eMedicine Specialties > Pediatrics: General Medicine > Nephrology

Henoch-Schönlein Purpura

Author: Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Coauthor(s): Elena L Jones, MD, Clinical Assistant Professor of Dermatology, College of Physicians and Surgeons of Columbia University; Clinic Chief, Department of Dermatology, St Luke's-Roosevelt Hospital Center
Contributor Information and Disclosures

Updated: May 28, 2008

Introduction

Background

Willan and Heberden appeared to have first noted Henoch-Schoenlein (or Henoch-Schönlein) purpura (HSP) in the early 1800s. However, Schönlein first described the combination of acute purpura and arthritis in children in 1837, and Henoch reported the manifestations of abdominal pain and nephritis in 1874.1

HSP is an acute immunoglobulin A (IgA)–mediated leukocytoclastic vasculitis that primarily affects children. The dominant clinical features of HSP include cutaneous purpura, arthritis, abdominal pain, GI bleeding, orchitis, and nephritis.

The prevalence of HSP peaks in children aged 3-10 years. In the Northern hemisphere, the disease occurs mostly from November to January. The male-to-female ratio is 1.5-2:1. In one half to two thirds of children, an upper respiratory tract infection precedes the clinical onset of HSP by 1-3 weeks. In general, patients with HSP appear mildly ill. They often have a fever, with a temperature usually not higher than 38°C (100.4°F). HSP is typically an acute, self-limited illness; however, one third of patients have one or more recurrences.

Pathophysiology

Etiology of Henoch-Schoenlein purpura

The etiology of HSP remains unknown. However, IgA clearly plays a critical role in the immunopathogenesis of HSP, as evidenced by increased serum IgA concentrations, IgA-containing circulating immune complexes, and IgA deposition in vessel walls and renal mesangium. HSP is almost exclusively associated with abnormalities involving IgA1, rather than IgA2.

The predominance of IgA1 in HSP may be a consequence of abnormal glycosylation of O-linked oligosaccharides unique to the hinge region of IgA1 molecules. Although several lines of evidence suggest a genetic susceptibility to HSP, the fundamental basis for this abnormality remains unclear.

IgA aggregates or IgA complexes with complement deposited in target organs, resulting in elaboration of inflammatory mediators, including vascular prostaglandins such as prostacyclin, may play a central role in the pathogenesis of HSP vasculitis.

A subpopulation of human lymphocytes bears surface Fc and/or C3 receptors (complement receptor lymphocytes), which can bind circulating immune complexes or C3 generated by activation of the alternative complement pathway. Such immune complexes appear in HSP and may be part of the pathogenetic mechanism.

Some have speculated that an antigen stimulates the production of IgA, which, in turn, causes the vasculitis. Allergens, such as foods, horse serum, insect bites, exposure to cold, and drugs (eg, ampicillin, erythromycin, penicillin, quinidine, quinine), may precipitate the illness. Infectious causes include bacteria (eg, Haemophilus, Parainfluenzae, Mycoplasma, Legionella, Yersinia, Shigella, or Salmonella species) and viruses (eg, adenoviruses, Epstein-Barr virus [EBV], parvoviruses, varicella). Vaccines such as those against cholera, measles, paratyphoid A and B, typhoid, and yellow fever have also been implicated. Evidence supporting a direct role of herpesvirus, retrovirus, or parvovirus infection in HSP is lacking.

Alterations in the production of interleukins (ILs) and growth factors may also have a role in the pathogenesis of HSP. Tumor necrosis factor (TNF), IL-1, and IL-6 may mediate the inflammatory process present in HSP. Transforming growth factor–beta (TGF-beta), is a recognized stimulant of IgA production. The elevated levels of hepatocyte growth factor present during the acute phase of HSP may reflect endothelial-cell damage or dysfunction. Increased levels of vascular endothelial growth factor may at least partly induce these changes. Cytokines have been implicated in the pathogenesis of HSP, and endothelins (ETs), which are vasoconstrictor hormones produced by endothelial cells, may also have a role. Levels of ET-1 are substantially higher during the acute phase of the disease than during remission or in a control group of children. However, ET-1 levels do not appear to be correlated with morbidity, severity of disease, or acute-phase reactant response.

A functional correlation of the IL1RN-2 allele and IL-1ra production in patients with IgA nephropathy and HSP nephritis (HSPN) has been described. Therefore, gene polymorphism may contribute to the diversity of clinical responses to inflammatory stimulation.

Emerging data

Results support a role of human leukocyte antigen (HLA)–B35 in the susceptibility to nephritis in unselected patients with HSP.

Researchers are currently investigating the importance of nitric oxide (NO) production in disease activity. Inducible NO synthase polymorphism is associated with susceptibility to HSP in northwestern Spain.

The prevalence of the human parvovirus B19 component NS1 gene in patients with HSP and hypersensitivity vasculitis is increased.

HSP that is likely due to montelukast has been noted in patients who present with subacute intestinal obstruction.

Other factors

Yilmaz et al (2005) examined 28 children with HSP and 79 healthy children to evaluate activities of protein C, free-protein S, and antithrombin; resistance to activated protein C; and levels of fibrinogen.2 D-dimer, thrombin-antithrombin (TAT) complex, prothrombin fragments (PFs) 1 and 2, and von Willebrand factor antigen (vWAg) and its activity (RiCof) were also investigated.

Among patients with HSP, fibrinogen, D-dimer, TAT complex, PF-1, PF-2, vWAg, and RiCof levels were significantly higher during the acute phase were than during recovery phase and were significantly higher than those of control subjects. The severity of disease was significantly correlated with TAT, PF-1, PF-2, vWAg, and D-dimer levels.

Aliyazicioglu et al (2007) has suggested that leptin and NO may play a role in the immunoinflammatory process of HSP, especially in the acute phase.3

Frequency

United States

In the United States, the prevalence is approximately 14-15 cases per 100,000 population.

International

In the United Kingdom, the estimated annual incidence of HSP is 20.4 cases per 100,000 population.4 In a Norwegian community hospital, the prevalence of HSP was 3.3 cases per 100,000 inhabitants.5

In a study that examined the renal biopsy results of 65 children younger than 18 years obtained by the Clinical Hospital in the Croatian region of Dalmatia over a 10-year period (1995-2005), 10.8% of glomerulonephritis cases were due to HSP.6  

Nong et al (2007)reviewed the records of 107 Taiwanese pediatric patients diagnosed with HSP between 1991 and 2005 who had a mean age of 6.2 ± 2.5 years (range, 2-13 y); the male-to-female ratio was 1:0.7.7 The primary symptoms included the following:

  • Skin rashes (95.3%)
  • GI symptoms (72.0%)
  • Joint involvement (46.7%)
  • Kidney involvement (28.0%)

The most common first manifestations were as follows:

  • Skin rashes (56.1%)
  • GI symptoms (35.5%)
  • Joint involvement (12.1%)

From January 1983 to June 2004, Suehiro et al (2007) followed 4,502 patients at the Pediatric Rheumatology clinic in Brazil.8  A diagnosis of HSP was made in 203 cases (4.5%); 5 patients (0.1%) had AHEI. All patients with AHEI were male, and the mean age at onset was 18 months (range, 8-21 mo). 

Mortality/Morbidity

  • HSP is only fatal in the rarest of cases.
  • Initial attacks of HSP can last several months, and relapses are possible. Kidney damage related to HSP is the primary cause of morbidity and mortality.
  • Overall, an estimated 2% of cases of HSP progress to renal failure; as many as 20% of children who have HSP and are treated in specialized centers require hemodialysis. The renal prognosis appears to be worse in adults than in children.

Race

  • Whites are affected more often than blacks.
  • In one study in Thailand, the most common age at presentation was 3-5 years.9 The frequency peaked from December to February. Organs involved included the skin (100%), GI tract (74.5%), and kidneys (46.8%). Joints were also affected (42.6%). Renal involvement was detected within the first 2 months in 16 patients (72.7%); however, it was delayed until 6 months after diagnosis in 6 patients. No risk factors for renal involvement could be identified. Mean follow-up was 2.6 years (range, 1-5 y). Residual renal disease occurred in 6 (38%) of 16 patients, but none were had end-stage disease.
  • In a study in China, a male predominance was observed in children but not in adults.10 Preceding infection was noted in 40.5% of children and 31.6% of adults; 8.3% of children and 13.2% of adults were receiving medication at the onset of the disease. Abdominal pain was more common in children than adults (70.2% vs 28.9%), but renal involvement was more common and severe in adults than in children; this involvement manifested as frequent hypertension and heavy proteinuria. During acute attacks, leukocytosis, thrombocytosis, and elevated serum C-reactive protein levels were most frequently observed in children, whereas elevated serum IgA and cryoglobulin levels were most common in adults.

Sex

HSP occurs more often in boys than in girls; the male-to-female ratio is 1.5-2:1.

Age

  • In the United States, the prevalence peaks in children aged 5 years.
  • Approximately 75% of cases occur in children aged 2-11 years.
  • HSP is rare in infants and young children.
  • A related milder condition called acute hemorrhagic edema of infancy (AHEI) occurs in infants younger than 2 years.

Clinical

History

  • Prais et al studied 267 children (56.7% males) who were hospitalized secondary to Henoch-Schoenlein purpura (HSP); 7 of the children (2.7%) had HSP that resulted in hospitalization 2 or more times.11 No specific risk factor for recurrence was determined. The mean age for the first recurrence in that subgroup was 3 years and 8 months (range, 10 mo to 7.4 y), and the mean age for the second recurrence was 5.03 years (2.2-10 y), with a mean lag time of 13.5 months ± 2.8 months (range, 2-26 mo). The duration of the recurrent clinical symptoms was 9-30 days. Resolution took more than 2 weeks in 72% of patients.
  • Associated conditions that precede HSP include those listed in Causes.
  • Scrotal involvement is not uncommon in HSP and may mimic testicular torsion, which must be excluded. Male patients may have associated inflammation and hemorrhage of the testes, appendix testes, spermatic cord, epididymis, or scrotal wall. True torsion is rare. Ha and Lee (2007) reported that neurologic symptoms, localized edema, and high serum C3 levels have a significant relationship with scrotal involvement in male patients with HSP.12
  • GI symptoms can accompany the onset of HSP or may develop later in the course of disease. Abdominal pain occurs in 35-85% of patients and is the third most common presenting symptom in HSP. GI problems usually follow the onset of rash and joint pain. Multiple and recurrent intestinal perforations are an unusual complication of HSP. In addition to abdominal pain, GI findings can include the following:
    • Nausea
    • Vomiting
    • Diarrhea with gross or occult blood
    • Hematemesis
    • Intussusception: This occurs in 2-3% of patients, and the lead point can be a submucosal hematoma.
    • Bowel infarction with or without perforation
    • Ileal stricture
    • Ileus with massive GI bleed
  • Arthralgias occur in 60-84% of patients with HSP and most commonly affect the knees, ankles, and, less frequently, the wrists and fingers. True arthritis does not occur, and joint effusions are rare. HSP leaves no permanent joint deformities.
  • In women, gynecologic symptoms can include painful menstruation.
  • HSP can be accompanied by neurologic manifestations, particularly headaches. Ozkaya et al reported cerebral vasculitis in a child with HSP and familial Mediterranean fever.13
    • In rare cases, HSP can be associated with seizures, paresis, or coma.
    • Other manifestations include altered mental status, apathy, hyperactivity, irritability, mood lability, somnolence, seizures (partial, complex partial, generalized, status epilepticus), and focal deficits (eg, aphasia, ataxia, chorea, cortical blindness, hemiparesis, paraparesis, quadriparesis).
    • Polyradiculoneuropathies (eg, brachial plexus neuropathy, Guillain-Barré syndrome) and mononeuropathies (eg, facial nerve, femoral nerve, peroneal nerve, sciatic nerve, ulnar nerve) may also occur.
  • The liver and gallbladder can be involved in HSP.
    • Hepatomegaly, hydrops of the gallbladder, and cholecystitis may ensue. These may contribute to a patient's abdominal pain.
    • Acute appendicitis has been noted in patients with HSP.
  • Skin involvement is usually purpura. Chan et al (2007) noted a case of HSP presented as painful bullae on both legs.14
  • Acute hemorrhagic edema of infancy (AHEI) usually occurs in infants aged 4-24 months.
    • AHEI often occurs after drug ingestion or infection. The onset of AHEI is dramatic, with acute palpable purpura, ecchymoses, and tender edema of the limbs and face. Fever, if present, remains mild. Infants remain hemodynamically stable.
    • Dermatologic findings are notable for a cockade (medallionlike), rosette-shaped pattern of macular-papular-hemorrhagic lesions on the face, auricles, and extremities. The lesions usually appear in successive crops. The cockades display variable stages of evolution at any given time.
    • Subcutaneous edema is most common in infants. Urticaria, petechiae, and ear lobe necrosis are additional rare skin manifestations of AHEI. Visceral involvement is rare.
  • Renal pathology is the most important cause of morbidity and mortality in patients with HSP.
    • Renal involvement may precede skin manifestations (1-4% of patients) but is usually evident during the acute phase of the disease.
    • In most cases, the severity of nephritis is not related to the extent of other HSP manifestations.
    • Hematuria, usually microscopic, can be accompanied by mild-to-moderate proteinuria (<2 g/d). Oliguria, hypertension, and azotemia are rarely present. Nephrotic syndrome (urinary protein excretion >40 mg/m2/h) can also occur.
    • In most cases, histologic examination of the kidneys reveals mesangial proliferation that can be diffuse or focal and segmental. Resolution of the renal involvement is the focus in these patients.
    • Patients who present with hematuria and persistent proteinuria have an approximate 15% risk of developing renal failure. The risk may increase to 50% in patients with a nephrotic-nephritic syndrome.
  • Urinary complications include bladder-wall hematoma, calcified ureter, hydronephrosis, and urethritis.
  • Hemoptysis and hemarthroses can develop in patients who have bleeding abnormalities such as coagulopathy, factor VIII deficiency, vitamin K deficiency, or hypoprothrombinemia. They also probably include factor V Leiden, protein C deficiency, and protein S deficiency, but this has not been documented.

Physical

Purpura of the skin is the most prominent physical finding in HSP, but renal, GI, and joint manifestations are commonly present. Other manifestations have also been reported.

HSP begins with a symmetrical erythematous macular rash on the lower extremities that quickly evolves into purpura. The rash may initially be confined to malleolar skin but usually extends to the dorsal surface of the legs, the buttocks, and the ulnar side of the arms. Within 12-24 hours, the macules evolve into purpuric lesions that are dusky red and have a diameter of 0.5-2 cm. The lesions may coalesce into larger plaques that resemble ecchymoses. Several cases of HSP have been observed after varicella infections.

In children younger than 2 years, the clinical picture may be dominated by edema of the scalp, periorbital area, hands, and feet. This presentation is termed AHEI. The severity of edema is correlated with the severity of the vasculitis and not with the degree of proteinuria. However, the edema has been attributed to the enteric loss of protein. 

Overview of renal findings
The most serious complication of HSP is renal involvement, which occurs in 50% of older children but is serious in only approximately 10% of patients. In 80% of patients, renal involvement becomes apparent within the first 4 weeks of illness. Overall, 2-5% of patients progress to end-stage renal failure (ESRF). In one series, acute glomerular lesions, including mesangial hypercellularity, endocapillary proliferation, necrosis, cellular crescents, and leukocyte infiltration, were observed in 41%, 12%, 50%, 29%, and 32% of patients, respectively.15 Only glomerular necrotizing lesions and cellular crescents correlated with the renal survival rate and were associated with clinically significant proteinuria and development of hypertension.

The relationship of HSP and IgA nephropathy requires further definition. Whether they are the same or distinct entities remains unclear. Evidence of both their commonality and distinctiveness is presented herein.

Evidence that they are the same entity includes the following:

  • The occurrence of extrarenal manifestations in IgA nephropathy is similar to those observed in HSP.
  • IgA nephropathy has developed in patients with a history of HSP. HSP and IgA nephropathy have occurred in the same families.
  • In a survey of 40 families in which 2 or more members had IgA nephropathy, 5 presented with HSP.16
  • Patients with HSP who undergo renal transplantation develop IgA deposits in the graft.
  • The prevalence of both conditions is high in certain geographic areas.
  • Similar changes in the IgA system (ie, high IgA, IgA-1C, IgA1-IC, IgA-fibronectin aggregates, aberrantly glycosylated IgA in the circulation) occur in both diseases.17,18,19
However, data indicate that HSP and IgA nephropathy are distinct diseases. Zhou et al (2003) examined 31 children aged 3-15 years with IgA nephropathy and 120 children aged 4-15 years with HSP, noting their clinical manifestations, blood biochemistries, serum immunology, and follow-up data.20 Renal pathologic findings on light microscopy, immunofluorescence study, and electron microscopy were analyzed and compared between 31 children with IgA nephropathy and 32 children with HSP.

The age of onset was more than 12 years in 25.8% of the children with IgA nephropathy. However, the age of onset was more than 12 years in only 10% of those with HSP. Clinical patterns of IgA nephropathy and HSP were similar, but extrarenal manifestations were observed most often in patients with HSP. All patients with HSP had skin purpura, 59% of patients had GI symptoms, and 47% of patients had arthralgia. Abdominal pain occurred in only 3.2% of children with IgA nephropathy. In patients with IgA nephrology and in patients with HSP, renal pathology revealed global sclerosis in 35.5% and 3.1%, mesangial sclerosis in 41.9% and 6.3%, endothelial proliferation in 29% and 65.6%, and thin basement-membrane nephropathy in 6.5% and 0%, respectively.

Electronically dense deposits in HSP were sparse, loose, and widely spread in the glomerular mesangium, in the subendothelial area, and even in the intrabasement membranes, whereas the deposits were dense, lumpy, and mostly limited in mesangium and paramesangium in IgA nephropathy. Immunoglobulin G (IgG) was found in glomerular immune deposits in 71.9% of patients with HSP but in only 19.4% of patients with IgA nephropathy. No IgG deposit was observed in 81.6% of those with IgA nephropathy; most had IgA and immunoglobulin M (IgM) and/or C3 deposit. Predominant IgG deposits were found in 12.5% of patients HSP, with relatively weak IgA deposits. Moreover, 6.3% of patients with HSP had linear IgG deposits in the glomerular capillary wall, which was not found in IgA nephropathy.

The follow-up data at an average of 20 months showed complete remission in 72.5% of patients with HSP; the rate was 19.4% of those with IgA nephropathy after 34 months follow-up. Moreover, 64.5% of patients with IgA nephropathy had consistent hematuria and proteinuria, and 16.1% had active nephritides. Therefore, the difference was significant (P <.05).

Zhou et al found important clinicopathologic differences between HSP and IgA nephropathy, which does not support the one-disease hypothesis.

Overview of GI findings
Abdominal pain and bloody diarrhea may precede the typical purpuric rash of HSP in 14-36% of patients, complicating the initial diagnosis and even resulting in unnecessary laparotomy. GI manifestations occur in about 50% of cases and usually consist of colicky abdominal pain, melena, or bloody diarrhea. Hematemesis occurs less frequently. Intussusception should be suspected in patients HSP with abdominal pain and/or melena. Barium enema is frequently therapeutic.

Overview of joint findings

Arthralgias occur in 60-84% of patients with HSP. The pain most commonly affects the knees and ankles and less frequently the wrists and fingers. Frank arthritis does not occur, and joint effusions are rare. HSP leaves no permanent joint deformities.
  • Skin manifestations
    • Palpable purpura usually occurs first on the lower limbs and then spreads to the buttocks. Purpura is usually most prominent over the buttocks, the posterior aspects of the lower legs, and the elbows.
    • Palpable purpura can also be present on the forearms and pinna. Scalp edema can occur. Hemorrhagic vesicles and bullae are rare. In most patients, the skin lesions are the first sign of HSP.
    • Hives, angioedema, and target lesions can also occur. Vesicular eruptions and swelling and tenderness of an entire limb have been noted. Erythema multiforme–like lesions can be present.
    • HSP with hemorrhagic bullae in children has been noted.
    • In AHEI, dermatologic findings are notable for a cockade (medallionlike), rosette-shaped pattern on the face, auricles, and extremities. The lesions usually appear in successive crops. The cockades display variable stages of evolution at any given time and look different from the normal purpura of HSP.
    • The subcutaneous edema of AHEI is more common in infants. Urticaria, petechiae, and necrosis of the ear lobe are additional, rare skin manifestations of AHEI. AHEI is rarely associated with visceral involvement.
  • Renal manifestations
    • Possible renal manifestations include microscopic hematuria, proteinuria, nephrotic syndrome, progressive glomerulonephritis, ESRF (rarely), and intestinal perforation.
    • The relationship of HSP and IgA nephropathy requires further definition. Whether they are the same or distinct entities remains unclear. Evidence of both their commonality and distinctiveness is presented in Overview of renal findings above.
    • If serial urine samples are obtained in patients with HSP, microscopic hematuria is usually found and is probably present in 100% of the patients. However, frank nephritis appears in only 20-30% of unselected children. The entire clinical spectrum of glomerulonephritis may occur in HSP. The most common renal manifestations are hematuria with mild-to-moderate proteinuria.
  • GI manifestations
    • The duodenum and small intestine are the most frequently involved segments of the GI tract.
    • Duodenal ulcers also occur.
    • Massive GI bleeding has been reported in an adult with HSP.
    • Ileal vasculitis has also been reported.
  • Joint manifestations
    • Arthritis and/or arthralgia affects 60-75% of patients and is the presenting feature in 25%.
    • Joints may be swollen, tender, and painful. The knees and ankles are most commonly affected. On rare occasions, symptoms involve the fingers and wrists.
    • Findings are transient but can occur again during active disease. The joints are not permanently deformed.
  • Cardiac manifestations: Vasculitis involving the myocardia can occur.
  • Pulmonary manifestations: Vasculitis can involve the lungs, resulting in pulmonary hemorrhage or severe bilateral pulmonary hemorrhage.
  • Urinary manifestations: Vasculitis may cause stenosing ureteritis, priapism, penile edema, or orchitis. Ha and Lee (2007) reported that neurologic symptoms, localized edema, and high serum C3 levels show a significant relation with scrotal involvement in male HSP patients.12
  • CNS manifestations: Vasculitis involving the CNS and intracranial hemorrhage has been reported.
  • Eye manifestations: Bilateral subperiosteal orbital hematomas have been noted.
  • Adrenal manifestations: Adrenal hematomas have occurred.
  • Pancreatic manifestations: In rare patients, acute pancreatitis is the sole presenting feature of HSP.

Causes

The following conditions may precede HSP:

More on Henoch-Schönlein Purpura

Overview: Henoch-Schönlein Purpura
Differential Diagnoses & Workup: Henoch-Schönlein Purpura
Treatment & Medication: Henoch-Schönlein Purpura
Follow-up: Henoch-Schönlein Purpura
References
[ CLOSE WINDOW ]

References

  1. Henoch EH. Uber ein eigenthe Form von Purpura. Berl Klin Wochenschr. 1974;11:641-3.

  2. Yilmaz D, Kavakli K, Ozkayin N. The elevated markers of hypercoagulability in children with Henoch-Schonleinpurpura. Pediatr Hematol Oncol. Jan-Feb 2005;22(1):41-8. [Medline].

  3. Aliyazicioglu Y, Ozkaya O, Yakut H, et al. Leptin levels in Henoch-Schonlein purpura. Clin Rheumatol. Mar 2007;26(3):371-5. [Medline].

  4. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. Oct 19 2002;360(9341):1197-202. [Medline].

  5. Haugeberg G, Bie R, Bendvold A, et al. Primary vasculitis in a Norwegian community hospital: a retrospective study. Clin Rheumatol. 1998;17(5):364-8. [Medline].

  6. Bazina M, Glavina-Durdov M, Scukanec-Spoljar M, et al. Epidemiology of renal disease in children in the region of Southern Croatia: A 10-year review of regional renal biopsy databases. Med Sci Monit. Mar 27 2007;13(4):CR172-176. [Medline].

  7. Nong BR, Huang YF, Chuang CM, Liu CC, Hsieh KS. Fifteen-year experience of children with Henoch-Schonlein purpura in southern Taiwan, 1991-2005. J Microbiol Immunol Infect. Aug 2007;40(4):371-6. [Medline].

  8. Suehiro RM, Soares BS, Eisencraft AP, Campos LM, Silva CA. Acute hemorrhagic edema of childhood. Turk J Pediatr. Apr-Jun 2007;49(2):189-92. [Medline].

  9. Pabunruang W, Treepongkaruna S, Tangnararatchakit K, et al. Henoch-Schonlein purpura: clinical manifestations and long-term outcomes in Thai children. J Med Assoc Thai. Nov 2002;85 Suppl 4:S1213-8. [Medline].

  10. Lin SJ, Huang JL. Henoch-Schonlein purpura in Chinese children and adults. Asian Pac J Allergy Immunol. Mar 1998;16(1):21-5. [Medline].

  11. Prais D, Amir J, Nussinovitch M. Recurrent Henoch-Schonlein purpura in children. J Clin Rheumatol. Feb 2007;13(1):25-8. [Medline].

  12. Ha TS, Lee JS. Scrotal involvement in childhood Henoch-Schonlein purpura. Acta Paediatr. Apr 2007;96(4):552-5. [Medline].

  13. Ozkaya O, Bek K, Alaca N, et al. Cerebral vasculitis in a child with Henoch-Schonlein purpura and familial Mediterranean fever. Clin Rheumatol. Oct 2007;26(10):1729-32. [Medline].

  14. Chan KH, Tang WY, Lo KK. Bullous lesions in Henoch-Schonlein purpura. Pediatr Dermatol. May-Jun 2007;24(3):325-6. [Medline].

  15. Szeto CC, Choi PC, To KF, et al. Grading of acute and chronic renal lesions in Henoch-Schönlein purpura. Mod Pathol. Jul 2001;14(7):635-40. [Medline].

  16. Levy M. Familial cases of Berger's disease and anaphylactoid purpura. Kidney Int. Oct 2001;60(4):1611-2. [Medline].

  17. Coppo R, Basolo B, Piccoli G, et al. IgA1 and IgA2 immune complexes in primary IgA nephropathy and Henoch-Schonlein nephritis. Clin Exp Immunol. Sep 1984;57(3):583-90. [Medline].

  18. Davin JC, Malaise M, Foidart J, Mahieu P. Anti-alpha-galactosyl antibodies and immune complexes in children with Henoch-Schonlein purpura or IgA nephropathy. Kidney Int. May 1987;31(5):1132-9. [Medline].

  19. Jennette JC, Wieslander J, Tuttle R, Falk RJ. Serum IgA-fibronectin aggregates in patients with IgA nephropathy and Henoch-Schonlein purpura: diagnostic value and pathogenic implications. The Glomerular Disease Collaborative Network. Am J Kidney Dis. Oct 1991;18(4):466-71. [Medline].

  20. Zhou JH, Huang AX, Liu TL. [A clinico-pathological study comparing Henoch-Schonlein purpura nephritis with IgA nephropathy in children]. Zhonghua Er Ke Za Zhi. Nov 2003;41(11):808-12. [Medline].

  21. Coppo R, Andrulli S, Amore A, et al. Predictors of outcome in Henoch-Schonlein nephritis in children and adults. Am J Kidney Dis. Jun 2006;47(6):993-1003. [Medline].

  22. Prenzel F, Pfaffle R, Thiele F, Schuster V. Decreased factor XIII activity during severe Henoch-Schoenlein purpura -- does it play a role?. Klin Padiatr. May-Jun 2006;218(3):174-6. [Medline].

  23. Nchimi A, Khamis J, Paquot I, Bury F, Magotteaux P. Significance of bowel wall abnormalities at ultrasound in Henoch-Schonlein purpura. J Pediatr Gastroenterol Nutr. Jan 2008;46(1):48-53. [Medline].

  24. Shenoy M, Ognjanovic MV, Coulthard MG. Treating severe Henoch-Schonlein and IgA nephritis with plasmapheresis alone. Pediatr Nephrol. Aug 2007;22(8):1167-71. [Medline].

  25. Zaffanello M, Brugnara M, Franchini M. Therapy for children with henoch-schonlein purpura nephritis: a systematic review. ScientificWorldJournal. 2007;7:20-30. [Medline].

  26. Fagbemi AA, Torrente F, Hilson AJ, Thomson MA, Heuschkel RB, Murch SH. Massive gastrointestinal haemorrhage in isolated intestinal Henoch-Schonlein purpura with response to intravenous immunoglobulin infusion. Eur J Pediatr. Sep 2007;166(9):915-9. [Medline].

  27. Faedda R, Pirisi M, Satta A, et al. Regression of Henoch-Schonlein disease with intensive immunosuppressive treatment. Clin Pharmacol Ther. Nov 1996;60(5):576-81. [Medline].

  28. Iqbal H, Evans A. Dapsone therapy for Henoch-Schonlein purpura: a case series. Arch Dis Child. Sep 2005;90(9):985-6. [Medline].

  29. Abend NS, Licht DJ, Spencer CH. Lupus anticoagulant and thrombosis following Henoch-Schonlein purpura. Pediatr Neurol. May 2007;36(5):345-7. [Medline].

  30. Amitai Y, Gillis D, Wasserman D, Kochman RH. Henoch-Schonlein purpura in infants. Pediatrics. Dec 1993;92(6):865-7. [Medline].

  31. Ansell BM, Falcini F. Cutaneous vasculitis in children. Clin Dermatol. Sep-Oct 1999;17(5):577-80. [Medline].

  32. Cioc AM, Sedmak DD, Nuovo GJ, et al. Parvovirus B19 associated adult Henoch Schonlein purpura. J Cutan Pathol. Nov 2002;29(10):602-7. [Medline].

  33. Dubin BA, Bronson DM, Eng AM. Acute hemorrhagic edema of childhood: an unusual variant of leukocytoclastic vasculitis. J Am Acad Dermatol. Aug 1990;23(2 Pt 2):347-50. [Medline].

  34. Dudley J, Afifi E, Gardner A, et al. Polymorphism of the ACE gene in Henoch-Schonlein purpura nephritis. Pediatr Nephrol. Mar 2000;14(3):218-20. [Medline].

  35. Gandy A. Henoch-Schonlein purpura. Children's Intensive Caring Web site. Available at http://www.intensivecaring.com.

  36. Garcia-Porrua C, Gonzalez-Louzao C, Llorca J, Gonzalez-Gay MA. Predictive factors for renal sequelae in adults with Henoch-Schonlein purpura. J Rheumatol. May 2001;28(5):1019-24. [Medline].

  37. GE Medical Systems. Henoch-schonlein purpura. Medcyclopaedia. Available at http://www.medcyclopaedia.com.

  38. Ha TS, Lee JS. Scrotal involvement in childhood Henoch-Schönlein purpura. Acta Paediatr. Apr 2007;96(4):552-5. [Medline].

  39. Harada T, Machida H, Ito S, Aihara Y, Yokota S. Henoch-Schonlein purpura presenting duodenal involvement similar to superior mesenteric artery syndrome in a girl. Eur J Pediatr. May 2007;166(5):489-90. [Medline].

  40. Kaku Y, Nohara K, Honda S. Renal involvement in Henoch-Schonlein purpura: a multivariate analysis of prognostic factors. Kidney Int. Jun 1998;53(6):1755-9. [Medline].

  41. Kawasaki Y, Suyama K, Matsumoto A, et al. Efficacy of tonsillectomy plus methylprednisolone pulse therapy for a child with Henoch-Schoenlein purpura nephritis. Tohoku J Exp Med. Mar 2007;211(3):291-5. [Medline].

  42. Kawasaki Y, Suzuki J, Murai M, et al. Plasmapheresis therapy for rapidly progressive Henoch-Schonlein nephritis. Pediatr Nephrol. Aug 2004;19(8):920-3. [Medline].

  43. Kim CJ, Chung HY, Kim SY, et al. Acute appendicitis in Henoch-Schonlein purpura: a case report. J Korean Med Sci. Oct 2005;20(5):899-900. [Medline].

  44. Korzets Z, Magen E, Tetro J, et al. [Crescentic glomerulonephritis and cerebral vasculitis in the course of Henoch-Schonlein purpura]. Harefuah. Nov 2002;141(11):960-3, 1010. [Medline].

  45. Koskimies O, Mir S, Rapola J, Vilska J. Henoch-Schonlein nephritis: long-term prognosis of unselected patients. Arch Dis Child. Jun 1981;56(6):482-4. [Medline].

  46. Kraft DM, Mckee D, Scott C. Henoch-Schonlein purpura: a review. Am Fam Physician. Aug 1998;58(2):405-8, 411. [Medline]. [Full Text].

  47. Lau KK, Wyatt RJ, Moldoveanu Z, et al. Serum levels of galactose-deficient IgA in children with IgA nephropathy and Henoch-Schonlein purpura. Pediatr Nephrol. Dec 2007;22(12):2067-72. [Medline].

  48. Legrain V, Lejean S, Taieb A, et al. Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol. Jan 1991;24(1):17-22. [Medline].

  49. Martinez Lopez MM, Rodriguez Arranz C, Pena Carrion A, Merino Munoz R, Garcia-Consuegra Molina J. [Henoch-Schonlein purpura. Study of factors associated with the development and course of the disease]. An Pediatr (Barc). May 2007;66(5):453-8. [Medline].

  50. Millard T, Harris A, MacDonald D. Acute infantile hemorrhagic oedema. J Am Acad Dermatol. Nov 1999;41(5 Pt 2):837-9. [Medline].

  51. Moses S. Henoch-Schonlein purpura. Family Practice Notebook Web site. Available at http://www.fpnotebook.com/HEM33.htm. Accessed 2003.

  52. Nan DN, Fernandez-Ayala M, Garcia-Ibarbia C. Henoch-Schonlein purpura after intravesical administration of bacillus Calmette-Guerin. Scand J Infect Dis. 2005;37(8):613-615. [Medline].

  53. Quercia O, Emiliani F, Foschi FG, Stefanini GF. Unusual reaction to hymenoptera sting: a case of Schonlein-Henoch purpura. Allergy. Mar 2007;62(3):333-4. [Medline].

  54. Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schonlein purpura: a retrospective cohort study. Lancet. Aug 31 2002;360(9334):666-70. [Medline].

  55. Saraclar Y, Tinaztepe K, Adalioglu G, Tuncer A. Acute hemorrhagic edema of infancy (AHEI)--a variant of Henoch-Schonlein purpura or a distinct clinical entity?. J Allergy Clin Immunol. Oct 1990;86(4 Pt 1):473-83. [Medline].

  56. Saulsbury FT. Henoch-Schonlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore). Nov 1999;78(6):395-409. [Medline].

  57. Saulsbury FT. Henoch-Schonlein purpura. Pediatr Dermatol. Jan 1984;1(3):195-201. [Medline].

  58. Shetty AK, Desselle BC, Ey JL, et al. Infantile Henoch-Schonlein purpura. Arch Fam Med. Jun 2000;9(6):553-6. [Medline].

  59. Snow IM. Purpura, urticaria and angioneurotic edema of the hands and feet in a nursing baby. JAMA. 1913;61:18-9.

  60. Soylemezoglu O, Ozkaya O, Erbas D, et al. Nitric oxide in Henoch-Schonlein purpura. Scand J Rheumatol. 2002;31(5):271-4. [Medline].

  61. Sugiyama H, Watanabe N, Onoda T, et al. Successful treatment of progressive Henoch-Schonlein purpura nephritis withtonsillectomy and steroid pulse therapy. Intern Med. Jun 2005;44(6):611-5. [Medline].

  62. Szer IS. Gastrointestinal and renal involvement in vasculitis: management strategies in Henoch-Schonlein purpura. Cleve Clin J Med. May 1999;66(5):312-7. [Medline].

  63. Szer IS. Henoch-Schonlein purpura. Curr Opin Rheumatol. Jan 1994;6(1):25-31. [Medline].

  64. Szer IS. Henoch-Schonlein purpura: when and how to treat. J Rheumatol. Sep 1996;23(9):1661-5. [Medline].

  65. Tizard EJ. Henoch-Schonlein purpura. Arch Dis Child. Apr 1999;80(4):380-3. [Medline].

  66. Vila Cots J, Gimenez Llort A, Camacho Diaz JA, Vila Santandreu A. [Nephropathy in Schonlein-Henoch purpura: a retrospective study of the last 25 years]. An Pediatr (Barc). Mar 2007;66(3):290-3. [Medline].

  67. Watanabe T, Sato Y. Renal involvement and hypocomplementemia in a patient with acute hemorrhagic edema of infancy. Pediatr Nephrol. Nov 2007;22(11):1979-81. [Medline].

  68. Zoch-Zwierz W, Biernacka A, Tomaszewska B, et al. [Vasculitis of cerebral vessels, probable cause of neurological complications in a child with Schonlein-Henoch purpura]. Pol Merkuriusz Lek. Apr 2002;12(70):306-8. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

Henoch Schönlein purpura, Henoch-Schönlein purpura, Henoch Schonlein purpura, Henoch-Schonlein purpura, Henoch Schoenlein purpura, Henoch-Schoenlein purpura, HSP, allergic purpura, Henoch's purpura, Schönlein purpura, Schönlein's purpura, Schönlein disease, Schönlein's disease, Henoch-Schönlein purpura, Henoch-Schönlein syndrome, Schönlein-Henoch syndrome, vascular purpura, acute vascular purpura, anaphylactoid purpura, hemorrhagic exudative erythema, purpura nervosa, purpura rheumatica, rheumatocelis, purpura fulminans, purpura hemorrhagica, nonthrombocytopenic purpura, rheumatoid purpura, allergic purpura

hemorrhagic capillary toxicosis, nonthrombocytopenic idiopathic purpura, peliosis rheumatica, rheumatic purpura, acute hemorrhagic edema of infancy, AHEI, postinfectious cockade purpura, Finkelstein disease, Finkelstein's disease, Seidelmayer syndrome, Seidelmayer's syndrome, infantile postinfectious irislike purpura and edema, vasculitis, arthritis, cutaneous purpura, orchitis, nephritis, Haemophilus, Parainfluenzae, Mycoplasma, Legionella, Yersinia, Shigella, Salmonella species, adenoviruses, Epstein-Barr virus, EBV, parvoviruses, varicella, hypertension, proteinuria, acute hemorrhagic edema of infancy, AHEI, mononucleosis, group A streptococcal infection, hepatitis, varicella-zoster infection

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Optigenex Consulting fee Independent contractor

Coauthor(s)

Elena L Jones, MD, Clinical Assistant Professor of Dermatology, College of Physicians and Surgeons of Columbia University; Clinic Chief, Department of Dermatology, St Luke's-Roosevelt Hospital Center
Disclosure: Nothing to disclose.

Medical Editor

Richard Neiberger, MD, PhD, Director of Pediatric Renal Stone Disease Clinic, Associate Professor, Department of Pediatrics, Division of Nephrology, University of Florida College of Medicine and Shands Hospital
Richard Neiberger, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Medical Association, American Society of Nephrology, American Society of Pediatric Nephrology, Christian Medical & Dental Society, Florida Medical Association, International Society for Peritoneal Dialysis, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Shock Society, Sigma Xi, Southern Medical Association, Southern Society for Pediatric Research, and Southwest Pediatric Nephrology Study Group
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Adrian Spitzer, MD, Professor, Department of Pediatrics, Albert Einstein College of Medicine; Director of NIH Training Program, Children's Hospital at Montefiore Medical Center
Adrian Spitzer, MD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Pediatric Society, American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Howard Trachtman, MD, Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine
Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD, The Isaac A Abt, MD, Professor of Kidney Diseases, Feinberg School of Medicine, Northwestern University; Division Head of Kidney Diseases, Children's Memorial Hospital, Chicago
Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology
Disclosure: Amgen Grant/research funds None; Abbott Honoraria Speaking and teaching; Altus Pharmaceuticals Grant/research funds None; Genzyme Grant/research funds None; Merck Grant/research funds None; NIH Grant/research funds None

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.