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Extramammary Paget's disease is a rare malignancy. The underlying cause of VPD remains not well understood. We provide an overview of the histopathology of vulvar Paget's disease, with or without invasion., (Copyright © 2020. Published by Elsevier Masson SAS.)

Paget's disease of the vulva is difficult to diagnose, as far as the extent of this lesion is concerned, since it is often coupled with invasive adenocarcinoma of the vulva. Thus, we present two cases of Paget's disease of the vulva, with use of sentinel lymph node analysis- a non-validated technique in this very context. Evaluation of sentinel lymph node may be useful in case of micro-invasive Paget's disease.

Objectives: Determine the characteristic features of sacroiliac lesions observed in patients with Paget's disease., Patients and Methods: A retrospective analysis of the hospital files of 87 patients cared for over a period of 12 years was performed. Six patients, 4 women and 2 men, mean age 79 years, were retained for study. In 4 patients one or both sacroiliac joints were involved with complete fusion of the sacral and iliac bones, confirmed by CT-scan in 3. In the 2 other patients, only one border was involved. Scintigraphy showed polyostotic Paget's disease in 3 cases with pelvic localization in the 3 others. Ankylosing spondylarthritis (B27+) was associated in 1 case and in 2 joint chondrocalcinosis without a calcium rim was visualized at the sacroiliac joint. Sacroiliac fusion was related either to new bone forming a bridge in front of the articular space or destruction of the joint cartilage with pagetic fusion., Conclusion: Sacroiliac involvement in Paget's disease leads to joint fusion by cartilaginous destruction and should suggest possible ankylosing spondylarthritis, ankylosing vertebral hyperostosis, or joint chondrocalcinosis.

Thirty six patients with Paget's disease of the vulva were reviewed. The median age of the patients at diagnosis was 67 years (range: 45-91 years). One patient had a history of previous mammary adenocarcinoma. Screening for malignancy revealed two colonic tumours. Two patients with negative screening at presentation developed, 12 and 18 months respectively after vulvectomy, an ovarian carcinoma stage IIc and a cervical and urethral adenocarcinoma. All patients were treated by surgery based on extent of the disease. The operations performed included total vulvectomy (n = 11), partial vulvectomy (n = 14) and wide local excision (n = 4). Out of the 36 patients, 29 were available to follow-up. The median follow-up period was 74 months (range 2-204 months). Three patients died of metastatic disease due to vulval adenocarcinoma and breast carcinoma, or of liver metastases from an unknown adenocarcinoma. Eighteen of the 29 patients followed up remained free of disease. Five out of the 16 patients with positive margins recurred, as did 5 out of 9 patients with negative margins. Treatment of Paget's disease of the vulva is surgical. In order to prevent recurrence, some authors have proposed surgical excision extending beyond the visible clinical lesions with intraoperative frozen sections. The data we recorded show that free margins do not seem to correlate with recurrence, so that large excision beyond the clinical lesion is not useful.

After defining the limits of the natal cleft, the author explains the process of establishing a precise diagnosis on the basis skin lesion. He describes successively: non-infiltrating eruptions or intertrigo, pigmented lesions, papular, papular-nodular, tuberculoid and tumoral eruptions, vegetating lesions, ulcerating and suppurative lesions. In each paragraph, he stresses the lesions which are usually confined to the natal cleft.