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
- Typically, the last sign to resolve
- First 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, such as legs and buttocks, which may extend to upper extremities and trunk
- Symmetric distribution
- May involve lower back, scrotum
- 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 in 30-40%
- 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 presenting symptom in 15-25%
- 70-80% of cases
- Migratory periarticular pain
- Most frequent in knees and ankles
- Angioedema
- Neurologic:
- Headache
- Seizure
- Altered mental status (can also see with intussusception)
- Focal deficits ± visual abnormalities and verbal disability
History
- Constitutional symptoms:
- Rash:
- Location, timing, duration, and progression of rash
- Associated symptoms:
- Abdominal pain ± cyclical in nature, vomiting, arthralgias, hematuria, and rare facial/scalp/hand /foot edema
- Timing, duration, and progression of symptoms
Physical Exam
- General appearance:
- Level of responsiveness, vital signs (assess for high BP, establish baseline)
- Cardiovascular:
- Quality of heart tones
- Perfusion (pulses, capillary refill)
- GI:
- Abdominal distention, tenderness, palpable masses, bloody stools
- Genitourinary:
- Testicular swelling, tenderness
- Skin: Rash
- Location
- Blanching vs. nonblanching
- Erythematous or purplish raised lesions (papules, purpura) vs. macular lesions (petechiae)
- Hemorrhagic bullous evolution seldom described in children, case reports of Koebner phenomenon
- Neurologic:
- Level of consciousness
- Presence of focal deficits
Essential Workup
Exclude life-threatening causes of petechiae, purpura, severe abdominal pain, hematuria, and CNS findings
Diagnostic Tests & Interpretation
Lab
- CBC:
- WBC and platelet counts normal or elevated
- PT, PTT (if bleeding or in shock; or if unsure of diagnosis and concerned about possibility of coagulopathy)
- ASO
- Electrolytes (if hypertension or urinalysis abnormal)
- BUN, creatinine (if hypertension or urinalysis abnormal):
- May be elevated in cases with serious renal complications
- Urinalysis may be the single most important lab:
- Hematuria is common
- Proteinuria is suggestive of glomerulonephritis
- Cultures to exclude common infections
- The following labs may help to differentiate from similar disease states: Serum ANA, ds-DNA, ANCA, C3/C4
Imaging
- Abdominal imaging studies:
- Indicated if abdominal pain or GI bleeding
- Flat and upright abdominal films of limited use
- To evaluate for intussusception: Abdominal US, air or barium enema, or CT scan (least preferred)
- Testicular US with Doppler:
- Indicated in patients with testicular pain and swelling
- Non-contrast head CT:
- Indicated if CNS findings to exclude bleed
Diagnostic Procedures/Surgery
Lumbar puncture, as clinically indicated
Differential Diagnosis
- Abdominal pain:
- Gastroenteritis
- Appendicitis
- Inflammatory bowel disease
- Intussusception
- Meckel diverticulum
- Testicular torsion
- Arthralgia:
- Acute rheumatic fever
- Polyarthritis nodosa
- Juvenile rheumatoid arthritis
- Systemic lupus erythematosus
- Rash:
- Infection:
- Meningococcemia
- Bacterial sepsis: Streptococcal or staphylococcal
- Rocky Mountain spotted fever
- Infectious mononucleosis
- Bacterial endocarditis
- Viral exanthem
- Trauma/child abuse
- Functional platelet disorders
- Thrombocytopenia
- Vasculitis
- Erythema nodosum
- Drugs/toxins
- Renal disease:
- Testicular swelling/pain:
- Incarcerated hernia
- Orchitis
- Testicular torsion ± reactive hydrocele
Prehospital
Stabilize as clinically indicated
Initial Stabilization/Therapy
- IV fluid boluses for shock
- Blood products for severe hemorrhage
ED Treatment/Procedures
- Emergent intervention for life-threatening conditions
- NSAIDs (ibuprofen):
- Prednisone:
- For severe abdominal pain once life-threatening pathology excluded
- May consider use as adjunct for analgesia
- Renal disease (controversial)
- CNS involvement
- Immunosuppressants, polyclonal Ig therapy, antiplatelet and anticoagulation drugs are controversial, recommend consultation to nephrology
Medication
- Ibuprofen: 10 mg/kg per dose PO q6h, max 600 mg per dose
- Prednisone: 1-2 mg/kg/d PO daily for 5-7 d, max 60 mg per day
Disposition
Admission Criteria
- Pain control
- CNS findings
- GI bleeding/severe abdominal pain/GI pathology
- Evidence of nephritis, renal failure
Discharge Criteria
- Normal platelet count and coagulation
- 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 (recommended for 6-12 mo in children, about 1/3 relapse within 1 yr)
- ChanH, TangYL, LvXH, et al. Risk factors associated with renal involvement in childhood Henoch-Schönlein purpura: A meta-analysis . PLoS ONE. 2016;11(11):e0167346.
- HahnD, HodsonEM, WillisNS, et al. Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP) . Cochrane Database Syst Rev. 2015;(8):CD005128.
- MasarwehK, HorovitzY, AvitalA, et al. Establishing hospital admission criteria of pediatric Henoch-Schönlein purpura . Rheumatol Int. 2014;34(11):1497-1503.
- Reid-AdamJ. Henoch-Schönlein purpura . Pediatr Rev. 2014;35(10):447-449; discussion 449.
- WeissPF, KlinkAJ, LocalioR, et al. Corticosteroids may improve clinical outcomes during hospitalization for Henoch-Schönlein purpura . Pediatrics. 2010;126(4):674-681.
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