Involvement In Henoch-Schönlein Purpura Research Articles

Articular involvement in Henoch-Schönlein Purpura: A review of literature.

Henoch-Schönlein purpura (HSP), the most typical kind of pediatric vasculitis, can also affect adults. Over the last 10 years, research has been increasing on improvements in HSP diagnosis, physiopathology, symptoms, and therapy. Joint involvement is highly frequent in this condition; however, it typically undergoes spontaneous resolution and does not lead to long-term complications. To provide a deeper understanding of the constituting pathogenic mechanisms and clinical presentation of articular involvement, focusing on the effect of neutrophil activation on systemic small vessels. This literature review utilized a systematic search of academic databases, employing specific keywords to select recent peer-reviewed articles and scholarly sources on the topic. The manifestations of joint involvement in HSP can vary in severity and frequency. Non-steroidal anti-inflammatory medications or acetaminophen are considered the first-line treatment for joint pain; however, corticosteroids may help achieve quick remission. In cases where standard treatment fails or manifestations persist, immunosuppressive drugs like rituximab, methotrexate, cyclophosphamide, or azathioprine have been used. While it tends to resolve without lasting joint damage, accurate diagnosis and appropriate management are crucial to ensure optimal patient outcomes.

An Assessment on the Effectiveness of the Immature Granulocyte Percentage in Predicting Internal Organ Involvement Among Children With Henoch-Schönlein Purpura.

Henoch-Schönlein purpura (HSP) is the most common childhood systemic vasculitis. The present study aims to investigate the effectiveness of the immature granulocyte (IG) percentage as a new marker for predicting internal organ involvement in HSP. This study included 75 patients below 18 years old who were diagnosed with HSP. The mean age was 7.48±2.77 years. The male/female ratio was 1.14. The findings showed that 35 (46.7%) of the patients had an internal organ involvement. The mean IG percentage was 0.88±0.68 among the patient group with HSP internal organ involvement, while it was 0.31±0.15 in the group without internal organ involvement, and a significant difference was determined between the 2 groups (P=0.000). The findings showed that the patients with renal involvement had the highest mean IG percentage (IG; 1.00±0.21). When the cutoff value for the IG percentage was specified as 0.45 to predict internal organ involvement, the sensitivity was 77.1%, and the specificity was 85%. In this study, the findings showed that IG percentage increased among patients with internal organ involvement in HSP and that its sensitivity, specificity, and predictive values were higher in predicting internal organ involvement compared with other markers.

Risk Factors Associated With Renal Involvement in Childhood Henoch-schonlein Purpura

Context: Henoch-Schonlein purpura (HSP) is a significant cause of chronic renal disease in children. This review determines some risk factors associated with renal involvement in childhood HSP. Evidence Acquisition: Electronic databases, including Google Scholar, PubMed, and Scopus were searched using the following keywords: “children”, “Henoch-Schonlein”, “risk factor”, “renal involvement”, and “IgA vasculitis”. This review was designed to identify the relevant electronic studies published in the English language from December 1998 to August 2018. Results: This review revealed that clinically older age at presentation, persistent rash, atypical rash, rash on unusual location, and gastrointestinal bleeding were significant risk factors for renal involvement. In contrast, joint involvement was not associated with renal involvement. Among biochemical markers, high red blood cell distribution width is a risk marker of renal involvement in HSP. In contrast, peripheral blood immunoglobulin A, antinuclear antibody, anti-streptolysin O titer, erythrocyte sedimentation rate, and C-reactive protein were not associated with renal involvement. In several studies, leukocytosis, thrombocytosis, or thrombocytopenia have been mentioned as predictors for renal involvement. Still, other studies showed the white blood cell count or platelet count are not risk factors. The effect of corticosteroids as a predictive factor of renal involvement in HSP is challenging and controversial. Furthermore, their impact was dose-dependent. Conclusions: Demographic factors, clinical features, and some abnormal laboratory findings are significant predictive factors for renal involvement in HSP.

Henoch-Schönlein purpura in children: Use of corticosteroids for prevention and treatment of renal disease.

A few patients have previously presented to my clinic with palpable purpura, joint inflammation, and severe abdominal pain characteristic of Henoch-Schönlein purpura (HSP). Considering that renal injury is the primary long-term complication of HSP, are corticosteroids effective in preventing or treating renal disease in children with HSP? Henoch-Schönlein purpura is self-limiting in 94% of children, but permanent renal injury is reported in one-fifth of children with nephritic or nephrotic features. Corticosteroids have been considered as candidates for preventing and treating renal involvement in HSP. There is a moderate level of evidence to suggest corticosteroids are not effective in preventing renal involvement in HSP. However, based on low-level evidence and similarities with primary immunoglobulin A nephropathy, experts recommend corticosteroids in treating renal involvement in HSP to prevent long-term kidney injury. Dose and duration of therapy should be carefully considered in consultation with a pediatric nephrologist.

SAT-401 RENAL PRONOSIS IN ADULTS IgA VASCULITIS

IgA vasculitis or Henoch-Schönlein purpura (HSP) is an acute immunoglobulin A (IgA)–mediated disorder characterized by a generalized vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and rarely, the lungs and the central nervous system (CNS). It is the most frequent vasculitis in childhood. The disease is much less common in adults, who often have a more severe course of the disease. Renal involvement in HSP affects the prognosis of the disease in adult patients. The aim of our study was to examine the clinical characteristics of this renal involvement and to identify factors influencing renal prognosis. Retrospective study including all patients with histologically confirmed HSP (EULAR classification criteria) with renal involvement treated in our department from 1990 to 2019. We analyzed renal survival and identified the factors influencing renal prognosis Twenty-eight patients were included (sex ratio M/F = 2.5), their average age at diagnosis of HSP was 38.14 years. In 22 patients HSP and the kidney damage were discovered at the same time. The other patients were followed for HSP and kidney damage occurred after an average follow-up time of 20.16 months. An Infectious disease was identified as a contributing factor in 6 patients. Smoking was noted in 10 cases. Fourteen patients had a normal renal function. Proteinuria was positive in 24 cases and 9 patients met the criteria for nephrotic syndrome. Acute nephritic syndrome was present in 4 patients. Histological analysis of the skin biopsy was performed in 15 cases and showed leukocytoclastic vasculitis in 8 patients with Ig A deposits in 4 patients. The most common histological lesion was segmental and focal glomerulonephritis found in 11 cases. The most important renal prognostic factors identified were gastrointestinal involvement including vomiting (p=0.022) and initial acute renal failure (p=0.0004). Our study shows that renal involvement during Ig A vasculitis can be relatively severe with poor renal prognosis. The identification of prognostic factors may be useful to guide prospective therapeutic studies.

Risk Factors for Severe Gastrointestinal Involvement in Childhood HenochSchoenlein Purpura

Background: Henoch–Schonlein Purpura (HSP) is an IgAmediated vasculitis of children. It is often regarded as a benign disease. Nevertheless, some patients may manifest severe renal or gastrointestinal (GI) involvement. We sought to identify the risk factors for severe GI involvement in HSP. Methods and findings: We performed a retrospective study of all children who had Henoch-Schonlein purpura with gastrointestinal (GI) involvement admitted during a 10 years period to a single pediatric department. The GI involvement was categorized as severe in children who isease: intense abdominal pain with a score > 3 on a 0-10 self- report scale, or scopresented at least one of the following items on initial presentation and/or on the whole course of the dre <10 on the Amiel-Tison scale for children younger than 3 years, the need for enteral tube feeding or corticosteroid administration, upper or lower gastrointestinal bleeding, intussusception or parietal hematoma on abdominal ultrasound data and if a surgical intervention was needed (group A). The group B included all children diagnosed with HSP without GI involvement or presented with mild abdominal pain (group B). We compared the two groups, using initially Chi square (χ2) and student “t” tests, and thereafter, we employed logistic stepwise regression analysis. Conclusion: Independent risk factors for severe digestive involvement were hyperleukocytosis (p=0.004; Adj OR: 4.5 [1.6–13]), neutrophilia (p<10-3; Adj OR: 8.7 [3.5-26.2]) and renal involvement. The risk factors for severe gastrointestinal involvement in SHP are close to those of severe renal impairment. The identification of these risk factors could guide the indication of hospitalization and the therapeutic modalities.

Severe Mitral Regurgitation in a Child With Henoch-Schönlein Purpura and Pulmonary Hemorrhage.

Introduction: Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. The classic triad of HSP consists of nonthrombocytopenic purpura, arthritis/arthralgia, and gastrointestinal complaints. Pulmonary hemorrhage and cardiac involvement are rare complications of HSP. Case Report: We report the case of a 10-year-old girl with HSP complicated by both severe mitral regurgitation and pulmonary hemorrhage. Discussion: HSP is typically a self-limited illness with an excellent prognosis in children. Pulmonary hemorrhage is a rare complication that increases morbidity and mortality; it generally indicates the presence of severe vasculitis. Cardiac involvement in HSP is extremely rare and associated with a poor prognosis. Conclusion: Cardiac involvement in HSP may be more common than believed. Because of the increased morbidity and mortality associated with HSP complicated by pulmonary hemorrhage and cardiac involvement, it is important for clinicians to be aware of these potential complications.

Risk Factors Associated with Renal Involvement in Childhood Henoch-Schönlein Purpura: A Meta-Analysis.

Background and objectiveHenoch-Schönlein purpura (HSP) is an important cause of chronic kidney disease in children. This meta-analysis identified risk factors associated with renal involvement in childhood HSP.MethodsPubMed, Embase, and Web of Science were searched. The quality of all eligible studies was assessed using the Newcastle-Ottawa scale criteria. An analysis of possible risk factors was conducted to report the odds ratio (OR) and weighted mean difference (WMD).ResultsThirteen studies (2398 children) revealed 20 possible and 13 significant risk factors associated with renal involvement in HSP, with the following meta-analysis estimates of OR and WMD, with 95% confidence intervals: older age (0.90, 0.61–1.19); age > 10 y (3.13, 1.39–7.07); male gender (1.36, 1.07–1.74); abdominal pain (1.94,1.24–3.04); gastrointestinal bleeding (1.86, 1.30–2.65); severe bowel angina (3.38, 1.17–9.80); persistent purpura (4.02, 1.22–13.25); relapse (4.70, 2.42–9.14); WBC > 15 × 109/L (2.42, 1.39–4.22); platelets > 500 × 109/L (2.98, 1.22–7.25); elevated antistreptolysin O (ASO) (2.17, 1.29–3.64); and decreased complement component 3 (C3) (3.13, 1.62–6.05). Factors not significantly associated with renal involvement were: blood pressure; orchitis; elevated C-reactive protein; elevated erythrocyte sedimentation rate (ESR); and elevated serum IgA/IgE or IgG. Arthritis/arthralgia may be a risk factor according to the criteria of the American College of Rheumatology (1.41, 1.01–1.96).ConclusionThe following are associated with renal involvement in pediatric HSP: male gender; > 10 y old; severe gastrointestinal symptoms (abdominal pain, gastrointestinal bleeding, and severe bowel angina); arthritis/arthralgia; persistent purpura or relapse; WBC > 15 × 109/L; platelets > 500 × 109/L; elevated ASO; and low C3. Relevant clinical interventions for these risk factors may exert positive effects on the prevention of kidney disease during the early stages of HSP. However, the results should be interpreted cautiously due to the limitations of the studies.

Intravenous immunoglobulins for severe gastrointestinal involvement in pediatric Henoch-Schönlein purpura: A French retrospective study

Severe gastrointestinal involvement of Henoch-Schönlein purpura (HSP) is rare but potentially life-threatening. Management of severe gastrointestinal involvement in HSP is not codified. Symptomatic care and steroids are a first-line therapy. Nonsteroidal immunomodulatory therapies have been anecdotally used to treat steroid-refractory forms. The aim of this study was to describe the outcome of patients with severe gastrointestinal involvement of HSP who required nonsteroidal immunomodulatory therapy. A French retrospective case series study was conducted. Pediatric consultants at 31French academic pediatric centers were contacted. Patients were identified from memory or via an informatics diagnosis-related code system. Clinical, paraclinical, and therapeutic data were collected. Twenty-nine responding centers provided nine cases, one of which was excluded. Five boys and three girls, aged 3-15years (median: 5.5years) from seven centers were included. Severe gastrointestinal involvement of HSP mainly included intense pain, digestive bleeding, and protein-losing enteropathy. All children had been treated with steroids at first line and intravenous immunoglobulins (IVIg) at second line. Six out of eight showed a complete response to IVIg within 7days and two out of eight had a partial response. Two out of eight relapsed with less severe gastrointestinal involvement requiring a second dose of IVIg and they did not relapse thereafter. Tolerance was good, but two out of eight developed high proteinuria on the day following IVIg infusion. Although a possible link with a flare-up of proteinuria needs to be addressed, IVIg appears to be a good candidate for treatment of severe gastrointestinal involvement of HSP.

Atopic Dermatitis and Association of Risk for Henoch-Schönlein Purpura (IgA Vasculitis) and Renal Involvement Among Children: Results From a Population-Based Cohort Study in Taiwan.

Elevation of Th2 cytokine-driven inflammatory mediators has been reported in acute stage of Henoch–Schönlein purpura (HSP). However, the temporal interaction between Th2-mediated allergic diseases and HSP with renal involvement remains unknown. Herein, we conducted a population-based cohort analysis to investigate the risk of HSP and renal involvement in children with atopic dermatitis (AD) as 1 of the first steps in the atopic march.From 2000 to 2007, 95,208 children with newly diagnosed AD and 190,416 randomly selected non-AD controls were included in the study. By the end of 2008, incidences of HSP in both cohorts and the AD cohort to non-AD cohort hazard ratios (HRs) and confidence intervals (CIs) were measured. Comparison of renal involvement in HSP between children with and without AD was analyzed.The incidence of HSP during the study period was 1.75-fold greater (95% CI: 1.27–2.42) in the AD cohort than in the non-AD cohort (14.2 vs 8.11 per 100,000 person-years). The AD to non-AD HR of HSP was greater for girls (1.92, 95% CI: 1.18–3.13), children older than 6 years (2.54, 95% CI: 1.15–5.59), and those living in less urbanized area (2.74, 95% CI: 1.10–6.82). Concurrent allergic rhinitis or asthma did not increase the HR of HSP further. The HR for HSP in AD children increased from 0.67 (95% CI: 0.41–1.11) for those with ≤2 AD-related visits to 9.77 (95% CI: 6.44–14.8) for those with >4 visits (P < 0.0001, by the trend test). The risk of developing HSP in the AD cohort was highest within first year after AD diagnosis (HR: 3.99; 95% CI: 1.61–9.89). AD cohort with HSP had higher occurrence rate of renal involvement, particular hematuria, than non-AD cohort with HSP.AD children had a greater risk of developing HSP and HSP with renal involvement. Further research is needed to clarify the role of allergy in the pathogenesis of HSP and renal involvement.

Obesity increases the risk of renal involvement in children with Henoch-Schönlein purpura.

The main aim of this study was to evaluate the relationship between obesity and renal involvement in children with Henoch-Schönlein purpura (HSP). A retrospective study of 141 pediatric patients with HSP was conducted in our hospital. The clinical data of all patients were collected from the electronic medical record management system from January 2010 to June 2014. The possible risk factors of renal involvement, especially obesity, were analyzed using univariate and multivariate analyses. Renal involvement occurred in 45/141 of the patients. A univariate analysis showed that an age more than 7 years at onset, persistent purpura, obesity, time from symptoms onset to diagnosis more than 14 days, and decreased C3 all increased the risk of renal involvement in HSP. The forward stepwise logistic regression analysis indicated obesity (odds ratio (OR) 4.43, 95 % confidence interval (CI) 1.896 to 10.358), age more than 7 years at onset (OR 2.81, 95 % CI 1.142 to 6.907), and persistent purpura (OR 2.57, 95 % CI 1.119 to 5.909) were independent risk factors for renal involvement. Our results show that obesity can increase the hazard of renal involvement in children with HSP and reconfirm that older age at onset and persistent purpura are the independent risk factors for renal involvement. • There have been some reports that obesity was associated with the development of renal injury. • It is not clear whether obesity can increase the risk of renal involvement in children with HSP. What is New: • The main finding of this study is that obesity can increase the hazard of renal involvement in children with HSP.

Plasma exchange therapy for severe gastrointestinal involvement of Henoch Schonlein purpura in children

The aim of this report is to evaluate the plasma exchange as a choice for the management of life-threating gastrointestinal system (GIS) involvement in Henoch-Schönlein purpura (HSP) when refractory to conventional therapies. We retrospectively reviewed the medical records of HSP patients who had plasma exchange therapy due to massive GIS involvement. We reported age, gender, initial HSP presentation, etiological or triggering factors and disease course. Treatment modalities, side effects and their outcomes were noted. We reported 7 cases of childhood HSP with severe gastrointestinal involvement refractory to common immunosuppression with systemic steroid and cytotoxic therapy. All patients gave inadequate response to pulse methyl prednisolone or oral prednisolone therapy with ongoing GIS bleeding and severe abdominal pain. Therefore, pulse cyclophosphamide was added to the treatment. Two patients received additional intravenous immunoglobulin (IVIG) therapy. Gastrointestinal manifestations continued and plasma exchange was performed. All patients improved after plasma exchange treatment. Treatment of GI involvement in HSP with plasma exchange has been mainly based on case reports. According to our data, we propose that, plasma exchange may be a safe and efficient management choice in paediatric HSP patients with massive GIS involvement that are refractory to other therapies.

Asia Pacific, and beyond

Wish you have a very Happy New Year! With your support, Asia Pacific allergy (AP Allergy) has been very successful for more than three years since the launching ceremony on November 6, 2010 and its inaugural issue release on April 30, 2011. AP Allergy has published forty nine review articles from Australia (7), Finland (1), India (3), Indonesia (2), Italia (1), Japan (5), Korea (13), Malaysia (1), Singapore (7), Slovak (1), Sri Lanka (1), Sweden (1), Taiwan (3), Thailand (2), and the United Kingdom (1). Total of forty five original articles from Australia (4), China (1), Indonesia (1), Iran (1), Japan (8), Korea (11), Mexico (2), Mongolia (1), New Zealand (1), Philippines (5), Singapore (4), Sri Lanka (1), Thailand (4), and Turkey (1) and 9 case reports from Indonesia (1), Korea (7), and United Kingdom (1). In addition to the case reports, 4 letters have been published so far. Recently the articles of AP Allergy have been frequently accessed, downloaded, and cited. Cough is a common symptom of patients who visit a clinic. Chronic cough lasts more than 8 weeks and the most common causes are closely related to allergic diseases such as upper airway cough syndrome due to rhinosinusitis, asthma, nonasthmatic eosinophilic bronchitis, and gastroesophageal disease. This issue of AP Allergy features important aspect of cough hypersensitivity in chronic cough [1]. Genetic regulation is a key phenomenon in understanding of life science. The interaction of environment and genetic regulation, in other words, epigenetic regulation in allergic diseases is reviewed in this issue [2]. Airway remodeling, a critical component in the management of asthma, and its relationship with mechanical force was reviewed as well [3]. Drug hypersensitivity can be related with some of viral infections such as human immunodeficiency virus (HIV) infection. As a matter of fact, antiviral drugs can result in drug hypersensitivity. Drug hypersensitivity in HIV-infected patients is also featured in this issue [4]. Readers of this journal will find original articles on the prevalence of asthma and allergic rhinitis among adult population in Ulaanbaatar, Mongolia [5], the pH of antiseptic cleansers for atopic dermatitis patients [6], the effects of wheezing in early childhood in the development of allergic rhinitis in later years [7], and the age of onset as a risk factor of renal involvement in Henoch-Schonlein purpura [8]. They may also have a new idea on the frequently asked questions in allergy practice from a study in Singapore [9]. The issue contains an educational case report of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis that were rapidly developed after ingestion of Acetaminophen [10]. Asia Pacific Association of Allergy, Asthma, and Clinical Immunology (APAAACI) had a successful congress (APCAACI 2013) in Taipei, Taiwan in last November. During the congress, Dr. Connie Kateralis who is the immediate past president of APAAACI has been elected as the new Editor-in-Chief in the editorial board meeting. As the founding editors, we thank you very much for your strong support. We wish Asia Pacific Allergy a brilliant future with the new Editor-in-Chief: Asia Pacific, and beyond.

A case of acute cholecystitis in Henoch–Schönlein purpura

A case of acute cholecystitis in Henoch–Schönlein purpura

AB0446 Gastrointestinal bleeding in adult patients with henoch-schönlein purpura

BackgroundAdult Henoch-Schönlein 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...

Familial Cases of Henoch-Schönlein Purpura in Taiwanese Aborigines

Henoch-Schönlein purpura (HSP) is a disorder whose cause and pathogenesis is unknown; some familial cases of this disease have been reported. The clinical heterogeneity in HSP may be conferred by a number of genetic loci, including the major histocompatibility complex. The racial and genetic factors responsible for the occurrence of the familial cases of HSP in Taiwan are unclear. The purpose of this study was to examine the racial and genetic factors in familial HSP cases in Taiwan. We retrospectively collected the HSP cases in our hospital during 2006 through 2010 and observed that familial HSP cases were only in Taroko Aborigines. Six cases of HSP in 3 Taroko families were found, and their human leukocyte antigens (HLA) were studied in the tissue typing laboratory of our hospital, to determine the possible association with familial HSP cases in Taiwanese Aborigines. Our results suggest an increased frequency of familial HSP cases with HLA-A24 in Taiwanese Taroko Aborigines. We concluded that racial and genetic predisposition was the possible cause for the familial occurrence of and renal involvement in HSP in Taiwanese Aborigines.

Cut‐off values for serum matrix metalloproteinase‐9: Is there a threshold to predict renal involvement for Henoch–Schonlein purpura in children?

To clarify whether the level of matrix metalloproteinase-9 (MMP-9), tissue inhibitor matrix metalloproteinase-1 (TIMP-1) or the ratio of MMP-9/TIMP-1 was associated with the renal involvement in Henoch-Schonlein purpura (HSP); and to explore whether there existed early diagnostic measure for HSP nephritis (HSPN). Sixty-six patients with HSPN, 68 patients with HSP and 60 healthy children (control group) were enrolled into our study. Serum and urine samples before treatment were collected for detection. Compared with the HSP group and control group, serum MMP-9, TIMP-1 and ratio of MMP-9/TIMP-1 in the HSPN group were significantly higher (P<0.05 and P<0.01, respectively). Urine MMP-9, TIMP-1 and ratio of MMP-9/TIMP-1 in the HSPN group were obviously higher than those of the control group (P<0.05) and the HSP group (P<0.05). Receiver-operator curve (ROC) analysis was performed to obtain the area under the curve (AUC) and the AUC and its 95% confidence interval (CI) of serum MMP-9 were 0.97 and 0.95-0.99, respectively. The optimal cut-off point (sensitivity; specificity) of serum MMP-9 for diagnosing HSPN was 179.79 mg/L (0.96; 0.88). Levels of MMP-9, TIMP-1 and ratio of MMP-9/TIMP-1 in serum and urine were remarkably high in the patients with HSPN, but the serum MMP-9 was more sensitive. Serum MMP-9 may be associated with the occurrence and development of renal involvement in HSPN and become an important indicator for early diagnosis of HSPN.

Glucocorticoid-Responsive Hypertension in Henoch-Schönlein Purpura

Henoch-Schonlein purpura (HSP) is a vasculitic syndrome which commonly involves small vessels of the skin, joints, intestines, and kidney. Renal involvement is the greatest cause of long-term morbidity and mortality and is identified by proteinuria and/or hematuria. Hypertension is an uncommon complication of renal involvement in HSP. Its occurrence is almost universally associated with other evidence of nephritis. Hypertension resistant to b-adrenergic blockade, calcium channel blocking agents, and vasodilators may occur early in the course of HSP in the absence of other evidence of renal disease. We report two children with Henoch-Schonlein purpura who had significant hypertension resistant to treatment with B-adrenergic blocking agents and vasodilators, one of whom had no other evidence of renal disease. In one child, the hypertension responded to therapy with captopril, suggesting a reninangiotensin mediated mechanism for the hypertension. In both children, initiation of corticosteroid therapy resulted in prompt resolution of hypertension, permitting the discontinuation of antihypertensive medications.

Management of haemorrhagic bullous lesions in Henoch–Schonlein purpura: Is there any consensus?

Henoch–Schonlein purpura (HSP) is the most common form of vasculitis affecting children. The cutaneous manifestations classically present as urticarial wheals, erythematous maculopapules, petechiae, purpura or oedema, which characteristically involve the lower extremities and buttocks. Haemorrhagic bullous lesions are a recognized but rare occurrence with HSP in children. We report a 6-year-old boy with HSP who developed extensive haemorrhagic bullae requiring dermatological referral and treatment. Scrutiny of our management and available literature reveals a lack of consensus in the management of extensive cutaneous involvement in HSP.

Letters to the Editor

2 May 2008 Dear Editor, PLASMA FACTOR XIII LEVELS IN HSP PANCREATITIS: RESPONSE TO SHIN ET AL. Shin et al. comment on our case report entitled ‘Acute pancreatitis: a rare presenting feature of Henoch Schonlein purpura’. They remind that plasma factor XIII is useful for the early diagnosis of Henoch-Schönlein purpura (HSP). Also, they proposed that this factor might have been decreased at pancreatitis preceeding HSP. Although, plasma factor XIII level is a well-known early diagnostic marker and commonly used in estimating the prognosis, it has not been reported in HSP pancreatitis previously. We also agree that plasma factor XIII levels can be evaluated to elucidate incidence of pancreatic involvement in HSP. It would be better for the readers if the authors would like to include the factor XIII levels in their two cases of HSP pancreatitis during their 12-year experience.