AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Background

Henoch-Schönlein purpura (HSP) is an inflammatory disorder characterized by a generalized vasculitis involving the small vessels of the skin, GI tract, kidneys, joints, and, rarely, the lungs and CNS. It is the most common vasculitis in children.

The syndrome takes its name from 2 German physicians. In 1837, Johan Schönlein first described several cases of peliosis rheumatica or purpura associated with arthritis. Thirty years later, Edouard Henoch described the GI manifestations, including vomiting, abdominal pain, and melena. HSP has also been referred to as rheumatica purpura, leukocytoclastic vasculitis, and allergic vasculitis.

Pathophysiology

The etiology of HSP is unclear. It is thought to be multifactorial with genetic, environmental, and antigenic components. More than 75% of patients report antecedent upper-respiratory, pharyngeal, or GI infections. Multiple bacterial and viral infectious agents have been associated with the development of HSP, and cases of HSP also have been reported after drug ingestions and vaccinations.

HSP is thought to be an immunoglobulin A (IgA)–mediated autoimmune phenomenon. An unknown antigenic stimulant has been postulated to cause a rise in IgA. The antigen-antibody complexes deposit locally throughout the body and activate pathways leading to necrotizing vasculitis.

Genetic research may reveal the potential role of cytokines, endothelia and nitric oxide metabolism in HSP.

HSP can involve nearly any organ system. Hallmarks of HSP include a characteristic rash, migratory polyarthritis, renal involvement, and GI involvement. The clinical manifestations of HSP are the result of antigen-antibody complexes depositing throughout the body, which cause migratory arthralgias, abdominal cramping, the petechial and/or vasculitic rash, and hematuria.

Frequency

United States

The rate is 14 cases per 100,000 population.

Mortality/Morbidity

HSP generally resolves without permanent complications. However, serious GI and renal complications may occur.

• GI complications include intussusception (usually ileoileal), bowel infarction, bowel perforation, hydrops of the gallbladder, pancreatitis, or massive GI bleeding.
• Approximately 20% of patients have renal manifestations, and 5% develop end-stage renal disease (ESRD). Patients with only hematuria do not develop ESRD. About 15% of patients with hematuria and proteinuria develop ESRD. Approximately 50% of patients with nephritic or nephrotic syndrome develop ESRD.
• The long-term morbidity is predominantly attributed to renal involvement.

Sex

• In children, the male-to-female ratio is 2:1.
• In adults, the male-to-female ratio is approximately 1:1.

Age

• HSP primarily affects children.
• Adults are rarely affected.
• Approximately 75% of cases occur in children aged 2-11 years. The median age is 5 years.
• Older age at disease onset is associated with development of chronic renal disease.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

History

• Prodrome
• • Headache
  • Anorexia
  • Fever
• After the prodrome, a rash, abdominal pain, peripheral edema, vomiting and/or arthritis develop.
• • The rash appears in 100% of patients and is the presenting feature in 50%.
  • The distribution is usually on dependent parts of the body including the lower trunk, lower extremities, buttocks and perineum.
  • The rash typically appears in crops with new crops appearing in waves.
  • Eruptions usually last an average of 3 weeks.
  • As many as 85% of patients will have GI symptoms, including abdominal pain, nausea, and vomiting.
  • The most common symptom is colicky abdominal pain.
  • Joint involvement is present in 75% of reported patients with HSP and the presenting sign in approximately 25%.
  • The large joints (eg, knees and ankles) are most commonly involved, with pain and edema being the only symptoms. The arthritis resolves completely over several days without permanent articular damage.
  • Renal involvement is present in 30-50% of patients and may persist as long as 6 months after the onset of the rash.
  • Renal involvement manifests in a range from mild hematuria or proteinuria to oliguria and renal failure.
  • Permanent renal impairment is seen in 20% of patients who have nephrotic or nephritic syndrome; however, this turns out to be less than 0.1 % of all patients diagnosed with HSP.

Physical

• Skin
• • Lesions consist of erythematous macules, urticarial papules, pruritic papules, and plaques. Skin lesions tend to appear in crops in the dependent portions of the body (eg, lower extremities, lower abdomen, buttocks).
  • Children younger than 2 years also may have involvement of the upper extremity, head, and trunk.
  • The rash typically appears as red macules and papules, which later become purple and then rust-colored.
  • Various stages of eruption are usually present simultaneously. The lesions may blanch initially, but they progress to palpable purpura as they mature.
• Abdomen
• • Heme-positive stool is the primary finding on GI examination.
  • Findings on abdominal examination are generally unremarkable.
  • On occasion, the abdomen is tender.
  • Signs of an acute abdomen are rarely present.
• Joints
• • The knees, ankles, and (less commonly) wrists are involved.
  • Tenderness and edema are periarticular. Warmth, erythema, and effusions are not typically associated with HSP.
• Other
• • Case reports describe patients with HSP presenting with protein-losing enteropathy without liver or kidney dysfunction.
  • The rash may appear late in the course, simplifying the diagnosis.

Causes

The current understanding of the etiology of HSP suggests the involvement of toxins, viruses, idiopathic causes, and drugs. No single etiology has been clearly identified; however, most cases are preceded by a recent upper airway infection.

• Infectious agents associated with HSP
• • Streptococcus species (especially group A)
  • Yersinia species
  • Legionella species
  • Parvovirus
  • Adenovirus
  • Mycoplasma species
  • Epstein-Barr virus
  • Varicella
• Drugs associated with HSP
• • Penicillin
  • Ampicillin
  • Erythromycin
  • Quinidine
  • Quinine
• Vaccines associated with HSP
• • Typhoid and paratyphoid A and B
  • Measles
  • Yellow fever
  • Cholera

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Lab Studies

• Electrolyte values are generally in the reference range, but excessive vomiting can affect the values.
• BUN and creatinine levels may be increased in the presence of renal involvement.
• Amylase and lipase levels may be elevated in patients with pancreatitis.
• A CBC usually reveals a leukocytosis with a left shift, possibly eosinophilia, and a normal or increased platelet count. Hemoglobin and/or hematocrit values may be normal or decreased secondary to bleeding.
• Urinalysis usually shows hematuria, proteinuria, and occasional red cell casts.
• Additional laboratory tests that can be helpful in narrowing the differential diagnosis include the following:
• • Assessment of antistreptolysin-O (ASO) titer
  • Monospot test
  • Antinuclear antibody (ANA) test
  • Rheumatoid factor (RF) test
  • Determination of C3/C4 levels
  • Measurement of the prothrombin time (PT)
  • Measurement of the activated partial thromboplastin time (aPTT)
  • Blood cultures

Imaging Studies

• Imaging studies are necessary only as the clinical picture dictates.
• CT scanning may aid in the exclusion of other causes of abdominal pain.
• Barium enema study or endoscopy might be needed to evaluate epigastric pain, hematemesis, and melena.
• Ultrasonography may be helpful for evaluating intussusception and to exclude appendicitis.
• A chest radiograph should be obtained after hemoptysis, and a head CT is necessary if neurologic symptoms or severe headache persist.
• Imaging of the scrotum by means of ultrasonography or a technetium radionuclide scanning may be necessary if scrotal edema is a presenting feature.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Emergency Department Care

• ED treatment is supportive, with frequent monitoring of vital signs. For minor complaints of arthritis, edema, fever or malaise, symptomatic treatment is advised, including use of acetaminophen, elevation of swollen extremities, eating a bland diet, and adequate hydration.
• Most patients with self-limited cases can be safely discharged home with close follow-up by their primary physician. Whether or not to admit the patient to the hospital depends on the practice of the admitting pediatrician and his or her preference. Admission to the hospital is recommended for control of abdominal pain or vomiting, monitoring of renal function, confirming a doubted diagnosis, and observation and monitoring.
• All unnecessary drugs should be discontinued if the etiology is suspected to be drug related.
• Patients with renal involvement require close attention in regard to their fluid balance, electrolyte status, and use of antihypertensives (if indicated).
• • Use of immunosuppressive and cytotoxic drugs is gaining favor based on research and case studies.
  • Dapsone has been used to treat associated purpura and arthralgias.
  • Factor VIII concentrate has been used to relieve abdominal pain when corticosteroids are contraindicated.
  • Plasmapheresis is currently under investigation.
  • Kidney transplantation may be indicated in patients with severe renal disease that is resistant to medical therapy.
• Surgery may be undertaken to treat severe bowel ischemia.

Consultations

If the patient has renal involvement, a nephrologist should be consulted for assistance in determining if dialysis is indicated. Because 1 in 20 patients with HSP develop renal failure, early consultation is desirable.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Treatment is largely supportive. Analgesia with nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen may reduce joint and soft tissue discomfort. The evidence does not clearly demonstrate that corticosteroid administration prevents the development of nephritis in patients with HSP, although its use in the treatment of intestinal and neurologic complications is gaining acceptance. If used, prednisone 1-2 mg/kg/d PO for 7 days is recommended. Antihypertensives may be indicated with renal involvement; for more information, see the pediatric topic Hypertension. Other drugs are currently under investigation (see Emergency Department Care). For more information on long-term medication management, see the pediatric general medicine topic Henoch-Schönlein Purpura.

Drug Category: Analgesic agents

Pain control is essential for quality patient care. Some analgesics (eg, acetaminophen, ibuprofen) also are effective for treating fever.

Drug Category: Glucocorticoids

Short-term use may be considered to decrease inflammation during neurologic or intestinal complications.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Further Inpatient Care

• Patients with HSP have the potential for severe complications, which may occur precipitously (eg, acute abdomen, acute scrotum, renal failure).
• Whether or not to admit the patient to the hospital for observation and monitoring depends on the practice of the admitting pediatrician and his or her preference.

Further Outpatient Care

• In all patients, urinalysis evaluation for renal involvement should be continued for up to 6 months after presentation, even if initial urinalysis results are normal.

Complications

• HSP can involve nearly every organ system
• GI complications include hydrops of the gallbladder, pancreatitis, and GI bleeding.
• Surgical complications include intussusception, bowel infarction, and perforation.
• Overall, 5% of patients develop ESRD.
• Other potential complications include the following:
• • Coronary artery vasculitis resulting in myocardial infarction (MI)
  • Headache
  • Irritability
  • Fever
  • Pulmonary hemorrhage
  • CNS bleeding
  • Scrotal edema
  • Pain

Prognosis

• About 50% of patients have at least 1 recurrence.
• Young patients (<3 y) usually have an improved prognosis.
• The prognosis is best for patients with minimal or no renal involvement at the onset of the illness.

MULTIMEDIA

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Media file 1:  A 9-year-old boy with Henoch-Schönlein purpura. Note confluence of purpura around the ankles. Courtesy of Pamela L Dyne, MD.
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Media type:  Photo

Media file 2:  A 7-year-old girl with Henoch-Schönlein purpura. Courtesy of Pamela L Dyne, MD.
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Media type:  Photo

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

• Chang WL, Yang YH, Wang LC, et al. Renal manifestations in Henoch-Schönlein purpura: a 10-year clinical study. Pediatr Nephrol. Sep 2005;20(9):1269-72. [Medline].
• Gonzalez-Gay MA, Garcia-Porrua C, Pujol RM. Clinical approach to cutaneous vasculitis. Curr Opin Rheumatol. Jan 2005;17(1):56-61. [Medline].
• Lanzkowsky S, Lanzkowsky L, Lanzkowsky P. Henoch-Schoenlein purpura. Pediatr Rev. Apr 1992;13(4):130-7. [Medline].
• Martin J, Paco L, Ruiz MP, et al. Inducible nitric oxide synthase polymorphism is associated with susceptibility to Henoch-Schönlein purpura in northwestern Spain. J Rheumatol. Jun 2005;32(6):1081-5. [Medline].
• Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child. Sep 2005;90(9):916-20. [Medline].
• Tapson KM. Henoch-Schonlein purpura. Am Fam Physician. Feb 15 1993;47(3):633-8. [Medline].
• Ting TV, Hashkes PJ. Update on childhood vasculitides. Curr Opin Rheumatol. Sep 2004;16(5):560-5. [Medline].
• Tintinalli JE, Kelen GD, Stapczynski JS. Henoch Schonlein purpura. In: Emergency Medicine: A Comprehensive Study Guide. 6^th ed. 2004:886.
• Trujillo H, Gunasekaran TS, Eisenberg GM, et al. Henoch-Schonlein purpura: a diagnosis not to be forgotten. J Fam Pract. Nov 1996;43(5):495-8. [Medline].
• Urbach AM, Londino AV. Rheumatology. In: Atlas of Pediatric Physical Diagnosis. 3^rd ed. 1997:203-4.
• Urbach AM, Londino AV. Dermatology. In: Atlas of Pediatric Physical Diagnosis. 3^rd ed. 1997:236-7.
• Yigiter M, Bosnali O, Sekmenli T, et al. Multiple and recurrent intestinal perforations: an unusual complication of Henoch-Schonlein purpura. Eur J Pediatr Surg. Apr 2005;15(2):125-7. [Medline].

Article Last Updated: Dec 20, 2007