SIGNS AND SYMPTOMS 
- General:
- Well-appearing child, despite nature and extent of rash
- Recent or current upper respiratory tract infection
- Malaise
- Low-grade fever
- Hypertension, if associated renal failure
- Children < 3 mo may have only skin manifestations
- Children < 2 yr of age may have facial edema alone as presenting symptom
- Skin:
- Purpuric rash:
- Presenting sign in 50% of patients
- 100% of patients develop purpura
- 1st appears as pink rounded papules that blanch
- Progresses to 2–3 cm circular palpable purpura within 24 hr; may be discrete or confluent
- Rash begins in gravity-dependent areas of legs and buttocks, which may extend to upper extremities and trunk
- Symmetric distribution
- May involve lower back
- Rarely involves the face
- Rash recurs in up to 40% of patients (within 6 wk).
- Abdominal:
- Abdominal pain:
- 70–80% of cases
- Colicky to severe
- Abdominal findings may precede the rash by 4 wk.
- GI bleeding:
- 75% of cases
- Occult to severe blood loss
- Intussusception (ileoileal or ileocolic)
- Renal-genitourinary:
- Asymptomatic hematuria:
- Scrotal pain
- Testicular swelling
- Renal failure
- Extremities:
- Arthritis:
- 70–80% of cases
- Migratory periarticular pain
- Most frequent in knees and ankles
- Angioedema
- Neurologic:
History
- Constitutional symptoms:
- Rash:
- Location, timing, duration, and progression of rash
- Associated symptoms:
- Abdominal pain, vomiting, and seldom facial edema
- Timing, duration, and progression of symptoms
- Progression of symptoms:
- Timing, duration, and progression of symptoms
Physical Exam
- General appearance:
- Level of responsiveness, vital signs (high BP)
- Cardiovascular:
- Quality of heart tones
- Perfusion (pulses, capillary refill)
- GI:
- Abdominal distention, tenderness, palpable masses, bloody stools
- Genitourinary:
- Testicular swelling, tenderness
- Skin:
- Location
- Blanching vs. nonblanching
- Erythematous or purplish raised lesions (papules, purpura) vs. macular lesions (petechia)
- Hemorrhagic bullous evolution seldom described in children
- Neurologic:
- Level of consciousness
- Presence of focal deficits
ESSENTIAL WORKUP
Exclude life-threatening causes of petechia, purpura, severe abdominal pain, hematuria, and CNS findings, if appropriate.
DIAGNOSIS TESTS & INTERPRETATION
Lab
- CBC:
- Platelet count normal
- WBC often elevated
- PT, PTT (if bleeding or in shock; or if unsure of diagnosis and concerned about possibility of coagulopathy)
- Electrolytes (if hypertension or urinalysis abnormal)
- BUN, creatinine (if hypertension or urinalysis abnormal):
- May be elevated in cases with serious renal complications
- Urinalysis:
- Hematuria is common
- Proteinuria is suggestive of glomerulonephritis
- Cultures to exclude common infections
Imaging
- Abdominal imaging studies:
- Indicated if abdominal pain or GI bleeding
- Flat and upright abdominal films of limited use
- Abdominal US, barium enema, or CT scan may be necessary to rule out intussusception.
- Testicular US:
- Indicated in patients with testicular pain and swelling
- Head CT:
- Indicated if CNS findings to exclude bleed
Diagnostic Procedures/Surgery
Lumbar puncture, as clinically indicated
DIFFERENTIAL DIAGNOSIS
- Abdominal pain:
- Arthralgia:
- Rash:
- Renal disease:
- Testicular swelling:
[Outline]
PRE-HOSPITAL
Stabilize as clinically indicated
INITIAL STABILIZATION/THERAPY
- IV fluids for shock
- Packed RBCs for massive GI hemorrhage
ED TREATMENT/PROCEDURES
- Emergent intervention for life-threatening conditions, if any
- NSAIDs (ibuprofen):
- Prednisone:
- Severe abdominal pain once life-threatening pathology excluded
- Painful SC edema or arthritis
- Renal disease (high-dose pulse therapy required with methylprednisolone)
- CNS involvement
- Immunosuppressant drugs:
- Severe, life-threatening disease with HSP nephritis
- Polyclonal immunoglobulin therapy:
- Severe, life-threatening disease (controversial)
MEDICATION
First Line
Ibuprofen: 600 mg (peds: 5–10 mg/kg per dose) PO q6h
Second Line
Prednisone: 60 mg (peds: 1–2 mg/kg/24 h) PO daily for 5–7 days
[Outline]
DISPOSITION
Admission Criteria
- Severe abdominal pain
- CNS findings
- GI bleeding
- Intussusception
- Evidence of renal failure
Discharge Criteria
- Normal platelet count
- Normal renal function
- Minimal or no abdominal pain
- If steroids started, follow up within 24 hr.
Issues for Referral
- GI:
- Renal:
- Evidence of renal failure or insufficiency
FOLLOW-UP RECOMMENDATIONS
Primary care physician:
- Close monitor of BP. Recheck CBC, urinalysis as clinically indicated (recommended for at least 6 mo in children)
[Outline]
- Chang WL, Yang YH, Wang LC, et al. Renal manifestations in Henoch-Schonlein purpura: A 10-year clinical study. Pediatr Nephrol. 2005;20:1269–1272.
- Chartapisak W, Opastiraku S, Hodson EM, et al. Interventions for preventing and treating kidney disease in Henoch Schonlein purpura: A systemic review. Cochrane Database Syst Rev. 2009;(3):CD005128.
- McCarthy HJ, Tizard EJ. Clinical practice: Diagnosis and management of Henoch-Schonlein purpura. Eur J Pediatrics. 2010;169(6):643–650.
- Saulsbury FT. Clinical update: Henoch-Schonlein purpura. Lancet. 2007;369:976–978.
- Trapani S, Mariotti P, Resti M, et al. Severe hemorrhagic bullous lesions in Henoch Schonlein purpura: Three pediatric cases and review of literature. Rheumatol Int. 2010;30(10):1355–1359.
- Zaffanello M, Brugnara M, Franchini M. Therapy for children with Henoch Schonlein purpura nephritis: A systematic review. ScientificWorldJournal. 2007;7:20–30.
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