Henoch-Schonlein purpura: Difference between revisions
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==Background==
==Background==
-HSP, aka anaphylactoid purpura
{{Skin anatomy background images}}
*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)<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


-systemic vasculitis associated with abdominal pain and rash
{{Primary Vasculitis DDX}}


-usually age 4-11, may occur in adults
==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]]


-commonly occurs in springtime after viral URI
===Classic===
[[File:HSPVasc01.jpg| thumb|Palpable Purpura]]
''Symptoms may develop over the course of days to weeks and vary in order of presentation''
#[[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)
#Acute [[Abdominal pain (peds)|abdominal pain]]
#*Typically diffuse and colicky
#*May have blood in the stool (massive bleeding is rare)
#[[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]]


==Diagnosis==
===Rare manifestations===
-clinical diagnosis based on presence of abdominal pain, classic rash, microscopic hematuria, arthralgias in otherwise well appearing child
*[[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>


-symptoms: abdominal pain, nausea, vomiting, diarrhea, rash, mild arthralgias
==Differential Diagnosis==
{{Pediatric abdominal pain DDX}}


-rash: palpable purpura on buttocks and lower extremities
{{Purpura DDX}}


-intussusception: ileoileal more common than ileocolic inpatients with HSP
{{Glomerulonephritis causes}}


==Work-Up==
==Evaluation==
#CBC, Chem7
*Usually a clinical diagnosis &mdash; rule out other causes
#UA
**No lab test is diagnostic
#Blood cx
**Biopsy of affected organ (eg skin or kidney) with predominantly IgA deposition supports diagnosis
#ESR
*CBC (increased WBC and PLT)
#consider CT abdomen (ileoileal intussusception difficult to detect by US)
*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]]


==DDx==
==Management==
#meningococcemia (febrile, ill appearing)
*Supportive
#erythema nodosum
*[[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]]


==Treatment==
==Disposition==
*dz self limited: 94% recover completely
*Outpatient management for most with rheumatology follow up
*consider corticosteroids for severe disease (abdominal pain, hematuria, arthralgias)
*Outpatient renal consult
**prednisone 1mg/kg/day
*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>
 
 
==Medication Dosing==
{{MedicationDose
| drug = Prednisone
| dose = 1mg/kg/day
| route = PO
| context = Severe arthralgias, abdominal or scrotal disease
| indication = Henoch-Schonlein purpura
| population = Pediatric
}}


==Disposition==
==See Also==
*most patients managed safely as outpatient
*[[Pediatric Rashes]]
*consider admission for:
**uncertain diagnosis to exclude possibility of meningococcemia
**severe abdominal pain and vomiting


==Source==
==References==
Adapted from Rosens 7th edition
<references/>


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

Latest revision as of 18:43, 20 March 2026

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]


Medication Dosing

Prednisone 1mg/kg/day PO

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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