Henoch-Schönlein Purpura criteria
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Items
☐Age <20 years at onset
☐Palpable purpura
☐Bowel Angina (diffuse abdominal pain or bowel ischemia; often with bloody diarrhea)
☐Biopsy of arterioles or venules showing granulocytic infiltration of the walls
R e s u l t s
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Henoch-Schönlein Purpura criteria

Henoch-Schönlein Purpura (HSP) criteria according to the American College of Rheumatology 1990 criteria include the following 4 items of which 2 are necessary to make the diagnosis of HSP.

  1. Age <20 years at onset
  2. Palpable purpura
  3. "Bowel Angina" (diffuse abdominal pain or bowel ischemia; often with bloody diarrhea)
  4. Biopsy of arterioles or venules showing granulocytic infiltration of the walls

Overview:

  • HSP is the most common vasculitic childhood disease
  • Affected areas most commonly include skin, joints, GI tract and kidneys
  • HSP is an IgA mediated autoimmune hypersensitivity vasculitis
  • Incidence 13.5-18/100,000 children yearly
  • Male predominance (2:1)
  • Onset age 6 months-20 years (75% of cases age 2-11 years)
  • Peak incidence: 5 years of age
  • Cause: Unknown; however, streptococcal, mycoplasmal, campylobacter, or viral infection often precedes HSP
  • Fall/Winter/Spring predominance of seasonal onset

Clinical Triad:

  • Purpuric rash of lower extremities
  • Abdominal pain (often with bloody diarrhea) or renal involvement
  • Arthritis (warmth, tenderness, swelling or joints; most commonly ankles and knees, but may get seen in elbows, hands, feet)

Differential Diagnosis:

  • Acute abdomen
  • Drug reactions
  • Idiopathic thrombocytopenic purpura
  • Microscopic Polyarteritis
  • Rheumatic fever
  • Rheumatoid arthritis
  • Rocky Mountain Spotted Fever
  • Sepsis (Meningococcemia)
  • Subacute bacterial endocarditis
  • Systemic Lupus Erythematosus
  • Wegener's granulomatosis

Rash description:

  • Most commonly involves lower extremities; but may involve buttocks, upper extremities, face or trunk
  • Is purpuric (non-blanching hemorrhage in the skin/mucous membranes)
  • Size: 5-10 mm in diameter
  • Usually palpable

References

Kraft DM, et al. Henoch-Schönlein Purpura: A Review. Am Fam Phy. August 1998;58(2):405-8.

Mills JA, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arth Rheum. 1990;33:1114-1121.

Tizard EJ. Henoch-Schönlein purpura. Arch Dis Child. April 1999;80:380-3.