Skip to main content

Präinvasive Erkrankungen der Vulva

Vulväre intraepitheliale Neoplasie (VIN) und M. Paget

Preinvasive vulvar diseases

Vulvar intraepithelial neoplasia (VIN) and vulvar Paget’s disease


Die vulväre intraepitheliale Neoplasie (VIN) und der M. Paget sind präinvasive Erkrankungen der Vulva, die sich in vielen Aspekten unterscheiden oder überlappen. Bei gleicher Symptomatik kann auch das klinische Bild sehr ähnlich sein. Die Ätiologie für beide Erkrankungen ist nicht ganz geklärt, nur für einen Teil der VIN-Erkrankungen ist eine persistierende HPV-Infektion ursächlich. Die lokale Exzision steht für beide Veränderungen im Vordergrund, bei VIN zusätzlich die Laserverdampfung. Daneben gibt es einige experimentelle Behandlungsformen von unterschiedlicher Wirksamkeit und Verträglichkeit. VIN und M. Paget, insbesondere Letzterer, neigen stark zum Rezidiv. Daraus ergeben sich Bedeutung und Notwendigkeit einer zuverlässigen klinischen Nachsorge.


Vulvar intraepithelial neoplasia (VIN) and vulvar Paget’s disease are preinvasive diseases of the vulva that differ in some aspects and overlap in others. With the same symptomatology, the clinical picture may be very similar, too. The etiology is not quite clear for either disease: Only the so-called usual type of VIN seems to be induced by persistent HPV infection. Local excision is the most prevalent form of therapy, along with laser vaporization for VIN. There are some experimental treatments of various effectiveness and tolerance. Both VIN and vulvar Paget’s disease have a strong tendency to recur; therefore, competent follow-up is necessary.

This is a preview of subscription content, access via your institution.

Abb. 1
Abb. 2
Abb. 3
Abb. 4
Abb. 5
Abb. 6
Abb. 7
Abb. 8
Abb. 9
Abb. 10
Abb. 11
Abb. 12
Abb. 13
Abb. 14
Abb. 15
Abb. 16
Abb. 17
Abb. 18
Abb. 19
Abb. 20
Abb. 21


  1. Beurden van M, ten Kate FW, Tjong-A-Hung SP et al (1998) Human papillomavirus DNA in multicentric vulvar intraepithelial neoplasia. Int J Gynecol Pathol 17:12–16

    Article  PubMed  Google Scholar 

  2. Brown HM, Wilkinson EJ (2002) Uroplakin-III to distinguish primary vulvar Paget disease from Paget disease secondary to urothelial carcinoma. Hum Pathol 33:545–548

    Article  CAS  PubMed  Google Scholar 

  3. Dubreuilh W (1901) Paget’s disease of the vulva. Br J Dermatol 13:407–413

    Article  Google Scholar 

  4. Fong KL, Jones RW, Rowan DM (2008) Women’s perception of the outcome of the surgical management of vulvar intraepithelial neoplasia. J Reprod Med 53:952–954

    PubMed  Google Scholar 

  5. Hatch KD, Davis JR (2008) Complete resolution of Paget disease of the vulva with imiquimod cream. J Low Genit Tract Dis 12:90–94

    Article  PubMed  Google Scholar 

  6. Joura EA, Leodolter S, Hernandez-Avila M et al (2007) Efficacy of a quadrivalent prophylactic human papillomavirus (types 6, 11, 16, and 18) L1 virus-like-particle vaccine against high-grade vulval and vaginal lesions: a combined analysis of three randomised clinical trials. Lancet 369:1693–1702

    Article  CAS  PubMed  Google Scholar 

  7. Judson PL, Habermann EB, Baxter NN et al (2006) Trends in the incidence of invasive and in situ vulvar carcinoma. Obstet Gynecol 107:1018–1022

    Article  PubMed  Google Scholar 

  8. Kürzl R (1996) Paget’s disease. Semin Dermatol 15:60–66

    Article  PubMed  Google Scholar 

  9. Kürzl R (2006) Vulvar Paget’s disease treated with topical imiquimod: preliminary results of three cases. ISSVD XVIII world congress, Queenstown, Neu Zealand

  10. Kürzl R (2009) Nicht maligne Hauterkrankungen der Vulva. Gynäkologe 42

  11. Mohs FE, Blessing J (1979) Microscopically controlled surgery for extramammary Paget’s disease. Arch Dermatol 115:706–708

    Article  CAS  PubMed  Google Scholar 

  12. Nadji M, Ganjei P, Penneys NS, Morales AR (1984) Immunohistochemistry of vulvar neoplasms: A brief review. Int J Gynecol Pathol 3:41–50

    Article  CAS  PubMed  Google Scholar 

  13. Parmley TH, Woodruff JD, Julian CG (1975) Invasive vulvar Paget’s disease. Obstet Gynecol 46:341–346

    CAS  PubMed  Google Scholar 

  14. Preti M, Mezzetti M, Robertson C, Sideri M (2000) Inter-observer variation in histopathological diagnosis and grading of vulvar intraepithelial neoplasia: results of an European collaborative study. BJOG 107:594–599

    Article  CAS  PubMed  Google Scholar 

  15. Preti M, Micheletti L, Massobrio M et al (2003) Vulvar paget disease: one century after first reported. J Low Genit Tract Dis 7:122–135

    Article  PubMed  Google Scholar 

  16. Preti M, Seters van M, Sideri M, Beurden van M (2005) Squamous vulvar intraepithelial neoplasia. Clin Obstet Gynecol 48:845–861

    Article  PubMed  Google Scholar 

  17. Regauer S (2006) Extramammary Paget’s disease – a proliferation of adnexal origin? Histopathology 48:723–729

    Article  CAS  PubMed  Google Scholar 

  18. Ronger Savle S, Julien V, Viallard AM et al (2004) Treatment of vulvar cutaneous extramammary Paget’s disease with topical imiquimod: report of two cases. 5th Congress of the European College for the Study of vulval diseases (ECSVD), Stockholm Sweden

  19. Schön M, Bong AB, Drewniok C et al (2003) Tumor-selective induction of apoptosis and the small-molecule immune response modifier imiquimod. J Natl Cancer Inst 95:1138–1149

    Article  PubMed  Google Scholar 

  20. Scurry J, Wilkinson EJ (2006) Review of terminology of precursors of vulvar squamous cell carcinoma. J Low Genit Tract Dis 10:161–169

    Article  PubMed  Google Scholar 

  21. Seters van M, Beurden van M, de Craen AJ (2005) Is the assumed natural history of vulvar intraepithelial neoplasia III based on enough evidence? A systematic review of 3322 published patients. Gynecol Oncol 97:645–651

    Article  PubMed  Google Scholar 

  22. Seters van M, Beurden van M, ten Kate FJ et al (2008) Treatment of vulvar intraepithelial neoplasia with topical imiquimod. N Engl J Med 358:1465–1473

    Article  PubMed  Google Scholar 

  23. Sideri M, Jones RW, Heller DS et al (2007) Comment on the article. In: Srodon M, Stoler MH, Baber GB (eds) The distribution of low and high-risk HPV types in vulvar and vaginal intraepithelial neoplasia (VIN and VaIN). Am J Surg Pathol 2006;30:1513–1518. Am J Surg Pathol 31:1452–1454

    Article  PubMed  Google Scholar 

  24. Sideri M, Jones RW, Wilkinson EJ et al (2005) Squamous vulvar intraepithelial neoplasia: 2004 modified terminology, ISSVD Vulvar Oncology Subcommittee. J Reprod Med 50:807–810

    PubMed  Google Scholar 

  25. Srodon M, Stoler MH, Baber GB, Kurman RJ (2006) The distribution of low and high-risk HPV types in vulvar and vaginal intraepithelial neoplasia (VIN and VaIN). Am J Surg Pathol 30:1513–1518

    Article  PubMed  Google Scholar 

  26. Stenn KS (1988) The skin. In: Weiss L (ed) Cell and tissue biology. A textbook of histology. Urban & Schwarzenberg, Baltimore München, pp 541–572

  27. Wang LC, Blanchard A, Jugde DE et al (2003) Successful treatment of recurrent extramammary Paget’s disease of the vulva with topical imiquimod 5% cream. J Am Acad Dermatol 49:769–771

    Article  PubMed  Google Scholar 

  28. Wilkinson EJ, Brown HM (2002) Vulvar Paget disease of urothelial origin: a report of three cases and a proposed classification of vulvar Paget disease. Hum Pathol 33:549–554

    Article  PubMed  Google Scholar 

  29. Wilkinson EJ, Kneale BL, Lynch PJ (1986) Report of the ISSVD Terminology Committee. J Reprod Med 31:973–974

    Google Scholar 

Download references


Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations


Corresponding author

Correspondence to R. Kürzl.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Kürzl, R. Präinvasive Erkrankungen der Vulva. Gynäkologe 42, 265–274 (2009).

Download citation

  • Published:

  • Issue Date:

  • DOI: