Henoch Schonlein Purpura PEDs MDM



MDM Templates

HSP — Uncomplicated

Child presents with palpable purpura on lower extremities and buttocks with joint pain and mild abdominal discomfort. Well appearing, tolerating oral intake, hemodynamically stable. No significant abdominal tenderness, no gross blood in stool, no scrotal swelling.

Presentation consistent with Henoch-Schönlein purpura (IgA vasculitis). Platelet count and coagulation studies normal, ruling out thrombocytopenic causes of purpura. Urinalysis shows no significant hematuria or proteinuria. Creatinine normal. Not consistent with meningococcemia, ITP, leukemia, HUS, or child abuse.[1]

Plan: Supportive care with NSAIDs for joint and abdominal pain. Discharge with PCP follow-up in 1 week. Weekly urinalysis for 4 weeks, then monthly for 3 months to monitor for delayed renal involvement. Return for worsening abdominal pain, bloody stool, decreased urine output, or swelling.


HSP — Complicated (GI or Renal)

Child with known or newly diagnosed HSP presents with severe abdominal pain, GI bleeding, or evidence of renal involvement (proteinuria, hematuria with elevated creatinine, hypertension). Abdomen is significantly tender with concern for intussusception or severe vasculitic bowel involvement.

Presentation concerning for complicated IgA vasculitis. Differential for abdominal symptoms includes intussusception (ileoileal in HSP, unlike typical ileocolic), mesenteric vasculitis, and bowel perforation. Renal findings concerning for IgA nephritis, the primary determinant of long-term morbidity.[1]

Plan: Abdominal ultrasound to evaluate for intussusception. Labs including BMP, CBC, urinalysis with protein-to-creatinine ratio. Pediatric nephrology consulted for renal involvement. Prednisone 1 mg/kg/day considered for severe abdominal pain or GI bleeding. Admit for monitoring, pain control, and serial abdominal exams.


Clinical Education

Diagnosis

HSP is a clinical diagnosis — no lab test confirms it. The classic tetrad: palpable non-blanching purpura (lower extremities and buttocks), arthritis/arthralgias (knees and ankles), abdominal pain, and renal involvement. Not all features need to be present simultaneously — components may appear over days to weeks. Peak age 3-10 years, often follows URI.[1]

The purpura is the diagnostic anchor. Palpable, non-blanching, gravity-dependent (legs and buttocks). If purpura is absent, the diagnosis is uncertain and biopsy showing IgA deposition may be needed. Normal platelet count and coagulation studies distinguish HSP from ITP and DIC.


Renal Involvement

Renal disease is the only cause of long-term morbidity in HSP. Occurs in 40-50% of children, ranging from microscopic hematuria to nephritic syndrome with proteinuria, hypertension, and rising creatinine. May develop weeks after initial presentation, which is why surveillance urinalysis is critical.[1]

Monitoring protocol: Weekly urinalysis for 4 weeks from diagnosis, then monthly for 3 months, then at 6 months. Nephrology referral for proteinuria >1 g/day, rising creatinine, or nephritic features. >95% of children with renal involvement recover fully, but those with significant proteinuria need close follow-up.


GI Complications

Intussusception occurs in 2-3% of HSP and is characteristically ileoileal (not ileocolic like typical pediatric intussusception). This makes it harder to detect on ultrasound and not amenable to air enema reduction — surgical management may be required. Suspect when abdominal pain becomes severe, colicky, or associated with obstruction.[2]

Other GI complications: mesenteric vasculitis causing GI bleeding, bowel wall edema, and rarely perforation. Abdominal pain is present in 50-75% of HSP but is usually mild and self-limited. Severe or worsening pain warrants imaging.


Scrotal Involvement

Scrotal involvement occurs in up to 15% of boys with HSP and can mimic testicular torsion with acute scrotal pain and swelling. Doppler ultrasound shows normal or increased testicular blood flow (unlike torsion). Important to recognize to avoid unnecessary surgical exploration.[3]


Treatment

HSP is primarily supportive. NSAIDs (ibuprofen) for joint and mild abdominal pain. Adequate hydration. Most episodes resolve in 4-6 weeks without specific therapy.[1]

Steroids (prednisone 1 mg/kg/day, max 60 mg, for 1-2 weeks with taper) are considered for: severe abdominal pain not responding to NSAIDs, significant GI bleeding, scrotal involvement, or severe arthritis. Steroids may reduce GI complications but do NOT prevent renal disease.

Recurrence occurs in up to one-third of children, typically within 3-6 months. Recurrences are usually milder and managed identically.


Disposition

Admit: Severe abdominal pain not controlled with oral analgesics. Concern for intussusception. Significant GI bleeding. Evidence of renal dysfunction (rising creatinine, hypertension, nephritic syndrome). Inability to tolerate oral intake.

Discharge: Mild-moderate symptoms controlled with NSAIDs. Normal renal function and urinalysis. Tolerating PO. Reliable follow-up with PCP for serial urinalysis monitoring. Return for worsening pain, bloody stool, decreased urine output, or new swelling.


References

  1. Saulsbury FT. Henoch-Schönlein purpura in children: report of 100 patients and review of the literature. Medicine (Baltimore). 1999;78(6):395-409. PubMed
  2. Chang WL, Yang YH, Lin YT, Chiang BL. Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of 261 patients. Acta Paediatr. 2004;93(11):1427-1431. PubMed
  3. Ha TS, Lee JS. Scrotal involvement in childhood Henoch-Schönlein purpura. Acta Paediatr. 2007;96(4):552-555. PubMed

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