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Henoch-Schönlein purpura (HSP) is the most common form of an immunological systemic vasculitis of childhood. The classic clinical symptoms include purpuric rash, abdominal pain, arthralgias, and haematuria, but the spectrum of HSP may vary to very rare forms. This article reports on an 8-year-old girl with a Henoch-Schönlein purpura (HSP) which resulted in an obstructive bladder mass and subsequent urinary retention. This is the first case reported in the literature, describing such a course., (© Georg Thieme Verlag KG Stuttgart · New York.)

Henoch-Schoenlein Purpura (PSH) is the most frequent vasculitis in childhood. Skin, bowel and joints are characteristically involved. Although renal manifestations are common PSH-nephritis is infrequent but characterized by a poor prognosis. In autumn 2005 diagnosis and treatment were discussed at the 8th consensus meeting in Wörlitz. These results are summarized.

A 17-year old adolescent presented with abdominal pain, petechial lesions, arthralgia, and hematochezia. Abdominal ultrasound showed a suspicious ileocecal region and a lower endoscopy revealed the picture of an ileitis terminalis. The diagnosis of Purpura Henoch Schoenlein was made and confirmed by skin-biopsy showing a leucocytoclastic vasculitis. This case report demonstrates the various clinical symptoms of Purpura Henoch Schoenlein and its clinical course.

History and Clinical Findings: A 23-year-old man with HIV-infection and recently diagnosed hepatitis B infection developed painful swelling and palpable purpura in the limbs. In addition, relapsing diarrhoea, arthralgia and pain at the level of the right kidney were noted. The liver, the palpable lymph nodes and the tonsils were enlarged., Investigations: The following laboratory parameters were abnormal: sedimentation rate 64/70 mmHg, CD4+ cells 462/ml, CD4/CD8 ratio 0.3, serum CRP 16 mg/l, antistreptolysin titre 542 kU/l, complement C3 < 0.1 g/l, GPT 37 U/l, albumin 49.5% and gamma-globulin 28.5%, in urine, leukocytes 25/microliter and protein 0.25 g/l. Hepatitis serology: anti-HBc, HBs-Ag and HBe-Ag positive, anti-HBs and anti-HBe negative, hepatitis A-Ab (IgG) positive, hepatitis A-Ab (IgM) negative, hepatitis C-Ab negative. Pharyngial swab: beta-haemolytic streptococci of group A. Stool examination: amoeba cysts, corona virus and fungi 10(4)-10(5)/ml (control specimen negative). Abdominal sonography showed minor hepatomegaly, splenomegaly and two 0.8 cm-large lymph nodes near the liver entrance. The histology of a skin lesion revealed a florid leukocytoclastic vasculitis at the upper and middle dermal vessels. The immunohistological examination detected IgA, IgM and C3 deposits., Treatment and Course: The multiple bacterial infections had initially been considered as triggering the Schönlein-Henoch purpura. However despite successful treatment of these infections severe relapses of both the skin lesions and the systemic manifestations occurred. A drug reaction, autoimmune diseases and tumours were excluded. Only after treatment of hepatitis B infection with recombinant interferon alpha together with administration of zidovudine (treatment of choice at that time) the lesions quickly disappeared without further relapses. During the 5-year-follow-up, recurrent bacterial infections did not induce relapses of the disease, whereas the hepatitis B infection was seroconverted., Conclusion: It is likely that the hepatitis B may have been the trigger for the Schönlein-Hennoch-purpura.

Unlabelled: The necessity of the controversially discussed general steroid prophylaxis in Henoch-Schönlein Purpura (HSP) was analysed based on frequency, risk factors and prognosis of renal involvement. Case histories and follow up of at least 1.5 years were evaluated in all 171 patients (median age 6 years) in our institution suffering from HSP between 1.1.1987 and 30.6.1997. HSP was frequently manifest with an involvement of joints (64%) and gastrointestinal tract (58%). Renal involvement occurred in only 29% of the children. Excepted one girl (age 12 year), all children with renal disease completely recovered. Renal involvement was significantly rarer in young children (16%) and after one week therapy with prednisone (7%). In young children, renal involvement always followed abdominal pain., Conclusion: In our collective, a general preventing of renal disease in HSP was unnecessary. Especially in young children, the most common manifestation age, renal involvement rarely occurred and had always a good prognosis.

A 51-year-old male patient admitted to the hospital because of colic-like abdominal pain, paralytic ileus, anal bleeding and microhaematuria with proteinuria, developed an intestinal ischemia with a serum lactate level of 6.3 mmol/l. An occlusion of the large vessels was excluded angiographically. Perfusion disorders were detected both endoscopically and histologically in the upper gastrointestinal tract and in the terminal ileum. When after two days a palpable purpura appeared on the anterior of both feet, a vasculitis type Schoenlein-Henoch was suspected and treated with high doses of steroids, resulting in decreasing symptoms. From the point of admittance, a nephritic urinary sediment had been apparent, and the renal affliction developed into a nephrotic syndrome without notable reduction in the glomerular filtration rate. On the 13th day of treatment the patient-being on a reduced dose of steroids-suffered from a severe relapse; however, this responded favorably to an increase of the dosage. The kidneys required approximately one year for complete recovery. Based on this case, the Schoenlein-Henoch purpura syndrome and its differential diagnosis are presented, particularly with respect to gastrointestinal symptoms and in view of the pertinent literature.

We evaluated the records of 139 pediatric patients with Schönlein-Henoch purpura (aged 0.4 to 15.1 years, median 5.4 years), referred from 1974 to 1993 to the University Children's Hospital, Berne. An acute febrile illness preceded Henoch-Schönlein purpura in 83 (60%) out of the 139 children. The purpuric papules were distributed over the lower extremities and the buttocks in 68 patients (49%), and more extensively in the remaining 71 (51%). Joint tenderness or swelling was observed in 110 patients (79%). Abdominal involvement occurred in 92 patients (66%): abdominal pain (n = 63), melena or hematemesis (n = 23), and intussusception (n = 6). The following rather rare features were observed: scrotal swelling (n = 11), neurologic involvement (n = 3), and stenosing ureteritis (n = 1). A remission lasting at least 4 weeks occurred within 4 weeks in 84, within 5-8 weeks in 19, and within 9-53 weeks in 18 out of 121 patients. A relapse was observed in 10 subjects. Renal involvement occurred in 60 patients. Severe renal involvement, defined as proteinuria exceeding 40 mg/[m2 X h], occurred in 18 of the patients with renal involvement. Progredient renal failure developed in one male. Children with renal involvement tended to be older (6.6 versus 4.1 years) and to have more prolonged extrarenal manifestations (6 versus 3 weeks). It is concluded that in children with Schönlein-Henoch purpura extrarenal involvement often lasts more than one month. Its outcome is almost always favourable. Children with heavy proteinuria tend in some cases to develop renal failure. For these patients, new treatment regimens aimed at preventing renal failure warrant evaluation in prospective controlled studies.

Clinical and morphological findings were evaluated in 25 children with mesangial IgA deposits. 19 patients had recurrent macroscopic hematuria (n = 10), chronic proteinuria > 40 mg/(m2.h) (n = 5), recurrent hematuria with chronic proteinuria (n = 3), or chronic nephrotic syndrome (n = 1). The glomerular involvement was similar in six patients with history of Schönlein-Henoch purpura and in 13 patients without such history: normal or nearly normal glomeruli (n = 3), focal and segmental glomerulonephritis (n = 11) and diffuse proliferative glomerulonephritis (n = 5). End-stage renal disease developed in two patients with proteinuria > 40 mg/(m2.h) and more than 50% of their glomeruli are affected by crescents. The common histopathological features in patients with and without history of Schönlein-Henoch purpura suggest a common pathogenesis. The risk of poor outcome appears, related to the severity of proteinuria and to the presence of crescents, in more than 50% of glomeruli. Mesangial IgA deposits were also demonstrated in six children with steroid-responsive idiopathic nephrotic syndrome: light microscopic studies revealed normal or nearly normal glomeruli in five and focal segmental glomerular sclerosis in one patient. The microscopic findings were clearly different in the six patients with idiopathic nephrotic syndrome as compared with the patient with chronic nephrotic syndrome, who presented with severe glomerular lesions and extensive crescent formation. The results indicate that the presence of mesangial IgA deposits in the clinical setting of idiopathic childhood nephrotic syndrome is incidental and that such patients should still be considered as having idiopathic nephrotic syndrome in spite of their immunopathological features.

Aim of Study: A wide spectrum of glomerular diseases manifests as a nephritic syndrome with haematuria, proteinuria, hypertension, edema, and impaired renal function. Little is known about the presentation of each symptom and the distribution of the underlying glomerular diseases., Methods: In order to delineate the clinical spectrum of glomerular disease presenting as a nephritic syndrome, we examined the records of 192 patients who had been followed in our paediatric nephrological outpatient clinic between 1973 and 1988 for a nephritic syndrome., Results: Oligosymptomatic courses with microhaematuria and proteinuria predominated. The mean age at presentation was 8.1 years. In a broad spectrum of 29 diagnoses, postinfectious glomerulonephritis and Henoch-Schönlein nephritis were the most prevalent. End-stage renal failure developed in 16% of the patients. Prognosis was poor in cases of rapidly progressive glomerulonephritis, focal segmental glomerulosclerosis, Henoch-Schönlein nephritis and in autoimmune disease, with end-stage renal failure developing in more than 20% of each of these groups., Conclusions: The nephritic syndrome often manifests oligosymptomatically. An intensive nephrological workup is indicated in order to early diagnose serious--and potentially treatable--glomerular diseases.

119 patients with Schönlein-Henoch purpura from 1 to 17 years were investigated. All patients had a rash, 76% joint affections, 76% gastrointestinal symptoms, 54% renal involvement, 17% of the boys orchitis, 2% intramuscular bleeding and 2% coagulation disorder. 1 boy showed perforation of the nasal septum, to our knowledge not described in Schönlein-Henoch purpura up to now. The mean age was 6.4 years. The seasonal peak was in October, 63% of our patients had a preceding infection, mostly of the upper respiratory tract, pointing to various infectious agents as a trigger of this disease. Patients with abdominal pain had a 4-fold, patients with bloody stools a 7.5-fold increased risk of renal involvement. 13/38 Patients had a serum albumin level below 3.5 g/dl that may have been caused either by renal or gastrointestinal losses.

We present a case of ioniazid poisoning in a two year old otherwise healthy male. Problems with history and diagnosis characteristical of such cases are discussed along with current views on the pathogenesis and therapy. A suggestion is put forward to help to avoid future similar accidents which--although very rare--are extremely dangerous.

Ten children suffering from anaphylactoid purpura Schoenlein-Henoch and presenting urinary abnormalities of various degrees and reported. The histological and immunohistochemical tests allowed to distinguish two types of glomerulonephritis in these patients: focal glomerulonephritis and extracapillary glomerulonephritis. The prognosis was always favorable: no relationship was found between the clinical signs and symptoms and the findings in the kidney. The authors discuss, on a practical and theoretical level, the indications for kidney biopsies in children with the Schoenlein-Henoch syndrome.

We report about a 40-year-old male patient suffering from a recurrent vasculitis with purpura since the age of 18. In 1983, a HBsAg-positive chronic active hepatitis with circulating immune complexes which contained HBsAg, immunoglobulin M and G as well as complement (C) was diagnosed. Serum and liver tissue were negative for HBcAg, HBeAg and hepatitis B virus (HBV) DNA; there was no evidence for HBV replication. HBsAg, IgM and C3 were demonstrable in the arteriolar walls of the skin. The results support the concept that complement activating immune complexes containing HBsAg and IgM anti HBs play a role in the pathogenesis of vasculitis as described here.

5 cases of intramural haematomas are discussed. The haematomas were of different origin, were localized at different sites and treated differently. In one case an obstructive duodenal haematoma developed after a liver and spleen rupture. This hematoma was treated by gastro-enterostomy. In the second case the cause could not be determined. The third haematoma was induced by an intestinal clamp. In the last two cases there was a tendency to haemorrhages (Morbus Schönlein-Henoch and haemophilia A).

In a 24-year-old patient suffering from recurrent purpura Schoenlein-Henoch, there were found positive Lyme-spirochete IFT. Due to the motion of titres in immune fluorescence examinations the purpura seems to be likely one form of a late manifestation in borrelia infection.

Recurrence of the original disease in the transplanted kidney is observed in 5.6%-9.3% of the patients. However, the clinical significance of recurrence is often minor. Diagnosis is easy in diseases with specific renal lesions, e.g., in dense deposit disease and IgA-nephropathy, but may be difficult if such a marker is missing. Recurrence is of special clinical importance in the following conditions: Membranoproliferative GN type I (in 33%, often severe) and type II (= dense deposit disease, recurrence in 90%, often minor), focal segmental glomerulosclerosis (in 48% of patients with a rapid course (less than 3 years) and in 12% of patients with a longer duration of the original disease; often severe), membranous nephropathy (recurrence rather rare, but often serious), and primary hyperoxaluria (in 100%). Mesangial IgA deposits recur in half of the patients with IgA-nephropathy and anaphylactoid purpura, but clinical findings are often minimal. Recurrence in anti-GBM-nephritis and SLE is rare. The study of recurrence may contribute to a better understanding of many renal diseases.

The clinical picture and the laboratory data of 40 patients with anaphylactoid purpura as well as its etiology and pathogenesis are discussed. The clinical expression and the rate of complications does not differ from that described in the literature. 45% of the patients showed renal involvement, three patients had severe glomerulonephritis. Up to now there is a lack of typical laboratory tests to confirm the diagnosis of anaphylactoid purpura. This study suggests neutrophilia and initial creased fibrin/fibrinogen split products in serum and decreased C3 levels are poor prognostic criteria correlating with appearence and severity of a complicating glomerulonephritis.

We measured the immunoglobulin, beta1 C globulin, beta1E globulin, C3a, and CH50 in 20 patients with anaphylactoid purpura (Schönlein-Henoch purpura). IgA values proved to be elevated in 5 out of 11 cases. beta1 C globulin values were lower in 2 out of 8 cases and fell within the range of normalcy in the remaining 6 cases. beta1 E globulin values were normal in all cases measured (5 patients). A 130% increase in C3a was found in 7 out of 9 cases; patients with higher C3a values suffered from lasting petechial hemorrhages. It can, therefore, be concluded that the activation of the "alternative pathway" induced by IgA led to the release of C3a.