Ver el código de «Henoch-Schonlein purpura»

==Background==
{{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

{{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]]

===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]]

===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==
{{Pediatric abdominal pain DDX}}

{{Purpura DDX}}

{{Glomerulonephritis causes}}

==Evaluation==
*Usually a clinical diagnosis &mdash; 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%)<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]]

==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. <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
}}

==See Also==
*[[Pediatric Rashes]]

==References==
<references/>

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