Pediatric Nephrology

, Volume 18, Issue 12, pp 1201–1203 | Cite as

Diagnosis of Henoch-Schönlein purpura: renal or skin biopsy?

  • Jean-Claude DavinEmail author
  • Jan J. Weening
Editorial Commentary


Henoch-Schönlein purpura (HSP) is a form of systemic vasculitis characterized by vascular wall deposits of predominantly IgA, typically involving small vessels in skin, gut, and glomeruli and associated with purpura, intestinal colic, hematuria, and arthralgia or arthritis. HSP nephritis leads to chronic renal failure in up to 20% of pediatric patients after 20 years of follow-up in selected series. The risk is related to the initial clinical presentation and is maximal (more than 50%) when initial signs are a combination of nephrotic and nephritic syndromes. Although less important, the risk persists for mild renal symptoms or when the patient has apparently completely recovered from the renal disease. Other types of non-IgA-related leukocytoclastic vasculitis may be difficult to discriminate from HSP, thus confounding the diagnosis. The clinical picture of HSP is often incomplete and renal signs can become manifest years after initial signs. When based on clinical signs only, the diagnosis of HSP can therefore be missed, and some patients risk developing silent chronic renal failure after decades without appropriate treatment. Patients can also be overdiagnosed as HSP and thus submitted to unnecessary follow-up. It is therefore important that HSP should be correctly diagnosed from the initial signs. As the finding of IgA deposits in vessel walls associated with the characteristic signs of small-vessel vasculitis is a sine qua non in the diagnosis, a skin biopsy should be performed for histological and immunofluorescence studies in cases of clinical suspicion of HSP. The systematic diagnostic use of a cutaneous biopsy should not only improve the follow-up of patients with HSP but will also allow a reliable epidemiological study of vasculitis in children and a better knowledge of the disease.


Vasculitis Glomerulonephritis IgA Henoch-Schönlein purpura 


  1. 1.
    Heberden W (1802) Commentaries on the history and cure of diseases. Payne, London, p 395Google Scholar
  2. 2.
    Schoenlein JL (1847) Allgemeine und spezielle Pathologie und Therapie, 3rd edn, vol 1, p 48Google Scholar
  3. 3.
    Henoch E (1874) Uber eine eigenthumliche Form von Purpura. Berliner Klin Wochenschr 11:641Google Scholar
  4. 4.
    Berger J, Hinglais N (1968) Les dépôts intercapillaires d'IgA-IgG. J Urol Nephrol 74:694–695Google Scholar
  5. 5.
    Urizar RE, Michael A, Sisson S, Vernier RL (1968) Anaphylactoid purpura. II. Immunofluorescent and electron microscopy studies of the glomerular lesions. Lab Invest 19:437–440PubMedGoogle Scholar
  6. 6.
    Ravelli A, Carnevale-Maffe G, Ruperto N, Ascari E, Martini A (1969) IgA nephropathy and Henoch-Schönlein syndrome occurring in the same patient. Nephron 72:111–112Google Scholar
  7. 7.
    Meadow SR, Scott DG (1985) Berger disease: Henoch-Schönlein without the rash. J Pediatr 106:27–32PubMedGoogle Scholar
  8. 8.
    Davin JC, Berghe I ten, Weening JJ (2001) Henoch-Schönlein purpura nephritis and IgA nephropathy: what makes the difference? Kidney Int 59:823–834CrossRefPubMedGoogle Scholar
  9. 9.
    Allen AC, Willis FR, Beattie J, Feehally J (1998) Abnormal IgA glycosylation in Henoch-Schönlein purpura restricted to patients with clinical nephritis. Nephrol Dial Transpl 13:930–934CrossRefGoogle Scholar
  10. 10.
    Novak J, Vu HL, Novak L, Julian BA, Mestecky J, Tomana M (2002) Interactions of human mesangial cells with IgA and IgA-containing immune complexes. Kidney Int 62:715–717CrossRefPubMedGoogle Scholar
  11. 11.
    Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37:187–192PubMedGoogle Scholar
  12. 12.
    Vernier RL, Farquhar MG, Brunson JG, Good RA (1961) Anaphylactoid purpura. I. Pathology of the skin and kidney and frequency of streptococcal infections. Pediatrics 27:181–187Google Scholar
  13. 13.
    Baart De La Faille-Kuyper EH, Kater L, Kuijten RH, Kooiker CJ, Wagenaar SS, Zouwen P van der, Dorh out Mees EJ (1976) Occurrence of vascular IgA deposits in clinically normal skin of patients with renal disease. Kidney Int 9:424–429PubMedGoogle Scholar
  14. 14.
    Haycock GB (1992) The nephritis of Henoch-Schönlein purpura. In: Cameron S, Davison AM, Grünfeld J-P, Kerr D, Ritz E (eds) Oxford textbook of clinical nephrology. Oxford University Press, Oxford, pp 595–612Google Scholar
  15. 15.
    Kaku Y, Nohara K, Honda S (1998) Renal involvement in Henoch-Schönlein purpura: a multivariate analysis of prognostic factors. Kidney Int 53:1755–1759PubMedGoogle Scholar
  16. 16.
    Goldstein AR, White RHR, Akuse R, Chantler C (1992) Long-term follow-up of childhood Henoch-Schönlein nephritis. Lancet 339:280–282PubMedGoogle Scholar
  17. 17.
    Michel BA, Hunder GG, Bloch DA, Calabrese LH (1992) Hypersensitivity vasculitis and Henoch-Schönlein purpura: a comparison between 2 disorders. J Rheumatol 19:721–728PubMedGoogle Scholar
  18. 18.
    Calabrese LH, Michel BA, Bloch DA, Arend WP, Edworthy SM, Fauci AS, Fries JF, Hunder GG, Leavitt RY, Lie JT et al (1990) The American College of Rheumatology 1990 criteria for the classification of hypersensitivity vasculitis. Arthritis Rheum 33:1108–1113PubMedGoogle Scholar
  19. 19.
    Mills JA, Michel BA, Bloch DA, Calabrese LH, Hunder GG, Arend WP, Edworthy SM, Fauci AS, Leavitt RY, Lie JT et al (1990) The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arthritis Rheum 33:1114–1121PubMedGoogle Scholar
  20. 20.
    Rao JK, Allen NB, Pincus T (1998) Limitations of the 1990 American College of Rheumatology classification criteria in the diagnosis of vasculitis. Ann Intern Med 129:345–352PubMedGoogle Scholar

Copyright information

© IPNA 2003

Authors and Affiliations

  1. 1.Department of Pediatrics, Academic Medical CenterUniversity of AmsterdamThe Netherlands
  2. 2.Department of Pathology, Academic Medical CenterUniversity of AmsterdamThe Netherlands

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