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acute leukocytoclastic vasculitis (Henoch-Schonlein purpura; IgA vasculitis)

Also see IgA nephropathy Etiology: 1) immune complex deposition 2) IgA most commonly found in immune complexes 3) may be associated with solid tumors, especially gastrointestinal tumors 4) may be associated with myelodysplastic syndrome 5) may follow upper respiratory tract infection [4] Epidemiology: 1) most common in young children 4-7 years of age 2) may affect infants, young adults & older adults [4] 3) male:female ratio 1.5:1 4) peak incidence in spring Pathology: 1) palpable purpura in the absence of thrombocytopenia secondary to dermal leukocytoclastic vasculitis with IgA deposits in vessel walls. 2) skin biopsy a) neutrophilic or uncommonly lymphocytic infiltrate surrounding small arteries, arterioles or venules b) deposits in vessel walls - immune complexes, most commonly IgA - fibrin deposition c) endothelial swelling & necrosis d) neutrophil leukocytoclasis with scattering of nuclear fragments or nuclear dust 3) renal biopsy a) segmental glomerulonephritis b) crescentic & mesangial IgA deposits * histopathology image [11] Clinical manifestations: 1) anticedent upper respiratory tract infection [15] 2) joint pain & swelling, arthralgia, arthritis (82%) - may involve metacarpophalangeal joints & proxymal interphalangeal joints [15] 3) gastrointestinal symptoms a) nausea/vomiting b) gastrointestinal bleeding (33%) [5] - bloody stools c) abdominal pain (63%) [5] 4) glomerulonephritis (IgA nephropathy), hematuria (40%) [5] 5) palpable purpura, most commonly on buttocks & lower extremities (100%) [5] 6) myocardial involvement may occur in adults 7) rarely involves the lungs - pleuritis - pneumonitis - pulmonary hemorrhage from vasculitis (hemoptysis) [4] * images [11] Laboratory: 1) complete blood count (CBC) a) leukocytosis b) normal platelet count c) eosinophilia may be present 2) normal complement levels (generally normal, may be transiently low) - anti-neutrophil cytoplasmic antibody negative [15] 3) serum IgA may be elevated (50%) 4) erythrocyte sedimentation rate (ESR) is generally elevated 5) microscopic hematuria 6) mild proteinuria 7) serum urea nitrogen & serum creatinine variable 8) skin biopsy with immunofluorescence microscopy 9) renal biopsy with immunofluorescence microscopy if skin biopsy is non-diagnostic 10) see ARUP consult [7] Complications: 1) intussusception 2) renal failure Differential diagnosis: - hypersensitity vasculitis - meningococcemia - mixed cryoglobulinemia: complement C4 low - microscopic polyangiitis: ANCA positive - granulomatosis with polyangiitis (Wegener's granulomatosis): ANCA positive - polyarteritis nodosa: - vasculitis of small to medium sized arteries - skin lesions consistent with inflammation of larger & deeper arteries than palpable purpura resulting in nodules & ulcers Management: 1) prognosis a) most patients recover completely - spontaneously resolution in 94% of children & 89% of adults [9] b) symptomatic management 1st line [15] - topical glucocorticoids, NSAIDs, leg elevation, antihistamines [15] 2) pharmacologic agents a) glucocorticoids [4] - oral prednisone 1 mg/kg/day tapered to clinical response - diminishes arthralgias, GI manifestations [6] - not useful for renal or skin manifestations - does not shorten disease or diminish incidence of recurrence b) other immunosuppressive agents; azathoprine [15] c) colchicine [15] d) dapsone [15] 3) rapidly progressive glomerulonephritis a) plasmapheresis b) intravenous immunoglobulin (monthly) c) cyclophosphamide [4] 4) no therapy is of proven benefit 5) older adults - workup for solid tumor & myelodysplastic syndrome [4]

Interactions

disease interactions

Related

IgA nephropathy; focal glomerulonephritis; Berger's disease leukocytoclasis

General

leukocytoclastic vasculitis; hypersensitivity vasculitis; trisymptome

References

  1. Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1918-19
  3. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 848-49
  4. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18,19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  5. Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226. PMID: 19501230
  6. Weiss PF et al. Corticosteroids may improve clinical outcomes during hospitalization for Henoch-Schonlein purpura. Pediatrics 2010 Oct; 126:674. PMID: 20855386
  7. ARUP Consult: Henoch Schonlein Purpura - IgA-Associated Vasculitis The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/iga-vasculitis
  8. Mitsui H, Shibagaki N, Kawamura T, Matsue H, Shimada S. A clinical study of Henoch-Schonlein Purpura associated with malignancy. J Eur Acad Dermatol Venereol. 2009 Apr;23(4):394-401 PMID: 19207675
  9. Reamy BV, Williams PM, Lindsay TJ. Henoch-Schonlein purpura. Am Fam Physician. 2009 Oct 1;80(7):697-704. PMID: 19817340
  10. Saulsbury FT Henoch-Schonlein purpura. Curr Opin Rheumatol. 2010 Sep;22(5):598-602 PMID: 20473173
  11. Eastham ABW, Diamond HS (images) Medscape: Leukocytoclastic Vasculitis. http://emedicine.medscape.com/article/333891-overview
  12. Pohl M. Henoch-Schonlein purpura nephritis. Pediatr Nephrol 2014 Apr 16; 30:245 PMID: 24733586
  13. Miloslavsky EM, Stone JH, Moy AP. Case 14-2017 - A 20-Year-Old Man with Pain and Swelling of the Left Calf and a Purpuric Rash. N Engl J Med 2017; 376:1868-1877. May 11, 2017 PMID: 28489991 http://www.nejm.org/doi/full/10.1056/NEJMcpc1616397
  14. Audemard-Verger A, Pillebout E, Guillevin L et al IgA vasculitis (Henoch-Shonlein purpura) in adults: Diagnostic and therapeutic aspects. Autoimmun Rev. 2015 Jul;14(7):579-85. Review. PMID: 25688001
  15. NEJM Knowledge+ Rheumatology