Pediatric Henoch-Schonlein Purpura Clinical Presentation

  • Author: Pamela L Dyne, MD; Chief Editor: Richard G Bachur, MD   more...
 
Updated: Jan 4, 2011
 

History

The following may be noted in the history of patients with Henoch-Schönlein purpura (HSP):

  • The prodrome is associated with the following:
    • 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 usually depends on 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 Henoch-Schönlein purpura 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 Henoch-Schönlein purpura.
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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 Henoch-Schönlein purpura.
  • Other
    • Case reports describe patients with Henoch-Schönlein purpura presenting with protein-losing enteropathy without liver or kidney dysfunction.
    • The rash may appear late in the course, simplifying the diagnosis.
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Causes

The current understanding of the etiology of Henoch-Schönlein purpura 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.

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Contributor Information and Disclosures
Author

Pamela L Dyne, MD  Professor of Clinical Medicine/Emergency Medicine, David Geffen School of Medicine at UCLA; Attending Physician, Department of Emergency Medicine, Olive View-UCLA Medical Center

Pamela L Dyne, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Stacy Sawtelle, MD  Clinical Instructor, Department of Emergency Medicine, University of California, San Francisco, School of Medicine

Disclosure: Nothing to disclose.

Heather Kesler DeVore, MD  Clinical Attending Physician, Assistant Professor Physician, Department of Emergency Medicine, Washington Hospital Center/Georgetown University Hospital

Heather Kesler DeVore, MD is a member of the following medical societies: Emergency Medicine Residents Association and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Debra Slapper, MD  Consulting Staff, Department of Emergency Medicine, St Anthony's Hospital

Debra Slapper, MD is a member of the following medical societies: American Academy of Emergency Medicine

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Wayne Wolfram, MD, MPH  Associate Professor, Department of Emergency Medicine, Mercy St Vincent Medical Center

Wayne Wolfram, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Pediatrics, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

John D Halamka, MD, MS  Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Richard G Bachur, MD  Associate Professor of Pediatrics, Harvard Medical School; Associate Chief and Fellowship Director, Attending Physician, Division of Emergency Medicine, Children's Hospital of Boston

Richard G Bachur, MD is a member of the following medical societies: American Academy of Pediatrics, Society for Academic Emergency Medicine, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

  1. [Best Evidence] Chartapisak W, Opastiraku S, Willis NS, Craig JC, Hodson EM. Prevention and treatment of renal disease in Henoch-Schonlein purpura: a systematic review. Arch Dis Child. Feb 2009;94(2):132-7. [Medline].

  2. 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].

  3. McCarthy HJ, Tizard EJ. Clinical practice: Diagnosis and management of Henoch-Schönlein purpura. Eur J Pediatr. Jun 2010;169(6):643-50. [Medline].

  4. Chang WL, Yang YH, Wang LC, et al. Renal manifestations in Henoch-Schonlein purpura: a 10-year clinical study. Pediatr Nephrol. Sep 2005;20(9):1269-72. [Medline].

  5. Gonzalez-Gay MA, Garcia-Porrua C, Pujol RM. Clinical approach to cutaneous vasculitis. Curr Opin Rheumatol. Jan 2005;17(1):56-61. [Medline].

  6. Lanzkowsky S, Lanzkowsky L, Lanzkowsky P. Henoch-Schoenlein purpura. Pediatr Rev. Apr 1992;13(4):130-7. [Medline].

  7. Martin J, Paco L, Ruiz MP, et al. Inducible nitric oxide synthase polymorphism is associated with susceptibility to Henoch-Schonlein purpura in northwestern Spain. J Rheumatol. Jun 2005;32(6):1081-5. [Medline].

  8. Tapson KM. Henoch-Schonlein purpura. Am Fam Physician. Feb 15 1993;47(3):633-8. [Medline].

  9. Ting TV, Hashkes PJ. Update on childhood vasculitides. Curr Opin Rheumatol. Sep 2004;16(5):560-5. [Medline].

  10. Tintinalli JE, Kelen GD, Stapczynski JS. Henoch Schonlein purpura. In: Emergency Medicine: A Comprehensive Study Guide. 6th ed. 2004:886.

  11. 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].

  12. Urbach AM, Londino AV. Rheumatology. In: Atlas of Pediatric Physical Diagnosis. 3rd ed. 1997:203-4.

  13. Urbach AM, Londino AV. Dermatology. In: Atlas of Pediatric Physical Diagnosis. 3rd ed. 1997:236-7.

  14. 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].

 
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A 9-year-old boy with Henoch-Schönlein purpura. Note confluence of purpura around the ankles. Courtesy of Pamela L Dyne, MD.
A 7-year-old girl with Henoch-Schönlein purpura. Courtesy of Pamela L Dyne, MD.
 
 
 
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