Extrammamary Paget’s disease of the vulva (EMPDV)

Extrammamary Paget’s disease of the vulva (EMPDV), this unknown

Francesco Sopracordevole1 – Orcid, Nicolò Clemente1 – Orcid, Anna del Fabro– Orcid

1 – CRO (Centro di Riferimento Oncologico – Oncological Reference Centre)  Cancer Institute Aviano

Corresponding author: Francesco Sopracordevole


DOI: 10.53146/lriog120214


Extramammary Paget’s disease of the vulva (EMPDv) is a rare neoplasia, with only intraepithelial spread in most of the cases, slowly evolving, with aspecific symptoms and high recurrence rate.
Extensive surgery is often necessary with repeated excision in case of recurrent disease. This can lead to significant genital disfigurement and impairment of the quality of life. Less invasive treatments (radiotherapy, immunotherapy, photodynamic therapy, laser ablation) have variable response and high recurrence rate. In case of invasive disease, poorly responsive to radio/chemotherapy, if a HER2 overexpression is detected, excisional surgery followed by specific target therapy is showing promising results. The key factor for the optimal management of EMPDv is the prompt diagnosis and treatment of lesions, in a multidisciplinary approach with proper therapies and a long-term follow-up, preserving the patient’s genital function and self-image.

Keywords: vulvar extramammary paget’s disease; surgery; diagnosis.

Available in LRIOG Nr.1 – 2021

e-ISSN: 1824-0283

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Clitoris: target of many vulvar diseases

Clitoris: target of many vulvar diseases

Pietro Lippa1 – Orcid, Federica Frascani2 – Orcid

1 – AIED, Viale Gorizia 14, Rome

2 – BIOS Via Archimede 12, Rome

Corresponding author: Pietro Lippa


DOI: 10.53146/lriog120216


This article aims to bring attention to how frequently the clitoris, despite being a small anatomical niche within the vulvar region, deserves careful diagnostic observation.
In fact, various inflammatory diseases often originate in the clitoral area and have specific clinical, symptomatic and perceptive characteristics. Lichen planus and scleroatrophic lichen are among the most frequent inflammatory vulvitis to originate in this anatomical region.
We describe in the morphological details all the diagnostic phases of these two pathologies. Often the pathologies affecting the clitoral area are subject to diagnostic delays for subjective reasons of the patients (delays in medical checks, long periods of tolerance of the disorders) or due to lack of attention from clinicians. We want to underline how an early intervention can determine a partial regression of an initial damage, its stabilization and, therefore, an improvement in the patient’s subjective perception with considerable advantages also for the relationship life. The clitoral area, being placed above the vulvar introitus, can very often be subject to in-depth observations. An adequate diagnostic observation, also with the help of the colposcope, a targeted biopsy and a consequent therapeutic behavior suitable for the clinical evolutionary phase in which the pathology itself is located, would also allow to intercept the possible proliferative changes that initially occur indeed in this anatomical site. On the basis of an algorithm based on these observations, it is possible to opt for drug therapy or non-invasive surgical therapy aimed at preventing and removing initially and potentially aggressive lesions.

Keywords: lichen sclerosus; lichen planus; vulva; clitoris; diagnosis; initial forms.

Available in LRIOG Nr.1 – 2021

e-ISSN: 1824-0283

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