Henoch Schonlein Purpura

INTRODUCTION:
  • Henoch-Schonlein purpura, the most common systemic vasculitis in children, occurs in adults as well.
  • Typical features are palpable purpura, abdominal pain, arthritis, and hematuria. 
  • Pathologic features include leukocytoclastic vasculitis with IgA deposition. 
CLINICAL FEATURES:
  1. Tetrad of symptoms
  2. Abdominal pain
  3. Renal disease
  4. Palpable purpura
  5. Arthritis/arthralgias – more common in adults and most common in knees and ankles. Generally self-limiting
  • Constitutional symptoms→ Fever, fatigue, malaise, Anorexia, weight loss
  • Skin
  1. The hallmark of this disease is rash begining as pinkish maculopapules that initially blanch on pressure and progress to petechiae or purpura which are characterized by clinically as palpable purpura.
  2. Purpura usually occurs on dependent areas of the body such as buttocks.
  •  Joints
  1. Arthralgia or arthritis of large joints that involves primarily the joint of lower exterimities, i.e. knee & ankle.
  2. Migratory pattern of joint involvement occurs.
  • Gastrointestinal tract
  1. Abdominal pain and GI bleeding
  2. Diarrhoea (with or without visible blood) or hematemesis.
  3. Intussusception may occur. 
  • Kidney
  1. Microscopic heamaturia due to Cresentic glomerulonephritis.
  2. Non-nephrotic range proteinura.
  3. There is deposition of IgA in the mesangium.
  • Others →Rarely CNS and pulmonary involvement may occur.
  1. Hypersensitive Angitis
DIAGNOSIS:
  • Generally a clinical diagnosis
  1. Skin Biopsy: can be helpful and used to confirm IgA and C3 deposits and leukocytoclastic vasculitis.
  2. perivascular neutrophilic infiltrate
  3. Renal Biopsy: not usually needed for diagnosis. Will show mesangial IgA deposits and segmental glomerulonephritis
MANAGEMENT:
  1. Usually self-limiting (1-6 weeks)
  2. Steroids:
  • may decrease tissue edema, may aid in arthralgias and some abdominal pain
  • Has not been shown to be beneficial in kidney disease or dermal manifestations
  • Does not lessen chance of recurrence
  • Does not shorten duration of disease
  • If rapidly progressive glomerulonephritis
  1. Multidrug regimens with cytotoxic drugs however not many reports with treatment in adults.
  2. Plasmaphoresis
  3. IVIG
  4. Symptomatic management of GI symptoms and surgical intervention if warranted. 
Exam Question
  • Henoch Schonlein purpura presents with deposition of Ig A
  • Cresentic Glomerulonephritis may be seen in Henoch Schonlein purpura
  • Henoch Schonlein purpura shows abdominal pain, rashes, palpable purpura and, arthritis
  • Hypersensitivity angitis is seen in Henoch schonlein purpura
  • The most common leukocytoclastic vasculitis affecting children is Henoch Schonlein purpura
  • Non blanching macules, papules and petecheal hemorrhage on lower extremities, mild abdominal pain, and skin biopsy showed IgA deposition along blood vessels and perivascular neutrophilic infiltrate is seen in Henoch Schonlein purpura
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