Henoch-Schonlein purpura

Background

  • Abbreviation: HSP
  • Classical triad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis
  • Most common vasculitis in childhood (usually 2-11 yr)
    • More common in white/asian males
    • Small vessel
    • IgA mediated
    • 5% of cases associated with intussusception (abdominal vasculitis)
    • Most cases preceded by a URI
    • 95% recover completely after 3-4wk
  • Progression to renal insufficiency is feared complication (5-15% in children)[1]
    • Presentation in adults is often worse, with persisting renal insufficiency in 30-50%
    • Adults may require more aggressive treatment with steroids +/- cyclophosphamide though evidence of benefit is lacking

Clinical Features

Classic

Symptoms may develop over the course of days to weeks and vary in order of presentation

  1. Rash: palpable purpura
    • Often begins with erythematous, macular, or urticarial wheals, progressing into the typical ecchymoses, petechiae, and palpable purpura (see picture)
    • Typically appears in crops, symmetrically distributed, and located primarily in gravity/pressure-dependent areas (e.g. lower extremities, buttocks)
  2. Acute abdominal pain
    • Typically diffuse and colicky
    • May have blood in the stool (massive bleeding is rare)
  3. Arthritis/arthralgia
    • Migratory, usually involves knees/ankles
  4. Glomerulonephritis/renal disease

Rare manifestations

  • Melena, hematemesis, hepatosplenomegaly
  • Headache, seizures
  • Fever
  • Non-pitting edema of the extremities and face
  • Nephritic range proteinuria
    • Long-term mortality directly related to renal involvement[3][4]

Differential Diagnosis

Pediatric Abdominal Pain

0–3 Months Old

3 mo–3 y old

3 y old–adolescence

Petechiae/Purpura (by cause)

Causes of Glomerulonephritis

Evaluation

  • Usually a clinical diagnosis rule out other causes
    • No lab test is diagnostic
    • Biopsy of affected organ (eg skin or kidney) with predominantly IgA deposition supports diagnosis
  • CBC (increased WBC and PLT)
  • CRP/ESR (increased)
  • Chemistry
  • PT/PTT (normal)
  • Urinalysis (hematuria, proteinuria)
  • Stool guaiac (positive in >50%)[5] indicates GI vasculitis
  • Consider abdominal ultrasound increased risk of intussusception

Management

  • Supportive
  • NSAIDs for pain, may worsen renal disease or GI disease
  • Consider prednisone 1mg/kg/day for severe arthralgias, abdominal or scrotal disease
  • May require IVIG to prevent and treat glomerulonephritis

Disposition

  • Outpatient management for most with rheumatology follow up
  • Outpatient renal consult
  • Recurrence rate of up to 33%
  • Indications for admission include renal failure, significant GI bleeding, and intussusception. [6]

See Also

  • Pediatric Rashes

References

  1. Pillebout E et al. Henoch-Schönlein Purpura in Adults: Outcome and Prognostic Factors. JASN May 1, 2002 vol. 13 no. 5 1271-1278.
  2. Chen JY et al. Henoch-Schönlein purpura nephritis in children: incidence, pathogenesis and management. World J Pediatr. 2015 Feb;11(1):29-34. doi: 10.1007/s12519-014-0534-5. Epub 2014 Dec 29.
  3. Calviño, MC, Llorca, J, García-Porrúa, C, Fernández-Iglesias, JL, Rodriguez-Ledo, P, González-Gay, MA (2001) Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine (Baltimore) 80: pp. 279-290
  4. Saulsbury, FT (1999) Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 78: pp. 395-409
  5. Chang WL, Yang YH, Lin YT, Chiang BL. "Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of 261 patients." Acta Paediatr. 2004;93(11):1427.
  6. Core EM Henoch-Schoenlein Purpura https://coreem.net/core/henoch-schoenlein-purpura/
This article is issued from Wikem. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.