clinical characteristics and long-term renal outcomes of IgAV in pediatrics and adults comparing to IgA nephropathy (IgAN). Our retrospective study included children and adults with IgAV and IgAN patients, admitted in a 13-year period (2007-2019) to rheumatology clinics and in hospital pediatric and internal medicine departments. We compared frequencies of clinical manifestations, laboratory findings, treatments, long-term outcomes at 1 year follow-up, including all-cause mortality and dialysis until the end of follow-up time. A total of 60 adult IgAV, 60 pediatric IgAV and 45 IgAN patients were evaluated. Adult IgAV patients were significantly older than IgAN patients (53.1 ± 17.4 years vs 45.1 ± 15.7 years respectively, P = .02) and had significantly higher rates of cardiovascular comorbidities. The risk and time to dialysis were similar among IgAN and adult IgAV groups. Yet, overall mortality at long term follow up was higher in IgAV adult group compared to IgAN. No dialysis or renal transplantation were reported in pediatric IgAV patients. IgAV and IgAN adult patients were comparable regarding risk of end stage renal disease. Of note, high mortality rates were observed among adult IgAV group., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

ADULT IGA VASCULITIS. IgA vasculitis previously named rheumatoid purpura is a rare systemic vasculitis in adults involving small vessels and associated with the presence of immunoglobulin A deposits. IgA vasculitis is often triggerd by infections, taking medication and vaccination. It is characterised by the presence of vascular purpura associated with joint (arthralgia), gastrointestinal (abdominal pain) and sometimes renal involvement (glomerulonephritis). Gastrointestinal involvement can be lifethreatening (bowel perforation), while the longterm prognosis is shaped by the renal involvement (renal failure). In most cases, the disease has a good outcome and only symptomatic treatment is recommended. In more severe forms, corticosteroids, combined with another immunosuppressant or biotherapy, may be discussed on a casebycase basis., Competing Interests: Les auteurs déclarent n’avoir aucun lien d’intérêts. Les auteurs remercient le Dr Kervarrec, anatomopathologiste au CHU de Tours, pour la réalisation des photographies de l’histologie cutanée et rénale.

Rationale: Immunocompromised patients who developed varicella-zoster virus (VZV)-associated disseminated intravascular coagulation (DIC) previously included recipients of bone marrow, hematopoietic stem cell, or organ transplantations, patients with primary nephropathy receiving corticosteroid therapy, cancer patients receiving chemotherapy, and patients with human immune deficiency virus infection. The case reported here is novel because, to our knowledge, there has been no report of VZV-associated DIC after the onset of Henoch-Schönlein purpura (HSP)., Purpose: To report the successful treatment of a novel pediatric case with VZV-associated DIC secondary to HSP., Diagnosis and Intervention: An 8-year-old girl developed VZV-associated DIC 24 days after diagnosis of HSP with renal and gastrointestinal involvement. She was treated with methylprednisolone at a local hospital for 19 days, and suddenly developed fever starting from day 4 in our hospital. Her fever persisted with vesicular skin rashes on her back, strong abdominal and lower back pain, epistaxis, hematochezia, erosion and bleeding on her lips, in her mouth and at puncture sites on day 5. She was diagnosed with DIC with the laboratory evidence of dramatically decreased platelet count and fibrinogen, prolonged activated partial thromboplastin time and prothrombin time, and increased fibrin degradation products including d-dimers. She also developed multiple organ dysfunction syndrome. On day 7, the patient VZV nucleic acid result turned out to be positive. Methylprednisolone treatment was discontinued, and she was given a multi-modality therapy including medications of acyclovir and antibiotics, intravenous gamma-immunoglobulin, various blood product transfusions, continuous renal replacement therapy, plasma exchange, and administration of liver and gastrointestinal system protection drugs., Outcomes: The patient multi-organ function damage gradually recovered. After VZV control, the patient was treated with oral methylprednisolone again for HSP with nephritis. Urine analysis was normal 1 year later, and oral hormone was discontinued. No complication or relapse occurred during 2 years of follow-up., Significance: This case report, for the first time, adds HSP treated with corticosteroids to the spectrum of clinical conditions that progressed to life-threatening secondary varicella-associated DIC. Early identification of varicella infection and DIC, combined with timely antiviral, immunoglobulin transfusion, plasma exchange, and other combined therapies are essential for saving patients' lives., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Patients with IgA nephropathy (IgAN), including Henoch-Schönlein purpura nephritis (HSP), who present with rapidly progressive glomerulonephritis (RPGN) have a poor prognosis despite aggressive immunosuppressive therapy. The utility of plasmapheresis/plasma exchange (PLEX) for IgAN/HSP is not well established. This systematic review aims to assess the efficacy of PLEX for IgAN and HSP patients with RPGN. A literature search was conducted using MEDLINE, EMBASE, and through Cochrane Database from inception through September 2022. Studies that reported outcomes of PLEX in IgAN or HSP patients with RPGN were enrolled. The protocol for this systematic review is registered with PROSPERO (no. CRD42022356411). The researchers systematically reviewed 38 articles (29 case reports and 9 case series articles) with a total of 102 RPGN patients (64 (62.8%) had IgAN and 38 (37.2%) had HSP). The mean age was 25 years and 69% were males. There was no specific PLEX regimen utilized in these studies, but most patients received at least 3 PLEX sessions that were titrated based on the patient's response/kidney recovery. The number of PLEX sessions ranged from 3 to 18, and patients additionally received steroids and immunosuppressive treatment (61.6% of patients received cyclophosphamide). Follow-up time ranged from 1 to 120 months, with the majority being followed for at least 2 months after PLEX. Among IgAN patients treated with PLEX, 42.1% ( n = 27/64) achieved remission; 20.3% ( n = 13/64) achieved complete remission (CR) and 18.7% ( n = 12/64) partial remission (PR). 60.9% ( n = 39/64) progressed to end-stage kidney disease (ESKD). Among HSP patients treated with PLEX, 76.3% (n = 29/38) achieved remission; of these, 68.4% ( n = 26/38) achieved CR and 7.8% achieved ( n = 3/38) PR. 23.6% ( n = 9/38) progressed to ESKD. Among kidney transplant patients, 20% (n = 1/5) achieved remission and 80% ( n = 4/5) progressed to ESKD. Adjunctive plasmapheresis/plasma exchange with immunosuppressive therapy showed benefits in some HSP patients with RPGN and possible benefits in IgAN patients with RPGN. Future prospective, multi-center, randomized clinical studies are needed to corroborate this systematic review's findings.

IgA vasculitis - similarities and differences to IgA nephropathy Abstract. IgA vasculitis (IgAV), formerly called Henoch-Schönlein Purpura (HSP) is an immune complex-mediated vasculitis of small vessels typically affecting the skin, gastrointestinal tract, and the kidneys. Based on distinct histopathological and pathophysiological commonalities of IgAV and IgA nephropathy (IgAN) they are viewed as part of a spectrum of IgA-mediated inflammatory syndromes. While the disease course in children is most often benign, IgAV has a high risk of renal and gastrointestinal complications when first appearing in adulthood. Acute morbidity and mortality in IgAV are determined by gastrointestinal complications such as intussusception or perforation, while chronic morbidity depends on renal involvement. Nephritis symptoms can appear, subclinically, many weeks after the initial manifestation of IgAV, and can therefore be missed. Continuous monitoring of renal parameters and blood pressure is therefore recommended even after apparent remission of the disease. As there are no data from randomized controlled trials available, the treatment of IgAV is currently based on consensus-based expert opinions.

Cutaneous Vasculitides - Clinical Manifestations, Diagnosis, and Aetiology Abstract. Vasculitides are a heterogeneous group of diseases that are classified differently, for example according to the size of the affected vessel or according to primary and secondary causes. The skin is most frequently affected; it can be involved both as single organ vasculitis and in the context of systemic forms. The combination of skin lesions, their anatomical location and information on the time course provide clues for a differential diagnosis. Purpura, blisters, necrosis, ulcerations and possibly a livedo are characteristic manifestations. Constitutional symptoms such as weight loss, exhaustion, fever, and arthralgias are indicative of a systemic form. It is important to differentiate vasculitides from vasculopathies, which can manifest similarly. The most common form in adults is cutaneous leukocytoclastic angiitis, in children IgA vasculitis (Schönlein-Henoch purpura). Various triggers are possible: infections, drugs, autoimmune diseases, and malignancies, whereby up to 50% remain etiologically unexplained. Skin biopsies and laboratory parameters, if necessary supplemented with imaging, are important steps in the clarification process. Treatment is primarily directed at the elimination of a possible triggering cause. Idiopathic cutaneous leukocytoclastic angiitis usually resolves spontaneously; treatment is symptomatic. In more severe cases, topical corticosteroids or calcineurin antagonists are primarily used. In case of therapeutic resistance, systemic immunosuppressants are recommended.

IgA-vasculitis is a systemic small-vessel leucocytoclastic vasculitis and is associated with a high morbidity. The disease can progress to IgA-vasculitis with nephritis (IgAVN) which can result in chronic renal failure. Complement activation is involved in the pathogenesis of IgA-vasculitis. A recent study has shown that cutaneous C3c deposition in IgA-vasculitis is associated with a higher risk to develop IgAVN. In the current study we investigated the different complement pathways that are activated in cutaneous IgA-vasculitis in order to reveal potential targets for intervention. In addition, we analyzed the association of complement factors with IgAVN and the clinical course of the disease. In this retrospective study, the clinicopathological features of 17 patients with IgA-vasculitis were compared with 25 non-IgA-vasculitis cases. Deposition of immunoglobulins and complement was analyzed by direct immunofluorescence for IgA, IgG, IgM, C1q, C4d, properdin, mannan-binding lectin (MBL), ficolin-2 (FCN2), MBL-associated serine protease 1/3 (MASP1/3), MASP2 and C3c. The vascular intensity and positive area was scored on a nominal scale and cumulative score was calculated by multiplying the intensity x area. Properdin was positive in 82% of IgA-vasculitis cases, reflecting alternative pathway activation. C4d was positive in 88% of IgA-vasculitis cases reflecting classical and/or lectin pathway activation, although only 12% of cases were positive for C1q. Lectin pathway activation was demonstrated by deposition of MBL (47%), MASP1/3 (53%) and MASP2 (6%) while FCN2 was found negative. Significantly more deposition of MASP1/3 was found in IgA-vasculitis versus non-IgA-vasculitis. This study demonstrates for the first time activation of lectin and alternative pathways in cutaneous manifestations of IgA-vasculitis. Hence, drugs that intervene in these complement pathways may be an interesting more targeted alternative to the current drugs, in reducing local cutaneous symptoms of the disease, with potentially less side-effects. No association was found between complement activation and IgAVN and/or response to therapy. Therefore, it is unlikely that intervention in complement activation will lead to a better clinical course of the disease., (Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: A retrospective study was conducted in order to investigate and describe the characteristics of Immunoglobulin A vasculitis (IgAV), previously known as Henoch-Schӧnlein purpura, in the paediatric population of a community-based healthcare delivery system in the Italian region of Abruzzo., Methods: This is a population-based retrospective chart review of the diagnosis of IgAV in children ages 0 to 18, admitted to the Department of Paediatrics of Chieti and Pescara between 1 January 2000 and 31 December 2016. All children enrolled presented with clinical symptoms and laboratory findings and met the EULAR/PRINTO/PRES 2008 criteria., Results: Two-hundred-eight children met the criteria for IgAV, with the highest incidence reported among children below 7-years of age. A correlation with recent infections was found in 64% of the cohort; the onset was more frequently during the winter and fall. Purpura had a diffuse distribution in the majority of patients; joint impairment was the second most frequent symptom (43%), whereas the gastrointestinal tract was involved in 28% of patients., Conclusions: Hereby, we confirm the relative benignity of IgAV in a cohort of Italian children; with regards to renal involvement, we report a better outcome compared to other studies. However, despite the low rate of renal disease, we observed a wide use of corticosteroids, especially for the treatment of persistent purpura., (© 2021. The Author(s).)

The amino acid-based formulae were extensively added to diet of children for the treatment of Henoch-Schonlein purpura (HSP), and the nutrition and growth situation of children were evaluated after giving new dietary intervention. Patients were randomly divided into restricted diet group (n=30) and dietary guidance group (n=30). Besides, 30 cases with bronchiolitis who had normal diet were selected as the control group. The dietary questionnaire was designed to record the types and intakes of various foods taken by children every day, and the intake levels of nutrients were analyzed. Physical examination, biochemical analysis of blood and urine routine were carried out to evaluate the effect of dietary guidance on their growth and development. The results showed that restricted diet group had lower levels of nutrient intake and the actual/recommended percentage. However, overall nutrient intake level of the dietary guidance group was higher, basically equal to the recommended intake level. Besides, the actual intake and actual/recommended percentage of nutrients of dietary guidance group were significantly higher than those of restricted diet group (p<0.05). Dietary guidance can improve nutrients and protein intake of children with HSP, and reduce the relapse of rash and incidence of complications., Competing Interests: Competing interests: None declared., (© American Federation for Medical Research 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Competing interests: None declared.

The coronavirus disease 2019 global pandemic is reshaping our understanding of medicine, including the diagnostic approach to common medical presentations. We describe a novel case of a 3-year-old male with a clinical diagnosis of Henoch-Schonlein Purpura vasculitis with concurrent SARS-CoV-2 infection. This case highlights a potentially newly described presentation of coronavirus disease 2019 infection., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To explore the reasonable and effective enteral nutrition regimen for children with abdominal Henoch-Schönlein purpura (HSP)., Methods: A retrospective analysis was performed on the medical data of children with abdominal HSP who were hospitalized from August 2013 to August 2018. According to the starting time of enteral nutrition after abdominal pain relief, the children were divided into three groups: < 24 hours ( n =68), 24-48 hours ( n =64), and 48-72 hours ( n =60). According to the type of enteral nutrition, they were divided into another three groups:amino acid-based formula ( n =53), extensively hydrolyzed lactoprotein formula ( n =67), and normal diet ( n =72). The recurrence rate of clinical symptoms and degree of satisfaction among family members were compared between groups. Based on the retrospective analysis, 166 children with abdominal HSP were enrolled in a prospective study. They were given extensively hydrolyzed lactoprotein formula after abdominal pain relief. According to the feeding time after abdominal pain relief, they were divided into three groups: < 24 hours ( n =52), 24-48 hours ( n =59), and 48-72 hours ( n =55). The three groups were compared in terms of the recurrence rates of abdominal pain, rash, and hematochezia, the rate of use of parenteral nutrition and intravenous steroids, and the incidence rate of weight loss at discharge., Results: The retrospective analysis showed that the children who were given extensively hydrolyzed lactoprotein formula for enteral nutrition at 24-48 hours after abdominal pain relief had a lower recurrence rate of clinical symptoms and the highest degree of satisfaction among their family members ( P < 0.0167). The prospective study showed that the children who were given extensively hydrolyzed lactoprotein formula for enteral nutrition at 24-48 hours after abdominal pain relief had lower recurrence rates of rash and abdominal pain, a lower rate of use of parenteral nutrition, and a lower incidence rate of weight loss at discharge ( P < 0.05)., Conclusions: It is reasonable and effective to start the feeding with extensively hydrolyzed lactoprotein formula at 24-48 hours after abdominal pain relief in children with abdominal HSP.

Background: Though outcome differences between children and adults with immunoglobulin A vasculitis (IgAV) has been well documented, it remains unclear if disease features in pediatric IgAV patients vary with onset age. We aimed to explore clinical features and prognosis of pediatric IgAV stratified by onset age., Methods: We retrospectively reviewed records of patients under 18 years old diagnosed with IgAV from January 1999 to December 2018 in one tertiary medical center in Taiwan. Patients were grouped by onset age: ≤ 6 years old, 6-12 years old (> 6, ≤ 12), and 12-18 years old (> 12, < 18). Demographics, laboratory data, incidence of gastrointestinal, renal, and joint involvement, corticosteroid dependence, recurrence, and refractory disease were analyzed. Recurrence was defined as disease flare-up after complete remission and discontinuation of all medications for at least 3 months. Corticosteroid dependence was defined by more than 6 weeks of daily oral corticosteroid intake. Refractory disease was defined as not achieving complete remission 6 months after disease onset. Statistical analysis was performed using R software (v3.6.0)., Results: There were 484 IgAV patients, with an onset age of 6.10 (4.72-8.58) (median (IQR)) years old. There were 234 (48.3%) patients ≤6 years old, 210 (43.4%) 6-12 years old, and 40 (8.3%) 12-18 years old. One hundred and thirty (26.9%) patients had renal involvement, which was more frequent in older children (≤ 6 years old, 18.4%; 6-12 years old, 31.0%; 12-18 years old, 55.0%; p <  0.001). There were 361 patients (74.6%) with joint involvement; younger children were affected more frequently (≤ 6 years old, 82.1%; 6-12 years old, 71.9%; 12-18 years old, 45.0%; p <  0.001). Gastrointestinal involvement was present in 311 (64.3%) patients, showing no difference among age groups. There were 46 patients (9.5%) with recurrent IgA vasculitis, 136 (28.1%) with corticosteroid dependent and 76 (15.7%) with refractory disease. Corticosteroid dependence and refractory disease occurred more frequently as onset age increased (p <  0.001)., Conclusion: Pediatric IgAV with different onset ages are associated with distinct clinical manifestations and outcomes. The risk of developing corticosteroid dependence, refractory disease and renal involvement increased with onset age.

Immunoglobulin A vasculitis (IgAV, formerly Henoch-Schönlein purpura) is a systemic inflammatory disease affecting small vessels. While it is common and usually benign in childhood, in adults it is rarer has a more severe course. Its main manifestations are cutaneous purpura, arthralgias or arthritis, acute enteritis and glomerulonephritis. Renal involvement is associated with a poor prognosis in adults. The treatment of adult-onset IgAV is still a matter of debate: although in patients with a non-severe phenotype remission can occur spontaneously, more severe cases may need immunosuppressive therapy. There are some areas of uncertainty with respect to the efficacy of immunosuppressive regimens: almost all data come from studies performed in children or from patients with IgA nephropathy and/or IgA-crescentic glomerulonephritis. The only randomised study performed in adults with IgAV and renal involvement showed that immunosuppressive therapy with cyclophosphamide did not improve renal outcome nor did it affect patient survival. The possible efficacy of other drugs is reported only in small case series. Recent evidences show that rituximab could be an effective therapeutic option for adult-onset IgAV, but this also needs to be confirmed in controlled trials. In this review, we focus on therapeutic options for adult-onset IgAV treatment, and discuss the main results of the studies performed so far., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objective: To investigate the clinical effect of acupuncture combined with western medicine in the treatment of children with abdominal Henoch-Schonlein purpura with spleen-stomach damp-heat syndrome., Methods: A total of 60 children with abdominal Henoch-Schonlein purpura with spleen-stomach damp-heat syndrome were randomly divided into treatment group and control group, with 30 patients in each group. The patients in the control group were given Hydroprednisone 2 mg•kg -1 •d -1 , and in addition to the treatment in the control group, those in the treatment group were given acupuncture at Tianshu (ST25), Neiguan (PC6), Zusanli (ST36), Zhongwan (CV12), Qihai (CV6), and Sanyinjiao (SP6) once a day, with a needle retaining time of 15 minutes. Both groups were treated for 7 days. The scores of abdominal pain, hematochezia, hematemesis, vomiting, poor appetite, abdominal distension, purpura, occult blood in stool, and abdominal ultrasound were determined before and after treatment, and the time to the disappearance of abdominal pain was observed. Clinical outcome was evaluated., Results: The treatment group had a significantly higher effective rate than the control group ［96.7% (29/30) vs 80.0% (24/30), P <0.05］. Both groups had significant reductions in the scores of abdominal pain, hematochezia, hematemesis, poor appetite, abdominal distension, purpura, occult blood and abdominal ultrasound and the total score after treatment ( P <0.05), and compared with the control group after treatment, the treatment group had significantly lower scores of abdominal pain, poor appetite, abdominal distension, and abdominal ultrasound and total score ( P <0.05). The treatment group had a significantly shorter time to disappea-rance of abdominal pain than the control group ( P <0.05)., Conclusion: Acupuncture combined with western medicine has a better clinical effect than western medicine alone in the treatment of abdominal Henoch-Schonlein purpura with spleen-stomach damp-heat syndrome and can significantly improve clinical symptoms and signs and shorten the time to disappearance of abdominal pain.

Henoch-Schönlein purpura, now called immunoglobulin A (IgA) vasculitis, is a systemic, immune complex-mediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain. It is the most common vasculitis in children but can also occur in adults. Diagnostic testing is required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy. Imaging or endoscopy may be needed to assess organ complications. IgA vasculitis spontaneously resolves in 94% of children and 89% of adults, making supportive treatment the primary management strategy. However, a subset of patients experience renal involvement that can persist and relapse years later. Additional complications can include gastrointestinal bleeding, orchitis, and central nervous system involvement. Systematic reviews have shown that steroids do not prevent complications and should not be used prophylactically. However, randomized trials have demonstrated success with high-dose steroids, cyclosporine, and mycophenolate in treating glomerulonephritis and other complications. Long-term prognosis depends on the extent of renal involvement. Six months of follow-up is prudent to assess for disease relapse or remission.

IgA vasculitis (Henoch-Schönlein purpura) affects various organs, including the skin, gastrointestinal (GI) tract, joints and kidneys. Its clinical course typically consists of two phases: initial appearance of purpura and delayed onset of arthralgia, GI symptoms and haematuria. We report the case of an adult patient with IgA vasculitis of the small bowel, without skin involvement, complicated by cytomegalovirus (CMV) enteritis following prednisolone administration. Single-balloon enteroscopy revealed mucosal oedema, redness, erosions and transverse ulcers of the duodenum and jejunum. Jejunal biopsy specimens showed IgA deposition in the capillary walls. CMV reactivation was confirmed by PCR and immunostaining using jejunal biopsy specimens. This case report strongly suggests that adult patients with IgA vasculitis can present with isolated GI involvement, without characteristic skin purpura. Furthermore, CMV reactivation needs to be considered in patients with IgA vasculitis showing poor response to glucocorticoids., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Tumour necrosis factor-α [TNF-α] inhibitors have revolutionised the management of chronic inflammatory conditions. A number of cutaneous adverse events have been reported with TNF inhibition, including vasculitis. Most reactions are mild and rarely warrant treatment withdrawal. Here we describe a patient with Crohn's disease treated with adalimumab in whom severe multivisceral Henoch-Schönlein purpura developed, including neurological involvement, requiring definitive TNF blocker withdrawal., (Copyright © 2019 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Many pediatric skin conditions can be safely monitored with minimal intervention, but certain skin conditions are emergent and require immediate attention and proper assessment of the neonate, infant, or child. We review the following pediatric dermatology emergencies so that clinicians can detect and accurately diagnose these conditions to avoid delayed treatment and considerable morbidity and mortality if missed: staphylococcal scalded skin syndrome (SSSS), impetigo, eczema herpeticum (EH), Langerhans cell histiocytosis (LCH), infantile hemangioma (IH), and IgA vasculitis.

Objectives: Immunoglobulin-A vasculitis (IgAV) is a systemic small-vessel vasculitis in which renal involvement indicates severity of illness, and chronic kidney disease represents the most serious long-term complication. No treatment at present is specifically recommended for IgAV. Recently, rituximab (RTX) has been shown to be effective in case series of adults with IgAV. However, long term results are lacking. Aim of the study is to evaluate the effectiveness of RTX as induction therapy and maintenance of remission in adults with severe IgAV and aggressive glomerulonephritis., Methods: This study included 12 adult-onset patients, 8 males and 4 females, mean age 45.1 years (range 19-75) with a mean follow-up duration of 33.7 months (range 6-144). All patients had a severe IgAV with biopsy proven crescentic nephritis. RTX was given for the treatment of a refractory disease or because of definite contraindications to standard therapies., Results: Eleven patients (91.7%) achieved a clinical response at 6 months. Ten patients had a complete response (CR) while one had a partial response and was given an additional dose of RTX after 12 months for persistent proteinuria (1gr/24 hrs) despite systemic remission. He achieved a CR 6 months later. One patient was considered unresponsive to RTX and was switched to MMF. Among the 10 patients with CR, 1 needed maintenance doses of RTX every 6 months for iterative relapsing of severe purpura, 1 relapsed after 15 months and received a new induction course showing a CR again. A significant decrease in BVAS (p=0.031) and 24-hour-proteinuria (p=0.043) from RTX initiation through the last follow-up has been detected. One patient, who had a CR with RTX alone died after 6 months for therapy-unrelated cardiovascular cause., Conclusions: RTX proved to be effective and safe for induction and maintenance of long-lasting remission in severe IgAV with aggresive renal involvement. Data also suggest that RTX can be indicated not only for refractory cases, but can be also proposed as a first line therapy.

Background: To explore the changes of inflammatory and oxidative stress responses in Henoch-Schönlein purpura (HSP) children, and further analyzed the therapeutic effects and mechanisms of hemoperfusion (HP) on HSP with severe gastrointestinal (GI) involvement., Methods: There were 200 children with HSP were divided into three groups according to their clinical manifestations: 60 in HSP without GI and renal involvement group, 60 in HSP with GI involvement group, and 80 in HSPN group. The HSP with GI involvement group was subdivided into conventional treatment (n = 30) and HP (n = 30) groups. Thirty children who visited the department of children healthcare for healthy physical examinations from January to December 2017 were set as healthy control group. The IL-6 and TNF-α levels were detected by chemoluminescence method. The MDA, SOD and T-AOC levels were determined by thiobarbituric acid colorimetric method, hydroxylamine method and chemical colorimetry., Results: Compared with healthy group, IL-6, TNF-α and MDA levels in HSP were increased in each group, while SOD and T-AOC were decreased (P = 0.000). IL-6, TNF-α and MDA levels in the HSPN group were the highest, SOD and T-AOC levels were the lowest (P = 0.000). Compared with those before treatment, IL-6, TNF-α and MDA levels in the conventional and HP groups were decreased and SOD and T-AOC levels were increased (P = 0.000). The changes in HP group were more significant than those in conventional group (P < 0.047). Compared with conventional group, glucocorticoid dosage and the occurrence rate of hematuria and/or proteinuria within 3 months were lower in HP group. (P = 0.000, 0.004)., Conclusions: Inflammatory and oxidative stress may be involved in the acute phase of HSP children. The intensity of inflammatory and oxidative stress responses were related to the degree of renal involvement. HP can reduce glucocorticoid dosage and the rate of renal involvement in children with severe HSP with GI involvement. The mechanism may be related to the fact that HP can effectively remove IL-6, TNF-α, MDA in HSP children.

Objective: It is unknown whether epidemiological or clinical characteristics of Henoch-Schönlein purpura (HSP) have changed over time. This study aimed at evaluating the epidemiological and clinical changes of HSP during 3 decades., Methods: We retrospectively analyzed the data of 515 children with HSP (0-15 years of age) between 1987 and 2015. We compared the two HSP patient groups: those admitted from 1987 to 1996 (group A, 238 cases) and those admitted from 2006 to 2015 (group B, 98 cases), apart a decade., Results: In total 515 patients, the mean age was 6.5 ± 3.0 years and the male-to-female ratio was 1.2:1 (278:237). The age distribution showed a peak at age 5 with a bell-shaped distribution pattern. The annual number of cases varied in each year with a trend of reduced cases in the recent decade. There were less cases during the summer season. Purpura, gastrointestinal involvement, joint involvement, and renal involvement were found in 100%, 56%, 38%, and 18% of the patients, respectively. In comparison between the two groups, there were similar findings in mean age, age distribution, and seasonal distribution. However, the hospitalization stay was longer, and the proportion of recurrent cases (14 cases vs. 0 case) and proteinuria (15% vs. 3%) were higher in the group A than in the group B., Conclusions: Long-term epidemiologic features of HSP were similar to those in other countries. Clinical manifestations of HSP showed a trend towards a less severe clinical phenotype over time in Deajeon, Korea. Key Points • It is unknown whether epidemiological and clinical traits of Henoch-Schönlein purpura (HSP) have changed over time. • We reported that clinical manifestations of HSP have changed to milder phenotype through a long-term observation of three decades at a single hospital in Daejeon, South Korea. • Clinical phenotype of infection-related diseases, including HSP, may be changed over time, and the etiology and the reason of clinical changes over time remain to be solved.

A 30-year-old Caucasian woman with no prior medical history presented with pedal oedema, arthralgias and abdominal pain with diarrhoea, following a respiratory infection. She had mild abdominal tenderness along with a purpuric rash on the extremities and was anaemic. Following initial workup for anaemia and rash, her condition deteriorated with renal impairment, respiratory failure and seizures necessitating ventilatory support, dialysis and steroids. Serologies were negative, and skin biopsy showed leucocytoclastic vasculitis without vascular IgA deposition, and renal biopsy showed subendothelial, mesangial deposits of IgA with C3 indicative of Henoch-Schonlein purpura (HSP). She was treated with steroids, haemodialysis and on 6-month follow-up recovered renal function. We present the case to illustrate that HSP, though rare in adults, can present with multiorgan failure, with renal, pulmonary and central nervous system involvement, and the need for early diagnosis and prompt treatment for rapid clinical recovery., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: Henoch-Schönlein purpura (HSP) is the most common vasculitis of children. The aim of this study is to evaluate the demographic and clinic findings of patients with HSP and also to determine predictive factors for assessing the development of gastrointestinal system (GIS) and renal involvement., Methods: This study was performed prospectively among children with HSP who are under 18 years of age and being followed-up in the Pediatric Rheumatology Unit of Health Sciences University Kanuni Sultan Süleyman Training and Research Hospital between January 2016 and January 2018., Results: A total of 265 patients, 137 boys (51.7%) and 128 girls (48.3%), were involved to the study. The mean ± standard deviation of age at the diagnosis was 7.5 ± 3.2. The most common disease onset season was spring (31.7%). The rate of arthritis, GIS involvement, and renal involvement were 54%, 51.3%, and 29.1%, respectively. GIS bleeding was more frequent in males than females (p = 0.007). Boys over 7 years of age had significantly more common GIS bleeding (p = 0.04). Intussusception, relapse, and serious GIS involvement requiring hospitalization and steroid treatment were highly associated with severe renal involvement., Conclusions: We demonstrated that patients suffering intussusception, relapse, and serious GIS involvement or requiring hospitalization and steroid treatment had tendency to present with severe renal involvement. Therefore, these patients should be followed up carefully for not overlooking renal involvement of HSP.

Henoch-Schonlein purpura (HSP) is the most common systemic vasculitis in childhood. There is no consensus about the management for isolated cutaneous manifestations in HSP. We describe a case of HSP presenting with severe skin lesions that did not respond to standard therapy with corticosteroids. The 11-year-old child was treated with intravenous immunoglobulins, which induced rapid and persistent resolution of symptomatology., (© 2018 Wiley Periodicals, Inc.)

Cutaneous and other vasculitides are specific inflammations of the blood vessel wall that can take place in any organ system of the body including the skin. Vasculitis has been traditionally divided according to the size of the vessel involved (small, medium, and large). Vasculitis is more of a reaction pattern rather than a specific disease entity. Therefore, the clinical presentation of vasculitis (most commonly palpable purpura on the lower extremities) dictates a thorough history, review of systems, and a meticulous physical examination. The diagnosis of vasculitis relies also on the histopathological and immunofluorescence studies. Wound care specialist may face with vasculitis-associated ulcers along with a spectrum of other cutaneous presentations associated with vasculitis. The focus of this article is to update the types, etiology, pathogenesis, and management options for cutaneous vasculitis.

Rationale: To explore the curative effect of human umbilical cord-derived mesenchymal stem cell (ucMSC) therapy for patients with liver cirrhosis complicated with immune thrombocytopenia and refractory Henoch-Schonlein purpura (HSP)., Patient Concerns: A 12-year-old boy presented to our hospital with an 11-month history of purpura on the skin of both lower limbs accompanied by thrombocytopenia. The patient had a history of repeated swelling and painful dorsum pedis, followed by skin redness., Diagnosis: Bone marrow slides showed megakaryocyte maturation disorder. Based on the pathology and drug abuse history, he was diagnosed with nodular cirrhosis, secondary allergic purpura, and thrombocytopenia, etiologies related to his drugs and an immune dysfunction., Interventions: ucMSC transplantation was performed, the liver damaging drugs were discontinued, and the appropriate liver immunosuppressive drugs were administered. ucMSCs were injected 8 times/wk in 2 months, with a median cell count of 5.65 × 10/L, ranging from 5.48 to 5.98 × 10/L., Outcomes: As the patient's skin rash resolved, his platelets gradually increased to >150 × 10/L and liver transaminase levels gradually decreased to a normal level. Ultrasonography of the abdomen indicated that the round nodules in the liver decreased in size and that the spleen thickness also decreased., Lessons: This is a unique case of significant HSP with associated thrombocytopenia in a patient with liver cirrhosis. Long-term oral administration of excessive herbal medicine may cause liver damage. We believe that ucMSCs provide a novel approach for the treatment of liver cirrhosis.

Rationale: The presence of purpura is a compulsory criteria for the diagnosis of Henoch-Schönlein purpura (HSP). Typical purpura of HSP is distributed symmetrically over the extensor surfaces of the lower limbs, buttocks, and forearms with the occasional involvement of trunk and face in children. It occurs only involving the bottom of the feet has never been reported., Patient Concerns: A 7-year-old girl was admitted to the hospital with abdominal pain, vomiting, and fever., Diagnoses: Combining clinical manifestations with results of radiologic examinations, acute appendicitis was suspected and a laparotomy was considered. Purpura was found on the bottom of her feet when she was in the operating room and HSP was diagnosed., Interventions: The patient was treated with glucocorticosteroids, antibiotics, cimetidine, and restriction of feeding., Outcomes: The abdominal pain and purpura resolved at discharge and there were no recurrences in the subsequent 3-, 6-, and 12-month follow-ups., Lessons: Careful examination of skin including the bottom of the feet can help to direct the diagnostic workup for children with abdominal pain.

Background: Henoch-Schönlein purpura nephritis (HSPN) is the most common pediatric secondary glomerular disease. This study aimed to investigate the significance of glomerular fibrinogen (Fib) deposition in children with HSPN., Methods: Eighty-two patients with HSPN were enrolled retrospectively at the Children's Hospital of Soochow University from January 2015 to March 2017. Patients were divided into groups according to the presence or absence and intensity of glomerular Fib deposits, and clinical and pathological features were compared among the groups., Results: Glomerular Fib deposition was observed in 64 children (78.05%), including 1 Fib± case (1.22%), 23 Fib+ cases (28.05%), 37 Fib++ cases (45.12%), and 3 Fib+++ cases (3.66%). Significantly different levels of high-sensitivity C-reactive protein (hs-CRP), D-dimer (DD), proportions of CD19 + CD23+ cells and urine microalbumin:creatinine ratios (UMA/Cr) were noted among the different Fib deposition groups (no, mild and severe). Pairwise comparison in multiple groups revealed significantly increased hs-CRP, proportion of CD19 + CD23+ cells and UMA/Cr in the severe deposition group compared with the mild and no deposition groups, and remarkably increased DD levels were noted in the severe and mild deposition groups compared with the no deposition group. The degree of glomerular Fib deposition was positively correlated with the degree of glomerular IgA deposition, and the incidence of glomerular IgG deposition in the severe deposition group was increased compared with the no deposition group., Conclusion: HSPN children with glomerular Fib deposition, especially those with severe Fib deposition, exhibit more severely disordered immunologic function, inflammatory reactions and hypercoagulability; glomerular damage in these patients may also be more severe.

Objective: To study the clinical effect and mechanism of hemoperfusion (HP) in the treatment of children with severe abdominal Henoch-Schönlein purpura (HSP)., Methods: A total of 24 children with severe abdominal HSP were divided into two groups: conventional treatment and HP (n=12 each). Ten healthy children who underwent physical examination were enrolled as the control group. Before and after treatment, chemiluminescence was used to measure the serum levels of interleukin-6 (IL-6) and tumor necrosis factor-&alpha; (TNF-&alpha;); thiobarbituric acid colorimetry was used to measure the plasma level of malondialdehyde (MDA); the hydroxylamine method was used to measure the plasma level of superoxide dismutase (SOD); chemical colorimetry was used to measure the plasma level of total anti-oxidant capability (T-AOC)., Results: Compared with the control group, the conventional treatment and HP groups had significantly higher IL-6, TNF-&alpha;, and MDA levels and significantly lower SOD and T-AOC levels before treatment (P&lt;0.05), but there were no significant differences between the conventional treatment and HP groups (P&gt;0.05). After treatment, the conventional treatment and HP groups had significant reductions in IL-6, TNF-&alpha;, and MDA levels and significant increases in SOD and T-AOC levels (P&lt;0.05). The HP group had significantly greater changes than the conventional treatment group; however, there were still significant differences in these indices between the HP and control groups (P&lt;0.05). Compared with the HP group, the conventional treatment group had a significantly lower percentage of children with disappearance of digestive tract symptoms at 4 days after treatment and significantly longer time to disappearance of rash and digestive tract symptoms (P&lt;0.05). Compared with the conventional treatment group, the HP group had a significantly lower amount of glucocorticoid used during treatment and a significantly lower percentage of children who experienced hematuria and/or proteinuria within 6 months of the disease course (P&lt;0.05). There were no significant differences between the two groups in length of hospital stay and recurrence rates of rash and abdominal pain within 6 months of the disease course., Conclusions: HP can reduce the amount of glucocorticoid used during treatment and the incidence rate of kidney injury in children with severe abdominal HSP, possibly by eliminating IL-6, TNF-&alpha;, and MDA.

Cutaneous vasculitis, inflammatory destruction of blood vessels, can present with a wide range of clinical and pathologic findings across a number of heterogeneous conditions. Although some vasculitides are present in both children and adults, some important differences exist in clinical presentation, etiology, management, and prognosis in childhood vasculitis versus adult vasculitis. Cutaneous vasculitis is rare in children, and most childhood vasculitides, of which Henoch-Schönlein purpura is the most common, histologically are small vessel leukocytoclastic vasculitis. In children, infectious etiologies are more common than in adults. Childhood cutaneous vasculitis is most often self-limited with a good prognosis, and treatment is mainly supportive. © 2017 Elsevier Inc. All rights reserved., (Copyright © 2017. Published by Elsevier Inc.)

Objective: To investigate risk factors related to kidney injury in children with Henoch-Schonlein purpura (HSP) and to study the therapeutic effects of hemoperfusion (HP) on kidney injury in HSP children, providing clinical evidence for early prevention and treatment of HSP., Patients and Methods: Children who suffered from HSP for the first time were selected as study objects and they were followed up for 12 months. Single factor analysis and multi-factor Logistic regression analysis were performed for children's demographic characteristics (age, gender), clinical manifestations (rash duration time, rash recurrence times, digestive tract hemorrhage, abdominal pain, arthralgia, HSP recurrence) and laboratory indexes (peripheral blood WBC, PLT, ESR, CRP, serum IgG, serum IgA, IgM, serum C3, serum C4, TC, TG, HDL, LDL). Meanwhile, participants were divided into treatment group (HP treatment) and control group, and the protective effects of HP on renal function of HSP children were discussed., Results: Single factor analysis indicated age ≥ 6 years, rash recurrence ≥ 3 times, rash duration time ≥ 1 month, digestive tract hemorrhage, peripheral blood PLT, WBC, serum TC and serum LDL levels had statistically significant differences between the two groups. Multi-factor Logistic regression analysis indicated rash recurrence ≥ 3 times, digestive tract hemorrhage, decline of peripheral blood PLT count, and increases of serum TC and LDL were closely related to kidney injury of HSP children. After discharge, kidney injury comparison between treatment group and control group in follow-up had a statistical difference., Conclusions: Rash recurrence ≥ 3 times, digestive tract hemorrhage, decline in peripheral blood PLT count, increases of serum TC and LDL, are risk factors of kidney injury in HSP children. HP can protect renal function of HSP children.

Henoch-Schönlein purpura (HSP) with involvement of the testes is extremely rare. Here, we present a pediatric case of HSP involving testicular ischemic necrosis in an 8-year-old child. Ultrasonography plays an important role not only in the differential diagnosis at onset, but also in the assessment of treatment response and prognosis during treatment and at follow-up. In this case report, we present the sonographic images for the entire course of testicular involvement in HSP and reveal histopathologically the pathogeny as testicular autoimmune vasculitis caused by the deposition of immunoglobulin A-containing immune complexes in the testicular vessels., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: In children with Henoch-Schonlein purpura nephritis (HSPN), the degree of proteinuria has been proven to be not only a sign of kidney damage, but also an accelerator of kidney disease progression. Nephrotic proteinuria at disease onset has been proposed as a predictor of a poor renal outcome. This study aims to assess the clinical and pathological features of HSPN with nephrotic proteinuria in a single center., Methods: One hundred thirty-seven patients with HSPN who visited Shanghai Children's Hospital from January 2009 to December 2013 were retrospectively reviewed. The patients were divided into 2 groups based on the 24-h urinary protein levels: nephrotic proteinuria group (NP group: 24-h urinary protein ≥50 mg/kg) and non-nephrotic proteinuria group (NNP group: 24-h urinary protein <50 mg/kg). In addition, data regarding their sex, age, clinical features, renal pathology, and prognosis were collected., Results: (1) There were 34 boys and 20 girls in the NP group with a mean age of 8.39 ± 2.85 years. The peak age of incidence was 6 to 11 years (72.22%). (2) There were 8 cases (14.81%) with joint symptoms and 9 cases (16.67%) with gastrointestinal symptoms in the NP group. According to the analysis of the laboratory test results, the serum albumin and IgG levels of the NP group were significantly lower than that of the NNP group (35.04 ± 8.45 in the NP group vs. 41.55 ± 4.46 in the NNP group, P < 0.0001; 7.68 ± 3.12 in the NP group vs. 9.53 ± 2.74 in the NNP group, P < 0.001, respectively); their blood urea nitrogen and cystatin C levels increased significantly (P < 0.05). (3) The majority of the pathological changes in the NP group were above the International Study of Kidney Disease in Children (ISKDC) grade III (62.97%). The NP group patients with tubulointerstitial injurie with grade 2 and above (50%) were prioritized. Immune complex deposition in the NP group was dominated by IgA. (4) The prognosis of the NP group was in complete remission (A), and their cases did not develop into end-stage renal disease; their prognosis was also associated with clinical classification (P < 0.01) but was not related to pathologic grading and tubulointerstitial injury (P > 0.05)., Conclusion: The serum albumin and IgG levels of the NP group were significantly lower; however, their blood urea nitrogen and cystatin C levels were higher. The ISKDC grades were mainly above grade III. The prognosis of the NP group was associated with clinical classification and improved after a timely and early treatment.

Background: Although Henoch-Schonlein purpura (HSP) is the most common form of systemic vasculitis in children, the long term effect of HSP on endothelial function is still not clear. The aim of our study was to evaluate the long term effect of HSP on endothelial function in children and adolescents., Methods: This research was an observational prospective study. The study group comprised of 19 children diagnosed with HSP. The minimum interval between the diagnosis with HSP and endothelial testing was 5 months. Endothelial function evaluation was assessed by a noninvasive technology named peripheral arterial tonometry, using an EndoPAT™ device. This method measures blood flow in the limb, in response to arterial occlusion, and calculates a Reactive Hyperemic Index (RHI) as an index of endothelial function. RHI values of the study group were compared to those of a known control group., Results: Nineteen children and adolescents with HSP underwent endothelial function studies. Endothelial function was compared to that of a known control group comprising of 23 healthy children and adolescents. The two groups had similar characteristics, including age, male to female ratio, height, weight and BMI. Mean RHI was 1.81 in the study group, and 1.87 in the control group (p = 0.18). Linear regression of the study group, showed a positive correlation between the time interval from HSP diagnosis to participation in the study, and between the RHI value (r = 0.542, p = 0.016). RHI levels were significantly higher in patients who had endothelial function measured more than 6 years since the diagnosis of HSP compared with those patients with less than 6 years follow up (1.98 + 0.74 vs. 1.38 ± 0.43 P = 0.037)., Conclusions: These results suggest that HSP causes short term endothelial dysfunction that improves with time.

Purpuric lesions appear in acral distribution in a variety of conditions and often provide clues to the clinical diagnosis. Purpuric means "hemorrhagic"-that is, the lesions do not blanch from pressure. This review focuses on dermatoses that produce hemorrhagic lesions in acral distribution from the large groups of the vasculitic diseases and their mimics. Cutaneous small vessel vasculitis is confined to the skin, involves mainly postcapillary venules, and has the hallmark manifestation of palpable purpura. Henoch-Schönlein purpura is an immune complex-mediated systemic vasculitis of the small vessels with manifestations from the skin, joints, kidneys, and gastrointestinal system. Only cases where the immune complexes contain immunoglobulin A type are classified as Henoch-Schönlein purpura. Cryoglobulinemic vasculitis is induced by the deposition of cold-precipitated immune complexes in the small vessels. Urticarial vasculitis comprises a spectrum of conditions with the characteristic course of chronic urticaria, with wheals that persist longer than 24 hours, leave hyperpigmentation, and have leukocytoclastic vasculitis on histologic examination. Polyarteritis nodosa is a rare multisystem, segmental necrotizing vasculitis of mainly the medium-sized vessels. Pigmented purpuric dermatoses are chronic benign dermatoses characterized by petechiae, purpura, and increased skin pigmentation. The hallmark of pigmented purpuric dermatoses is their orange-brown, speckled, cayenne pepper-like discoloration., (Copyright © 2016 Elsevier Inc. All rights reserved.)

We report a case of recurrent Henoch-Schönlein purpura nephritis (HSPN) treated successfully with a tonsillectomy and steroid pulse therapy in a kidney transplant patient. A 29-year-old woman was admitted to our hospital for an episode biopsy; she had a serum creatinine (S-Cr) of 1.0 mg/dL and 1.34 g/day proteinuria 26 months after kidney transplantation. Histological examination revealed increased amounts of mesangial matrix and mesangial hypercellularity with IgA deposition. Of note, one glomerulus showed focal endocapillary proliferation and tuft necrosis. We diagnosed active recurrent HSPN. Considering both the histological findings and refractory clinical course of the native kidney, she was treated for 3 consecutive days with steroid pulse therapy and a tonsillectomy. The patient's proteinuria decreased gradually to less than 150 mg/day 6 months later. A second biopsy 6 years after kidney transplantation showed an excellent response to treatment and revealed a marked reduction in both the mesangial matrix and mesangial hypercellularity, with trace IgA deposition. We conclude that a tonsillectomy and steroid pulse therapy appeared to be useful in this patient with active recurrent HSPN. This paper is the first to report a tonsillectomy and steroid pulse therapy as a therapeutic option for active recurrent HSPN. Further studies are needed to elucidate the efficacy and mechanisms of tonsillectomy with recurrent HSPN in kidney transplant patients., (© 2016 Asian Pacific Society of Nephrology.)

Henoch-Schonlein purpura (HSP) may present in a variety of ways, most commonly with joint pain or the distinctive palpable purpura. Genitourinary manifestations of HSP are less common and may precede the classic signs and symptoms of HSP, making the diagnosis difficult. We report a case of a 19-month-old boy with penile and scrotal erythema and swelling at presentation who was later diagnosed with HSP.

Objective: To investigate the changes and clinical significance of biomarker fecal bile acids (BA) in children with Henoch-Schönlein purpura (HSP)., Methods: Nineteen children with HSP and twenty-seven healthy children were enrolled in this study. The stool samples were obtained at the acute and remission phases. Fecal BA levels were measured by high performance liquid chromatography mass spectrometry (HPLC-MS)., Results: The fecal cholic acid level in the HSP remission group was significantly higher than in the HSP acute group and the healthy control group (P<0.016). The fecal chenodeoxycholic acid level in the HSP remission group was significantly higher than in the healthy control group (P<0.016). The levels of fecal secondary colonic bile acids, deoxycholic acid and lithocholic acid, in the HSP acute and remission groups were significantly lower than in the healthy control group(P<0.05, P<0.016 respectively). No significant differences were found in the levels of fecal urosodeoxycholic acid among the three groups (P>0.05)., Conclusions: Fecal secondary colonic bile acids, deoxycholic acid and lithocholic acid, are in decrease in children with HSP at the acute stage, which may be involved in the pathogenesis and treatment outcomes of HSP.