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.     

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.     

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.     

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.     

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.     

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.     

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.     

Penile involvement in Henoch-Schönlein purpura with good prognosis

Henoch-Sch?nlein purpura (HSP), the commonest vasculitis in children, occurs most frequently between the ages of 4 and 6 years. We report the case of a 3-year-old boy with an otomastoiditis who was treated with cephalosporin and corticosteroids following a typical purpuric skin rash diagnosed as HSP. The patient also developed an acute occurrence of impairment of the glans, prepuce and penis 4 days after recovery that completely disappeared after a further 2 days, with the cutaneous rash subsiding on discharge from hospital.     

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.     

Muscle involvement in a patient with Henoch-Schönlein purpura nephritis

Although Henoch-Schönlein purpura (HSP) is a small-vessel vasculitis affecting multiple organ systems, muscle involvement has rarely been reported. This report describes muscle pain and weakness in a patient with HSP nephritis (HSPN). A 13-year-old boy suffered from purpura, abdominal pain, and symmetrical muscle pain and weakness of the extremities. He was diagnosed as having HSP with muscle involvement. His abdominal pain and muscle involvement improved 1 week after commencing oral prednisolone therapy. The patient subsequently developed biopsy proven HSPN. It should be noted that, although rare, muscle involvement might occur in HSP as in other systemic vasculitides. The precise pathogenic mechanism underlying its development is currently unclear.     

Henoch-Schönlein purpura associated with acetaminophen and codeine

We report a case of a relapse of Henoch-Sch?nlein Purpura (HSP) associated with intake of paracetamol (also known as acetaminophene) and codeine. A 69-year-old man presented with fever, gross hematuria, acute renal failure, palpable purpuric skin rash over the legs, feet and arms, arthralgias and abdominal discomfort. 1 week before he had started therapy with co-efferalgan (association of paracetamol and codeine) for cervical arthrosis. Blood test revealed increase in serum creatinine levels (2.6 mg/dl), CRP (375 mg/dl), with no thrombocytopenia or hypocomplementemia. Co-efferalgan was discontinued. Gross hematuria resolved in 2 days, purpuric rash disappeared in 10 days, renal function returned to normal after 2 weeks and abdominal pain and arthralgias improved on the following 2 - 3 weeks. An objective causality assessment in accordance with the Naranjo algorithm, revealed that the adverse drug reaction was probable between paracetamol/codeine and Henoch-Sch?nlein purpura. To our knowledge, and based on a medline search (up to 2005), we believe that this could be considered the first case of Henoch-Sch?nlein purpura, associated with intake of paracetamol and codein. Although this event could be considered rare, clinicians should to be aware of possible associations between HUS and the intake of paracetamol and/or codeine to provide an early therapeutic intervention and a close monitoring.     

Adrenomedullin and total nitrite levels in children with Henoch-Schönlein purpura

Nitric oxide (NO) is synthesized from endothelium and has an important role in the control of vascular tonus. Adrenomedullin (AM) is a potent vasodilator, and cytoprotective peptide is produced not only in adrenal medulla, but also in the vascular smooth muscle and endothelial cells. To investigate the endothelial synthesis of AM and NO, and endothelial injury in Henoch-Schönlein purpura (HSP), we measured their levels in 16 children with HSP, who were evaluated during the acute and remission phases, and compared with 12 healthy controls. Plasma AM levels (pmol/ml) were significantly higher in acute phase children (46.87±11.49) than in those in remission (35.59±12.39, p<0.01) and controls (30.70±9.12, p<0.001). Similarly, plasma total nitrite levels (mol/l) were higher in acute phase patients (47.50±12.30) than in those in remission (35.94±10.08, p<0.005) and controls (34.56±11.51, p<0.05). Urinary excretion of AM (pmol/mg creatinine) was higher in acute phase patients (53.85±23.22) than in remission patients (29.97±9.33, p<0.01) and controls (37.43±15.78, p<0.05). Patients had increased urinary nitrite excretion (mol/mg creatinine) in acute phase (2.39±1.18) compared to those in remission (1.53±0.90, p<0.05) and controls (1.05±0.61, p<0.005). There was no significant difference between remission phase and controls in AM and nitrite levels (p>0.05). This study concluded that AM and NO may have a role in the immunoinflammatory process of HSP, especially in the active stage, although whether this perpetuates, or protects against, further vascular injury is not clear. Further studies are needed to clearly establish the roles of AM and NO in the pathogenesis of HSP.