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Henoch schonlein purpura
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Henoch schonlein purpura

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  • 1. Anaphylactoid purpura -Vasculitis of small vessels Henoch Schonlein Purpura CSN Vittal
  • 2. HSP  Typically follows an attack of URI  More frequent in children (2-8 yrs)  Most frequently in winter months  Males twice as frequently as females  Incidence 9/100,000 population CSN Vittal
  • 3. Pathogenesis  IgA mediated vasculitis of small vessels  Deposition of IgA & C3 in small vessels of skin and renal glomeruli  ASO titers increase - ? Group A streptococcus CSN Vittal
  • 4. Clinical  Acute  Insidious  Rash – palpable purpura  Red – purple – rusty brown – fade  Lesions in crops – last from 3-10 days  Intervals vary from 3-4 months  Local angioedema in dependent or distensible areas  Arthritis in 2/3 knees, ankles  Serous effusions – resolve without residual damage CSN Vittal
  • 5. Clinical  Edema and damage to vasculature of GI tract leads to pain abdomen – often colicky  Occult heme – positive stools, diarrhoea with or without visible blood or hematemesis  Intessusception with current jelly stools may occur (ileo-ileal)  Renal involvement in 25-50%  HSM, lymphadenopathy CSN Vittal
  • 6. Complications  Rarely CNS involvement  Seizures, peresis. Coma  Rheumatoid like nodules  Cardiac and eye involvement  Pancreatitis  Pulmonary or intramuscular hemorrhages CSN Vittal
  • 7. Diagnosis  Palpable purpura  Crops in dependant areas DD:  Kawasaki disease  Polyarteritis nodosa  Meningococcemia  Systemic JRA  Factor V Leiden / Protein C deficiency CSN Vittal
  • 8. Labs  Normal  Biopsy : confirmatory [cutaneous vasculitis] CSN Vittal
  • 9. Treatment  Symptomatic  Hydration  Pain control  Avoidance of competitive activities  GI symptoms ; IV Corticosteroids :  1-2 mg/kg/hr CSN Vittal
  • 10. Complications  Renal involvement : Nephrotic syndrome  Bowel perforation  Testicular torsion CSN Vittal
  • 11. Prognosis  Self limiting  < 1% develop renal involvement and , 0.1% develop serious renal disease  Rarely death in acute stage due to bowel infarction CSN Vittal