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Henoch-Schönlein Purpura

Shani H Cunningham, DO,M.Ed,FAAP Reviewed 04/2024
 


BASICS

Increasingly referred to as Immunoglobulin-A Vasculitis (IgAV) 

DESCRIPTION

  • Henoch-Schönlein purpura (HSP) is a nonthrombocytopenic, predominantly IgA-mediated, small vessel vasculitis that affec...

DIAGNOSIS

Palpable purpura/petechiae (mandatory criteria)—without thrombocytopenia and at least one of the following: 
  • Diffuse abdominal pain

  • Biopsy with predominant IgA deposition

  • Arthralgia or arthriti...

TREATMENT

GENERAL MEASURES

Rest and elevation of affected areas may limit purpura. Hydration and nutrition play a supportive role in treatment. 

MEDICATION

  • In the absence of renal dysfunction or complica...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Seen weekly during the acute illness with history, physical exam (include BP measurement), and urinalysis.

  • Because ~100% of patients who develop ...

REFERENCES

1
Pohl M. Henoch-Schonlein purpura nephritis. Pediatr Nephrol. 2015;30(2):245–252.

ADDITIONAL READING

Kawasaki  Y. The pathogenesis and treatment of pediatric Henoch-Schönlein purpura nephritis. Clin Exp Nephrol.  2011; 15(5): 648– 657. 21695413  

CODES

ICD10

D69.0 Allergic purpura 

SNOMED

  • 191306005 Henoch-Schonlein purpura (disorder)

  • 239946005 Postinfective Henoch-Schnlein purpura

CLINICAL PEARLS

  • HSP is a systemic small vessel vasculitis characterized by clinical tetrad of palpable purpura, abdominal pain, arthralgia, and renal dysfunction.

  • The main form of treatment is supporti...

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