Henoch-Schonlein purpura

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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

Typical purpura on lower legs and buttocks
More severe case of HSP on child's foot, leg, and arm

Classic

Palpable Purpura

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

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

Disposition

  • Outpatient management for most with rheumatology follow up
  • Outpatient renal consult
  • Recurrence rate of up to 33%

See Also

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.