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Hematology and Oncology
Dermatology  
   
Coagulopathy
Purpura fulminans
 
   
Platelet
Purpura
Purpura Causes
 
   
Rheumatology
Henoch-Schoenlein Purpura
 
   
     
  Henoch-Schoenlein Purpura
Henoch Schoenlein Purpura
Henoch Schonlein Purpura
Henoch-Schonlein Purpura
HSP
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  1. Epidemiology
    1. Children and young adults
      1. Peak Incidence at 5 year old
      2. Ages 2-11 years represent 75% of cases
      3. Milder case occur in children under age 2 years
    2. Occurs more often in boys (2:1)
    3. Incidence: 14 cases per 100,000
    4. Occurs most frequently in spring and fall

  2. Pathophysiology
    1. Idiopathic inflammatory IgA hypersensitivity
    2. Diffuse vasculitis from immune complex deposition
    3. Upper respiratory infection preceeds in 60-75% cases

  3. Associated conditions preceeding HSP
    1. Infectious agents
      1. Group A Streptococcus (most common)
      2. Mycoplasma
      3. Epstein-Barr Virus
      4. Varicella Zoster Virus
      5. Parvovirus B19
      6. Cambylobacter enteritis
    2. Vaccinations
      1. Typhoid
      2. Measles
      3. Cholera
      4. Yellow Fever
    3. Environmental exposures
      1. Allergens in drugs and foods
      2. Cold exposure
      3. Insect Bites

  4. Symptoms: Classic Triad
    1. Palpable Purpuric rash on lower extremities
    2. Abdominal pain or renal involvement (Nephritis)
    3. Arthritis

  5. Signs: Rash (100% of cases)
    1. Timing
      1. Rash preceeds other signs and symptoms of HSP
      2. First appears as erythematous Papules
      3. Purpuric rash follows
    2. Distribution
      1. Typically appears on lower extremities and buttocks
      2. Can involve extremities, face and trunk
      3. Gravity and pressure dependent
    3. Classic lesions
      1. Urticarial wheels
      2. Erythematous Macules
      3. Erythematous Papules
      4. Target lesions
      5. Petechiae
      6. Pupura
        1. Initially blanch on pressure; later they do not
        2. Purpura Color changes over time
          1. Initially red-purple
          2. Becomes rust-colored with browninsh hue
          3. Fades as illness resolves
          4. Lesions may become hemorrhagic or necrotic

  6. Signs: Abdominal pain (65% of cases)
    1. Colicky abdominal pain (may mimic Acute Abdomen)
    2. Stools may show occult or gross blood
    3. Vomiting, Hematemesis

  7. Signs: Joint Involvement (70% of cases)
    1. Transient arthritis with no permanent deformity
      1. Ankles and knees most commonly affected
      2. Elbows, hands and feet may also be affected
    2. Arthritis precedes rash in 25% of cases

  8. Signs: Renal Disease (25-50% of cases)
    1. General
      1. Most serious complication of HSP
      2. Develops within 3 months of rash
    2. Presentation
      1. Hematuria most common presenting sign

  9. Complications
    1. Myocardial Infarction
    2. Pulmonary hemorrhage
    3. Pleural Effusion
    4. Intussusception
    5. Gastrointestinal Bleeding
    6. Bowel infarction
    7. Renal failue
    8. Hematuria
    9. Proteinuria
    10. Seizures
    11. Mononeuropathies

  10. Differential Diagnosis
    1. Hypersensitivity Vasculitis
      1. Elevated Renal Function tests (BUN, Creatinine)
      2. Global organ involvement
    2. Acute Abdomen
    3. Meningococcal Meningitis or septicemia
    4. Rheumatoid Arthritis
    5. Rheumatic Fever
    6. Idiopathic Thrombocytopenic Purpura
    7. Systemic Lupus Erythematosus
    8. Child abuse
    9. Drug reactions
    10. Bacterial endocarditis
    11. Rocky Mountain Spotted Fever

  11. Diagnostics
    1. Abdominal Ultrasound
      1. If GI symptoms present
    2. Skin Biopsy
      1. Leukocytoclastic Vasculitis
    3. Renal Biopsy
      1. Glomerular crescents
      2. Indistinguishable from IgA Nephropathy

  12. Labs
    1. Complete Blood Count (CBC)
      1. Leukocytosis with eosinophilia
      2. Platelets may be elevated
        1. Low platelets suggest Thrombocytopenic Purpura
    2. Sedimentation rate (ESR) variably elevated
    3. Urinalysis
      1. Hematuria may be present
    4. Stool Guiac
      1. Occult or gross blood may be present
    5. Renal Function tests (BUN, Creatinine)
      1. Elevation may suggest Hypersensitivity Vasculitis

  13. Management
    1. No specific treatments are available
    2. Bedrest
    3. Hydration
    4. NSAIDs for joint pain
    5. Systemic Corticosteroids for severe abdominal pain

  14. Course
    1. Lasts 4-6 weeks
    2. Recurrence in 50% of patients

  15. Prognosis
    1. Excellent in general
    2. Renal Disease develops in 5% (<1% develop ESRD)
    3. Predictors of serious nephropathy or ESRD
      1. Bloody stools
      2. Rash persistence
      3. Nephritis-Nephrotic Signs
        1. Progresses to ESRD within 10 years in 50% of cases
      4. Renal Biopsy with glomerular crescents
        1. Progresses to ESRD in 100% of cases

  16. References
    1. Kraft (1998) Am Fam Physician 58(2): 405-408
    2. Saulsbury (2001) Curr Opin Rheumatol 13(1):35-40


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