Paget disease of de Vulva: About a rare case and a literature review

• Published in: International journal of surgery case reports Vol. 111; p. 108870
• Main Authors: Bencherifi, Y., El Abbassi, Imane, Amghar, A., Ennachit, M., Benhessou, M., Elkarroumi, M.
• Format: Journal Article
• Language: English
• Published: Elsevier Ltd 01-10-2023; Elsevier
• Subjects: Case Report; Immunohistochemistry; Paget disease; Vulva; Immunohistochemistry; Case report; Vulva; Paget disease
• ISSN: 2210-2612; 2210-2612
• DOI: 10.1016/j.ijscr.2023.108870

Paget disease of de Vulva (PDV) is a rare neoplastic intraepithelial pathology. In the majority of cases, neoplastic proliferation remains intraepithelial and the prognosis is favorable. The standard treatment for Paget disease is surgical excision. We report the observation of a patient with extensive and recurrent vulvar Paget's disease which we treated with surgery.
A 39-year-old single woman presented with itchy heterogeneous erythematous vulvar lesions suggestive of Paget disease. A biopsy confirmed the diagnosis of non-invasive Paget disease. Surgical excision of the lesions was realized, with the anatomopathological examination confirming the diagnosis.
MPV is often diagnosed late due to the absence of specific symptoms in its initial phase. Clinically, it manifests as an erythematous lesion or eczema. Immunohistochemistry plays a crucial role in the diagnosis of VPM, helping to distinguish the disease from other vulvar conditions. Vulvar MPV has recently been subdivided into two subtypes: type 1, which concerns primary vulvar lesions, and type 2, which concerns associated primary non-cutaneous adenocarcinomatous proliferations or pagetoid intraepithelial urothelial carcinomas. Surgery is considered the gold standard treatment for MPV.
PDV is a complex disease requiring appropriate diagnosis and management, with surgery as the main treatment, but other less invasive therapeutic options may be considered on a case-by-case basis. Prognostic factors play an important role in the choice of treatment and disease progression.
•Paget's disease of the vulva is a rare intraepithelial neoplastic pathology, representing approximately 1 % of vulvar cancers.•The standard treatment for Paget's disease is surgical excision. However, the recurrence rate is high regardless of the surgical technique used.•In order to limit the functional and aesthetic after-effects, other more conservative treatments have been proposed with variable effectiveness