Henoch-Schonlein purpura

Background

  • Most common vasculitis in childhood
    • Small vessel
  • Most cases preceded by a URI
  • Usually affects 2-11 yr
  • 5% of cases associated with intussusception (abdominal vasculitis)
  • Renal involvement is feared complication
  • 95% recover completely after 3-4wk

Clinical Presentation

  • Tetrad:
    • Palpable purpura bilaterally (extremities, buttock)[1]
      Palpable Purpura
    • Acute abdominal pain (diffuse, colicky)
      • Usually develops after onset of rash
    • Arthritis
      • Migratory, usually involves knees/ankles
    • Nephritis (>50% of the time)[2]
  • Rare manifestations
    • Melena, hematemesis, hepatosplenomegaly
    • Headache, seizures
    • Fever
    • Non-pitting edema of the extremities and face
    • Nephrotic Syndrome
      • Long-term mortality directly related to renal involvement[3][4]

Differential Diagnosis

Pediatric Rash

Causes of Glomerulonephritis

Diagnosis

Work-Up

  1. UA
    • Hematuria, proteinuria
  2. Chemistry
  3. Consider stool guaiac if concern for melena

Treatment

  • Supportive
  • NSAIDs for pain, may worsen renal disease or GI disease
  • Consider prednisone 1mg/kg/day for severe arthralgias, abdominal or scrotal disease

Disposition

  • Outpt management for most w/ rheum f/u
  • Recurrence rate of up to 33%

See Also

Pediatric Rashes

References

  1. Image obtained from University of Iowa Dept. of Dermatology
  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