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Open Access Publications from the University of California

Primary vulvar Paget disease – the importance of clinical suspicion

  • Author(s): Mota, Fernando
  • Horta, Miguel
  • Marques, Claudia
  • Foreid, Susan
  • Selores, Manuela
  • et al.
Creative Commons 'BY-NC-ND' version 4.0 license

Extramammary Paget disease of the vulva is a rare condition that accounts for only 1-2% of vulvar malignancies and represents a frequent cause of misdiagnosis. It is most commonly seen in postmenopausal women. Clinically it is similar to Paget disease of the breast, appearing as red, well-demarcated eczematoid lesions, with slightly raised edges. A high degree of clinical suspicion is very important when evaluating these lesions in order to avoid misdiagnosis and delay of effective treatment. We present a case of vulvar Paget disease treated with vulvectomy.

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