Henoch-Schonlein purpura: Difference between revisions

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==Background==
==Background==
*Classical triad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis
{{Skin anatomy background images}}
*Most common vasculitis in childhood (usually 2-11 yr)
*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
**Small vessel
**IgA mediated
**IgA mediated
**5% of cases associated with intussusception (abdominal vasculitis)
**5% of cases associated with [[intussusception]] (abdominal vasculitis)
**Most cases preceded by a URI
**Most cases preceded by a [[URI]]
**95% recover completely after 3-4wk
**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>
*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%
**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
**Adults may require more aggressive treatment with [[steroids]] +/- [[cyclophosphamide]] though evidence of benefit is lacking
 
{{Primary Vasculitis DDX}}
 
==Clinical Features==
[[File:Henoch-schonlein-purpura.jpg|thumb|Typical purpura on lower legs and buttocks]]
[[File:Purpura2.jpg|thumb|More severe case of HSP on child's foot, leg, and arm]]


==Clinical Presentation==
===Classic===
===Classic===
[[File:HSPVasc01.jpg| thumb|Palpable Purpura]]
[[File:HSPVasc01.jpg| thumb|Palpable Purpura]]
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#[[Arthritis]]/arthralgia
#[[Arthritis]]/arthralgia
#*Migratory, usually involves knees/ankles
#*Migratory, usually involves knees/ankles
#[[Nephritis]]/renal disease
#[[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>
#*(~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
#*[[Hematuria]] +/- [[proteinuria]]


===Rare manifestations===
===Rare manifestations===
* [[Melena]], [[hematemesis]], hepatosplenomegaly
*[[Melena]], [[hematemesis]], hepatosplenomegaly
* [[Headache]], [[seizures]]
*[[Headache]], [[seizures]]
* [[Fever]]
*[[Fever]]
* Non-pitting edema of the extremities and face
*Non-pitting edema of the extremities and face
*[[Nephrotic syndrome]]
*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>
**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>


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


==Diagnosis==
==Evaluation==
===Work-Up===
*Usually a clinical diagnosis &mdash; rule out other causes
*CBC (normal)
**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  
*Chemistry  
*PT/PTT (normal)
*PT/PTT (normal)
*UA ([[hematuria]], proteinuria)
*[[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>
*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 if concerns for [[intussusception]] (at higher risk)
*Consider abdominal ultrasound &mdash; increased risk of [[intussusception]]
 
===Evaluation===
*Usually a clinical diagnosis
**Rule out other possible causes (see differential diagnosis)
*In patients with an incomplete or unusual presentation, no lab test is diagnostic but biopsy of the affected organ (e.g. skin or kidney) with predominantly immunoglobulin A (IgA) deposition supports diagnosis


==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 with rheumatology follow up
*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==
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<references/>
<references/>


 
[[Category:Dermatology]]
[[Category:Derm]]
[[Category:Pediatrics]]
[[Category:Peds]]

Latest revision as of 16:26, 11 December 2024

Background

Normal dermal anatomy.
  • 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

Vasculitis Syndrome Types

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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