Henoch-Schönlein purpura in Wiskott-Aldrich syndrome

Wiskott-Aldrich syndrome (WAS) is a rare immune deficiency disease. Sialophorin glycosylation is defective in WAS. Although it is not very common, renal involvement including IgA nephropathy (IgAN) was reported. Abnormal glycosylation plays a key role in the pathogenesis of IgAN. We present an 8-year-old boy with WAS who had recurrent episodes of Henoch-Schönlein purpura with renal involvement following upper respiratory tract infections. His renal function did not deteriorate. Both IgAN and WAS have glycosylation defects, but there must be some other factors (genetic and environmental) to explain their rare association.     

Subcutaneous nodules in Henoch-Schönlein purpura

A 6.5-year-old boy with active Henoch-Schönlein purpura developed subcutaneous nodules (SCN), a vasculitic manifestation previously unreported in children with this disease. The authors suggest that pressure played a role in the pathogenesis of the SCN.     

Pathogenesis of Henoch-Schönlein purpura nephritis

The severity of renal involvement is the major factor determining the long-term outcome of children with Henoch-Schönlein purpura (HSP) nephritis (HSPN). Approximately 40% children with HSP develop nephritis, usually within 4 to 6 weeks after the initial onset of the typical purpuric rashes. Although the pathogenetic mechanisms are still not fully delineated, several studies suggest that galactose-deficient IgA1 (Gd-IgA1) is recognized by anti-glycan antibodies, leading to the formation of the circulating immune complexes and their mesangial deposition that induce renal injury in HSPN.     

Adult Henoch-Schönlein purpura

Henoch-Sch?nlein purpura (HSP) is a systemic IgA vasculitis affecting small vessels. HSP usually affect children whereas it is rare in adults (150 to 200 for 1) in which the disease is often more serious with more frequent and severe nephritis. Prevalence of adult PR is unknown and its annual incidence is 1 in 1 million. The dominant clinical features include cutaneous purpura, arthritis and gastrointestinal symptoms. Some times nephritis can add, typically as glomerulonephritis with IgA mesangial deposits. Pulmonary, cardiac, genital and neurological symptoms have also been observed. Although the cause is unknown, it is clear that IgA plays a pivotal role in the immunopathogenesis of HSP. Only symptomatic treatment is advised in case of self limited disease. Treatment of severe HSP, nephritis or gastrointestinal manifestations, is not established but some studies, which need to be confirmed, reported the benefit of corticosteroids combined with immunosuppressive drugs. Short term outcome depends on the severity of the gastro-intestinal manifestations. The long term prognosis is heavily dependent on the presence and severity of nephritis. Studies with prolonged follow-up show up to one third of adult patients reaching end stage renal failure.     

Potential role of tumor necrosis factor-α in downregulating sex hormone-binding globulin

Low plasma sex hormone-binding globulin (SHBG) levels are associated with obesity and predict the development of type 2 diabetes. The reason why obese individuals have low circulating SHBG has been attributed to hyperinsulinemia, but no mechanistic evidence has been described. The aim of the current study is to explore whether tumor necrosis factor-α (TNF-α) rather than insulin could be the main factor accounting for low SHBG levels in obesity. We performed in vitro and in vivo studies using human HepG2 cells and human SHBG transgenic mice. In addition, a cross-sectional study to explore the relationship between TNF-α and SHBG in obese patients and an interventional study to examine the effect of insulin administration on circulating SHBG in type 2 diabetic patients were performed. We provide evidence that TNF-α, but not insulin, is the main factor by which SHBG is reduced in obesity. Plasma SHBG was significantly increased rather than decreased after insulin treatment in diabetic patients. TNF-α-induced reduction of SHBG expression was mediated by downregulating HNF4A. Finally, a negative and independent correlation was found between plasma TNF-α receptor 1 and SHBG levels in obese patients. Our results suggest that TNF-α plays an important role downregulating SHBG in chronic low-grade inflammatory diseases such as obesity and type 2 diabetes.     

Impaired vascular endothelium-dependent relaxation in Henoch-Schönlein purpura

Few reports have focused on vascular endothelial function in children with Henoch-Schönlein purpura (HSP). The purpose of the present study was to assess endothelial function and to follow serial changes from the acute to convalescent phases in children with HSP. Forearm flow-mediated vasodilation was evaluated in 21 patients with HSP, aged 4.0–10.3 years (median 6.2 years), and in 14 control subjects. Vascular dimension, mean velocity, and flow volume were measured by ultrasonography in brachial artery before and after hyperemia, and during incremental infusions of nitroglycerin (0.5, 1.0 g/kg per min). In the controls, significant increases in dimension, mean velocity, and flow volume were observed in reactive hyperemia (P<0.01). In contrast, patients in the acute phase of HSP showed a flow velocity profile indicating a highly resistant forearm circulation, and significantly attenuated responses after hyperemia (P<0.01 vs. control), whereas the responses to nitroglycerin were well preserved. In addition, the impaired hyperemic responses recovered in the convalescent phase, with no significant differences compared with controls. These results clearly suggest that forearm vascular endothelium-dependent relaxation was attenuated in patients with acute HSP.     

Polymorphism of the ACE gene in Henoch-Schönlein purpura nephritis

Individuals with IgA nephropathy (IgAN) who are homozygous for the deletion (D) polymorphism of the gene for angiotensin converting enzyme (ACE) are reported to be at increased risk of progressive renal damage. Since IgAN and Henoch-Schönlein purpura with associated nephritis (HSPN) share a common aetiology, we have investigated this influence in 31 children with HSPN. The distribution of genotypes was as follows: II: 4, ID: 17 and DD: 10 patients. Median length of follow-up was 4.5 years (range 0.5–15.75 years). Severe onset with nephrotic oedema and crescent formation on renal biopsy was seen in 10 of 17 patients with ID genotype and 5 of 10 patients with DD genotype. In the ID group, 2 patients have undergone renal transplantation and 4 have persistent proteinuria 4, 7, 9 and 10 years after presentation. One patient in the DD group has been transplanted and 1 patient has proteinuria and a reduced glomerular filtration rate 5 years after initial presentation. All other patients have either made a complete recovery or have microscopic haematuria alone. These results do not support an association between disease severity and DD genotype in children with HSPN; however larger studies are required to confirm this.     

Therapeutic strategy in children with Henoch-Schönlein purpura

Henoch-Sch?nlein Purpura (HSP) is the most frequent childhood primary immune vasculitis which affected skin, joints, gastro-intestinal tract and kidney. Renal involvement signs the future disease prognosis. The HSP don't have etiologic treatment but its immuno-histological aspects, leukocytoclastic vasculitis with immune complexes, imposed the including in the therapeutic equation, immunosuppressive and/or cytotoxic drugs, plasmapheresis, kidney transplant (severe renal deficiency resistant to medical treatment) and surgical treatment (in severe gastro-intestinal complications). Identifications of serum and/or urinary markers for the therapeutic answer contribute to appreciate long prognosis disease and precocious and individualized treatment.     

Contrast enema in children with Henoch-Schönlein purpura

Background/Purpose

There are references in the medical literature that Henoch-Schönle purpura (HSP) and abdominal pain are contraindictions to performing contrast enemas (CEs) for diagnosis and possible reduction of intussusceptions. We investigated the safety of performing CEs in patients with abdominal pain and HSP.

Methods

A retrospective chart review and literature search were conducted.

Results

CEs were not associated with complications in patients with HSP and abdominal pain and intussusceptions.

Conclusions

CEs are safe to perform in patients with HSP and suspected intussusceptions and may be useful for diagnosis and treatment.     

Henoch-Schönlein purpura and intestinal perforation

Sch?nlein-Henoch purpura is one of the most common forms of vasculitis in childhood, and intestinal perforation, necrosis and intussusception constitute the major surgical conditions. We present one recent case of spontaneous small bowel perforation without intussusception. An intestinal resection and ileostomy were performed. Perforation, usually ileal, frequently is accompanied by intussusception. We believe the the perforation is secondary to deep ischemic phenomenon of the bowel.     

Glomerulonephritis without IgA deposits in a case of Henoch-Schönlein purpura

A boy aged 3 years 8 months with Henoch-Schönlein purpura (HSP) developed significant proteinuria with hematuria 2 days after the appearance of purpura rash. Although thought to be purpura nephritis, a percutaneous renal biopsy revealed diffuse mesangial proliferative glomerulonephritis (MesPGN) without deposition of immunoglobulin A or complement. Since his urine screening test during a health check at the age of 3.5 years had been unremarkable, HSP might have played a role in the pathogenesis of his non-IgA MesPGN. To our knowledge, non-IgA MesPGN is an uncommon renal manifestation of HSP.     

Perforated appendicitis in a child with Henoch-Schönlein purpura

Henoch-Sch?nlein purpura (HSP) is a common childhood vasculitis. Abdominal pain is a common feature of HSP, often leading to surgical consultation for evaluation of possible intussusception. Appendicitis is a rare complication of HSP, and in each of the 3 reported cases, appendectomy preceded the appearance of the purpuric rash. More often, unnecessary laparotomies are performed on patients in whom appendicitis is suspected, but who subsequently develop the characteristic purpura. This is the first reported case of appendicitis developing in a patient with the established HSP rash. This case is also the first report of perforated appendicitis in HSP. Clinical vigilance and serial physical and ultrasonographic examinations are needed to detect conditions necessitating surgery in patients with HSP.     

Henoch-Schönlein purpura and Crohn's disease in a family

A 16-year-old female who underwent an appendicectomy had terminal segmental ileitis, and developed Henoch-Schonlein purpura (HSP) a few days later. Her brother had suffered from post-infection HSP, while her mother has suffered from Crohn's disease. Human leukocyte antigen (HLA) typing in the patient disclosed the DRB1*11 allele, which has been reported to be associated with HSP, but the brother proved negative, suggesting that this allele was irrelevant to the HSP pathogenesis. The patient and the other relatives did not disclose HLA DRB1*01, which is the only class II phenotype reported to be associated with both diseases. While this case report lends support to the idea that the earlier observation of concomitant Crohn's disease and HSP in the same patients is no chance association, it suggests that if the two pathological conditions share a common genetic background, this does not seem to be related to class II HLA phenotypes. Other, as yet unknown genes could be involved.     

Laboratory signs of activated coagulation are common in Henoch-Schönlein purpura

We investigated 17 patients with Henoch-Sch?nlein purpura (HSP) and describe as yet unreported abnormal results of blood coagulation tests. In parallel to the activity of the disease, D-dimer concentrations in plasma were found to be significantly increased in 15 of the 17 patients; almost 50% of all patients showed values higher than 10 times the upper limit of the normal range. In 11 patients, plasma concentrations of thrombin-antithrombin complex (TAT) and prothrombin fragments 1 and 2 (F1+2) were examined; six of them showed abnormal results. The pathologic values were correlated to the activity of the disease, but abnormalities were also found in milder cases of HSP. These findings probably reflect local reactions within inflamed blood vessels rather than a systemic activation of coagulation and hyperfibrinolysis. Clinicians should be aware of these laboratory findings in order not to confuse common cases of HSP with purpura necroticans, a very severe type of vasculitis in which signs of disseminated intravascular coagulation (DIC) have been reported. Our findings suggest that an activation of coagulation including hyperfibrinolysis secondary to the endothelial damage is a typical feature of the common types of HSP. Received: 13 February 2001 / Revised: 26 July 2001 / Accepted: 26 July 2001     

A case of Henoch-Schönlein purpura in a patient on hemodialysis

Henoch-Sch?nlein purpura(HSP) is a systemic vasculitis and characterized by the tissue deposition of IgA-containing immune complexes. A 50-year-old man with end stage renal failure due to diabetic nephropathy on maintenance hemodialysis, presented purpura, hematuria, abdominal pain, and joint pain. He also presented a high fever with neutrophilia. Biopsy of skin lesions revealed inflammation of the small vessel accompanied by vascular IgA deposition. Based on the clinical symptoms and skin biopsy, we made the diagnosis of HSP. Oral prednisolone was administered resulting in an improvement of the clinical symptoms. A skin biopsy should be performed for histological and immunofluorescence studies in the case of clinical suspicion of HSP with end stage renal disease on hemodialysis.