Henoch-Schonlein purpura: Difference between revisions

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==Clinical Presentation==
==Clinical Presentation==
===Tetrad ("Classic")===
===Classic===
*Palpable purpura bilaterally (extremities, buttock)<ref>Image obtained from University of Iowa Dept. of Dermatology</ref>[[File:HSPVasc01.jpg| thumb|Palpable Purpura]]
[[File:HSPVasc01.jpg| thumb|Palpable Purpura]]
*Acute abdominal pain (diffuse, colicky)
''May develop over the course of days to weeks and may vary in their order of presentation''
**Usually develops after onset of rash
#Rash: palpable purpura
*Arthritis
#*Often begins with erythematous, macular, or urticarial wheals, progressing into the typical ecchymoses, petechiae, and palpable purpura (see picture)
**Migratory, usually involves knees/ankles
#*Typically appears in crops, symmetrically distributed, and located primarily in gravity/pressure-dependent areas (e.g. lower extremities, buttocks)
#Acute abdominal pain  
#*Typically diffuse and colicky
#*May have blood in the stool (massive bleeding is rare)
#Arthritis/arthralgia
#*Migratory, usually involves knees/ankles
*Nephritis (>50% of the time)<ref>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.</ref>
*Nephritis (>50% of the time)<ref>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.</ref>
#Renal disease
#*Hematuria (~1/3 of cases)


===Rare manifestations===
===Rare manifestations===

Revision as of 17:58, 27 October 2015

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

Classic

Palpable Purpura

May develop over the course of days to weeks and may vary in their 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
  • Nephritis (>50% of the time)[1]
  1. Renal disease
    • Hematuria (~1/3 of cases)

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[2][3]

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

References

  1. 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.
  2. 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
  3. Saulsbury, FT (1999) Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 78: pp. 395-409