A Case Report on an Adult Presentation of Henoch-Schönlein Purpura.

Henoch-Schönlein purpura (HSP) is an immunoglobulin A (IgA)-mediated multisystem vasculitis commonly affecting children under 10 years of age. Although diagnostic criteria exist, making a diagnosis is often difficult as this condition can present atypically in adults. We discuss a 22-year-old female with a delayed diagnosis of HSP, resulting in significant anxiety and distress. Our patient's symptoms improved with analgesia and corticosteroids, which were initiated upon diagnosis and she experienced two mild, self-limiting relapses over two years following symptom resolution. Our case illustrates that an integrated multidisciplinary approach is needed to effectively diagnose, safely manage and monitor patients presenting with HSP. Although self-limiting in nature, HSP has the potential to manifest into life-threatening conditions such as end-stage renal failure, which stresses the importance of early diagnosis and management.

Clinical significance of serum gastrin, C-reactive protein and D-dimer in Henoch-Schönlein purpura children with abdominal symptoms / 中华实用儿科临床杂志

Objective@#To investigate the clinical significance of serum gastrin-17, C-reactive protein (CRP) and D-dimer (D-D) levels in early diagnosis and severity evaluation of Henoch-Schölein purpura (HSP) children with abdominal symptoms.@*Methods@#Retrospective analysis was performed in 120 children with initial HSP admitted to the Department of Pediatrics, the First Affiliated Hospital of Anhui Medical University from December 2016 to December 2017, among them, there were 70 cases with abdominal symptoms, and 50 patients without abdominal symptoms.And 20 healthy children who underwent health consultation at the First Affiliated Hospital of Anhui Medical University were selected as a healthy control group.Serum gastrin-17, CRP and D-D levels in acute phase in the HSP children were detected, and the correlation between these parameters and purpura symptom scores was analyzed.Together with gastroscope, the severity of HSP with abdominal symptoms was evaluated.@*Results@#(1) Serum gastrin-17 level in HSP children with abdominal symptoms were obviously lower than that of the other of type HSP group and the healthy control group [(3.12±1.64) pmol/L vs.(6.85±1.28) pmol/L and (7.15±1.03) pmol/L], and the differences were statistically significant (all P<0.001); the levels were decreased most significantly in HSP children with early gastrointestinal symptoms [(1.77±0.50) pmol/L vs.(4.01±1.51) pmol/L], and the difference was statistically significant (P<0.001); the pathological changes under gastroscope were obvious in HSP children with early gastrointestinal symptoms (χ2=8.095 2, P<0.05). (2) Serum CRP and D-D levels in the HSP children with abdo-minal symptoms were striking higher than those in the healthy control group [(18.39±4.48) mg/L vs.(3.95±1.65) mg/L; (2.53±1.17) mg/L vs.(0.59±0.41) mg/L], and the differences were statistically significant (all P<0.001), which were both increased most significantly in the HSP children with early gastrointestinal symptoms [(19.98±5.45) mg/L vs.(17.33±3.37) mg/L; (3.48±0.96) mg/L vs.(1.89±0.80) mg/L], and the diffe-rences were statistically significant (all P<0.05). (3) Serum gastrin-17 level was negatively correlated with purpura symptom scores (r=-0.907, P<0.01); serum CRP and D-D levels were both positively correlated with purpura symptom scores (r=0.974, 0.928, all P<0.01).@*Conclusions@#Serum gastrin-17, CRP and D-D levels in acute phase can be used as serological markers for early diagnosis of HSP children with abdominal symptoms, especially in HSP children with early gastrointestinal symptoms.

Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database.

BACKGROUND: The recurrence rate of Henoch-Schönlein purpura (HSP) is 2.7%-30%, with varied average intervals between the first and second episodes. Few studies have explored the incidence and risk factors for recurrent HSP. METHODS: We used a 16-year nationwide database to analyze the incidence of recurrent HSP. Patients with HSP were identified, and risk factors for recurrent HSP were explored. Kaplan-Meier and Cox regression model analyses were performed, and covariates were adjusted in the multivariate model. RESULTS: From January 1, 1997 to December 31, 2012, among 2,886,836 individuals in the National Health Insurance Research Database, 1002 HSP patients aged < 18 years were identified. Among them, 164 had ≥2 HSP episodes (recurrence rate, 16.4%; incidence of recurrent HSP, 7.05 per 100 person-years); 83.6% patients with one HSP episode remained free of secondary HSP. The average time intervals between the first and second and second and third HSP episodes were 9.2 and 6.4 months, respectively. After adjusting for demographic parameters, comorbidities, and socioeconomic status, recurrent HSP was found to occur more frequently in patients who had renal involvement (adjusted hazard ratio, 2.41; 95% confidence interval [CI], 1.64-3.54; p < 0.001), were receiving steroid therapy for > 10 days (adjusted hazard ratio, 8.13; 95%CI, 2.51-26.36; p < 0.001), and had allergic rhinitis (adjusted hazard ratio, 1.63; 95%CI, 1.06-2.50; p = 0.026). CONCLUSIONS: The annual incidence of recurrent HSP was low. However, children who had underlying allergic rhinitis, presented with renal involvement, and received steroid treatment for > 10 days should be notified regarding the possibility of recurrence.

The clinical characteristics of adult abdominal type Henoch Scholein purpura / 中国综合临床

ObjectiveTo summarize the clinical features of adult patients with abdominal type Henoch Scholein Purpura(HSP) to improve the early diagnosis and therapeutic outcome of HSP.Methods The clinical features of 31 adult patients with abdominal type HSP from the Hematology department of Handan First Hospital between March 2007 to June 2010 were retrospectively reviewed and compared with that of 30 adult patients with non-abdominal type HSP.Results All the 31 patients had abdominal pain.Of those patients,48.4％ had periumbilical colicky pain, 38.7％ had whole abdominal pain and 12.9％ had lower abdominal pain.The other clinical manifestations included nausea/vomiting(19.4％),positive feces occult blood test(45.1％) .EGD and Colonoscopy of 13 patients showed mucosal congestion, red macda, erosion and ulceration mainly in the descending duodenum, distal ileum and rectosigmoid.There was no difference in age, gender, allergic history and predisposing infection between those with abdominal pain and without.The patients with abdominal pain had more cases of mixed-type HSP than those without and the difference was significant (P ＜ 0.01) .Conclusion Patients with abdominal-type of HSP are mainly young adults.So we must be more alert to abdominal-type of HSP when facing young patients with abdominal emergency.Abdominal-pain-manifesting HSP patients are mostly mixed-type cases.Gastrointestinal endoscopy is specific and might provide valuable clues for early diagnosis.

Transforming growth factor-beta gene promoter polymorphism:its association with renal involvement in Henoch-Scholein Purpura in childhood / 소아과

Purpose: Several cytokines play important roles in the inflammatory process of Henoch-Scholein Purpura (HSP). It is likely that transforming growth factor-beta (TGF-beta) is involved in the pathogenesis of HSP. The purpose of this study is to investigate whether TGF-beta promoter polymorphism is associated with the renal involvement of childhood HSP. Methods: Thirty-four patients younger than 15 years, who had been diagnosed with HSP, as well as 27 controls, were examined. Patients and controls were genotyped for TGF-beta C-509T by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Results: The T allelic frequencies in patients and controls showed no difference (45% vs. 48.8%). No allele or genotype differences between the group of HSP group and control group were observed. The frequencies of TGF-beta 509 genotypes TT, TC, and CC were no different between patients and controls (26% vs. 22%). The TT genotype of polymorphism of the TGF-beta C-509T gene had no relation to the susceptibility of children to HSP and renal involvement in HSP. Conclusion: TGF-beta T allele may not be related to the susceptibility of children to HSP. The TT genotype of polymorphism of the TGF-beta C 509T gene does not appear to have an influence on renal involvement in childhood HSP.

Study Progress of Gastrointestinal Manifestations in Henoch-Scholein Purpura / 实用儿科临床杂志

Henoch-Scholein purpura(HSP)is a common systemic vasculitis of the small vessel in children,with a variety of clinical manifestations.The main diagnostic criteria include palpable skin purpura.Patients who have gastrointestinal tract as the main symptoms suffer clinically abdominal pain and gastrointestinal bleeding,they are easily misdiagnosed before skin purpura,the rate of misdiagnosis can be as high as 87.5%,and the main treatment is early application-oriented glucocorticoids.This review include etiopathogenisis,pathogenesis,pathology,clinical manifestations,auxiliary examination and treatment about gastrointestinal manifestations in HSP.

Henoch-Scholein Purpura Presenting with Acute Abdominal Pain Preceding Skin Rash: Review of 23 Cases

PURPOSE: For the early diagnosis of Henoch-Schonlein purpura(HSP) presenting with acute abdominal pain preceding skin rash. METHODS: The clinical, endoscopic and radiological records of 23 cases of HSP, presenting with gastrointestinal symptoms preceding skin rash were reviewed. RESULTS: The intervals from the onset of abdominal pain to the development of the skin rash were one day to 30 days(median five days), most of them were within two weeks. The presenting abdominal symptoms were abdominal pain(23 cases), vomiting(16 cases), hematochezia or melena(eight cases) and hematemesis(three cases). The abnormal endoscopic findings include coalescing erythematous lesions, areas of submucosal hemorrhage and superficial erosions and ulcers. The upper gastro intestinal endoscopy showed the abnormalities in 21 of 23 cases, which were observed in the duodenum(21 cases), the stomach(12 cases) and the esophagus(one case). Duodenitis with hemorrhage and/or erosions in the descending duodenum was the sole endoscopic abnormality in two cases and was the most marked finding in three cases. Sigmoidoscopy showed the abnormalities in six of eight cases. The abdominal ultrasonogram showed abnormalities in 12 of 17 cases, which included small bowel wall thickening(eight cases) and intramural hemorrhage(three cases). Recurrences after three months of symptom free intervals developed in four cases; three of them had persistent nephritis beyond one year. CONCLUSION: The erosive hemorrhagic duodenitis in the descending duodenum in the upper endoscopy and the small bowel wall thickening in the abdominal ultrasonogram can be useful findings in the diagnosis of HSP presenting with acute abdomen.

A Case of Hemorrhagic Bullous Henoch-Scholein Purpura: Cellulitis Presenting as a Complication / 대한피부과학회지

Henoch-Scholein Purpura(HSP) is a syndrome characterized by nonthrombocytopenic purpura accompanied by a arthralgia, gastrointestinal symptom, or renal involvement. This report describes a 13-year-old boy with hemorrhaic bullous HSP followed by cellulitis as a complication. This unusual cutaneous manifestation of HSP may be a source of diagnostic confusion, but it does not appear to have prognostic implication.