Henoch-Schonlein purpura: Difference between revisions
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==Background==
==Background==
*Most common vasculitis in childhood
*Abbreviation: HSP
*Most cases preceded by a URI
*Classical triad of non-thrombocytopenic palpable [[purpura]], [[abdominal pain]], [[arthritis]]
*Usually affects 2-11yr
*Most common [[vasculitis]] in childhood (usually 2-11 yr)
*5% of cases are a/w intussusception (abd vasculitis)
**More common in white/asian males
*Renal involvement is feared complication
**Small vessel
*95% recover completely after 3-4wk
**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)<ref>Pillebout E et al. Henoch-Schönlein Purpura in Adults: Outcome and Prognostic Factors. JASN May 1, 2002 vol. 13 no. 5 1271-1278.</ref>
**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


==Diagnosis==
==Clinical Features==
*Tetrad:
[[File:Henoch-schonlein-purpura.jpg|thumb|Typical purpura on lower legs and buttocks]]
**Palpable purpura (extremities, buttock)
[[File:Purpura2.jpg|thumb|More severe case of HSP on child's foot, leg, and arm]]
**Acute abdominal pain (diffuse, colicky)
 
***Usually develops after onset of rash
===Classic===
**Arthritis
[[File:HSPVasc01.jpg| thumb|Palpable Purpura]]
***Migratory, usually involves knees/ankles
''Symptoms may develop over the course of days to weeks and vary in order of presentation''
**Renal disease (50% of the time)
#[[Rash]]: palpable purpura
[[File:HSPVasc01.jpg|center|frame|500px|Palpable Purpura]]
#*Often begins with erythematous, macular, or urticarial wheals, progressing into the typical ecchymoses, petechiae, and palpable purpura (see picture)
*Rare manifestations  
#*Typically appears in crops, symmetrically distributed, and located primarily in gravity/pressure-dependent areas (e.g. lower extremities, buttocks)
** Melena, hematemesis, hepatosplenomegaly
#Acute [[Abdominal pain (peds)|abdominal pain]]
** Headache, seizures
#*Typically diffuse and colicky
** Fever
#*May have blood in the stool (massive bleeding is rare)
** Non-pitting edema of the extremities and face
#[[Arthritis]]/arthralgia
#*Migratory, usually involves knees/ankles
#[[Glomerulonephritis]]/renal disease
#*(~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>
#*[[Hematuria]] +/- [[proteinuria]]
 
===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<ref>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 </ref><ref>Saulsbury, FT (1999) Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 78: pp. 395-409 </ref>


==Differential Diagnosis==
==Differential Diagnosis==
*[[Erythema nodosum]]
{{Pediatric abdominal pain DDX}}
*[[Intussusception]]
*[[Rheumatic fever]]
*[[Polyarteritis nodosa]]
*[[SLE]]
*[[Rheumatoid arthritis]]
*[[Drug reaction]]


{{Peds Rash DDX}}
{{Purpura DDX}}


{{Glomerulonephritis causes}}
{{Glomerulonephritis causes}}


==Work-Up==
==Evaluation==
#UA
*Usually a clinical diagnosis &mdash; rule out other causes
##Hematuria, proteinuria
**No lab test is diagnostic
#Chemistry
**Biopsy of affected organ (eg skin or kidney) with predominantly IgA deposition supports diagnosis
#Consider stool guaiac if concern for melena
*CBC (increased WBC and PLT)
*CRP/ESR (increased)
*Chemistry
*PT/PTT (normal)
*[[Urinalysis]] ([[hematuria]], proteinuria)
*Stool guaiac (positive in >50%)<ref>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.</ref> &mdash; indicates GI vasculitis
*Consider abdominal ultrasound &mdash; increased risk of [[intussusception]]


==Treatment==
==Management==
*Supportive
*Supportive
*NSAIDs for pain, may worsen renal disease or GI disease
*[[NSAIDs]] for pain, may worsen renal disease or GI disease
*consider prednisone 1mg/kg/day for severe arthralgias, abdominal or scrotal disease
*Consider [[prednisone]] 1mg/kg/day for severe arthralgias, abdominal or scrotal disease
*May require [[IVIG]] to prevent and treat [[glomerulonephritis]]


==Disposition==
==Disposition==
*Outpt management for most w/ rheum f/u
*Outpatient management for most with rheumatology follow up
*Outpatient renal consult
*Recurrence rate of up to 33%
*Recurrence rate of up to 33%
*Indications for admission include renal failure, significant GI bleeding, and intussusception. <ref>Core EM Henoch-Schoenlein Purpura https://coreem.net/core/henoch-schoenlein-purpura/</ref>


==See Also==
==See Also==
[[Pediatric Rashes]]
*[[Pediatric Rashes]]
 
==References==
<references/>


==Source==
[[Category:Dermatology]]
*Rosen's, Tintinalli
[[Category:Pediatrics]]
*Images provided by University of Iowa Dept. of Dermatology
*First Aid for the Emergency Medicine Boards
[[Category:Derm]]
[[Category:Peds]]

Revision as of 18:37, 22 October 2020

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
  • 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

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/
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