Cases reported "Vulvar Diseases"

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1/8. Squamous cell carcinoma arising in Hailey-Hailey disease of the vulva.

    A 61-year-old woman, who was known to have Hailey-Hailey disease, presented with increasing vulval soreness. biopsy showed vulval intraepithelial neoplasia (VIN) 3 and subsequent histology from a vulvectomy specimen showed extensive VIN with early invasive squamous cell carcinoma. This may be another example of chronic inflammation of the vulval area leading to the development of squamous cell carcinoma. However, in this case, chronic human papillomavirus may also have played a part, leading to VIN and reactivation of the Hailey-Hailey disease. We can find no previous reports of squamous cell carcinoma developing in the setting of Hailey-Hailey disease.
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ranking = 1
keywords = intraepithelial neoplasia, intraepithelial, neoplasia
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2/8. Pseudoepitheliomatous hyperplasia in lichen sclerosus of the vulva.

    Small tentacles or separated nests of squamous cells in the dermis are not uncommonly seen in long-standing vulvar lichen sclerosus (LS) associated with epidermal thickening. We recently encountered a case where separated nests of well-differentiated squamous cells in the dermis were difficult to distinguish from squamous cell carcinoma (SCC). Further biopsies showed similar nests originating from every hair follicle. We postulated a diagnosis of multifocal pseudoepitheliomatous hyperplasia (PEH) to explain this phenomenon. Because we could find no reference to PEH in the setting of LS, we reviewed the biopsies of 92 women with extragenital and vulvar LS with and without carcinoma to determine its frequency and histological appearance. The study population, which excluded the index case, comprised 10 women with extra-anogenital LS, 58 with vulvar LS without carcinoma, and 24 with vulvar LS with carcinoma. The presence of PEH, epidermal thickness, predominant dermal collagen change, degree of inflammation, and presence of fibrin and red blood cells were recorded. The presence or absence of lichen simplex chronicus (LSC), squamous cell hyperplasia (SCH), and differentiated vulvar intraepithelial neoplasia (VIN) were recorded. PEH was identified only in vulvar LS, where it was seen in 7/58 (12.1%) women without carcinoma, 1/24 (8.3%) with carcinoma, and 0/10 (0%) with extra-anogenital LS. Two forms of PEH were seen: predominantly epidermal 7/8 (87.5%) and predominantly follicular 1/8 (12.5%). PEH was associated with increased epidermal thickness, less dermal edema, more dermal inflammation, fresh fibrin, and red blood cell extravasation. In all cases, there was associated LSC, but there was no SCH or differentiated VIN. In conclusion, PEH may explain many of the cases of dermal tentacles and separated squamous nests in vulvar LS with LSC. The association with fresh fibrin and red blood cells suggests that PEH might be a reaction to tissue damage. PEH is distinguished from SCC by its lack of atypia, confinement to the abnormal collagen, and limited growth. The pathologist must be careful about making a diagnosis of PEH in LS with epidermal thickening, looking carefully for basal atypia and other features of differentiated VIN in the overlying epidermis or dermal proliferation. We do not know whether PEH occurs in differentiated VIN and, if it does, how it could be distinguished from SCC.
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ranking = 1
keywords = intraepithelial neoplasia, intraepithelial, neoplasia
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3/8. Successful treatment of a high-grade intraepithelial neoplasia with imiquimod, with vulvar pemphigus as a side effect.

    Imiquimod modulates the immune response, and is a new approach for treatment of papillomavirus-associated lesions, although it has not been approved for the treatment of intraepithelial neoplasia. We present a case of a patient treated with imiquimod on account of high-grade intraepithelial neoplasia in the vulva and other locations. The posterior biopsies confirm the absence of lesions but show drug-induced pemphigus as a side effect.
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ranking = 6
keywords = intraepithelial neoplasia, intraepithelial, neoplasia
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4/8. Recurrent verruciform xanthoma of the vulva.

    Verruciform xanthoma is a rare, benign, mucocutaneous, nondestructive lesion characterized by proliferation of non-Langerhans lipid-rich histiocytes. We describe the clinical and pathologic findings in a 30-year-old female with recurrent verruciform xanthoma of the vulva 8 years after initial therapy. The differential diagnosis includes seborrheic keratosis, verruca simplex, condyloma acuminatum, granular cell myoblastoma, vulvar intraepithelial neoplasia, bowenoid papulosis, erythroplasia of Queyrat, and verrucous carcinoma.
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ranking = 1
keywords = intraepithelial neoplasia, intraepithelial, neoplasia
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5/8. Synchronous vulvar intraepithelial neoplasia (VIN) of warty type and cervical intraepithelial neoplasia (CIN): case report.

    Vulvar intraepithelial neoplasia is a precancerous lesion of the vulva, which has been referred to in the past with varied terminology. It can be associated with multicentricity of other neoplastic squamous lesions in the cervix and vagina. We report a case of vaginal intraepithelial neoplasia and concomitant cervical intraepithelial neoplasia in a 30 year old female. An attempt is made to put forth the recent terminology of vulvar intraepithelial neoplasia.
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ranking = 12
keywords = intraepithelial neoplasia, intraepithelial, neoplasia
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6/8. Vaginal and vulvar adenosis. An unsuspected side effect of CO2 laser vaporization.

    During a 15-month period, September 1984 through January 1986, 10 women who were treated in the Section of Gynecologic Oncology, Department of obstetrics and gynecology, pennsylvania Hospital, philadelphia, pennsylvania, developed vaginal or vulvar adenosis after CO2 laser vaporization. The indications for therapy were condylomata of the cervix, vagina and vulva refractory to conservative management in 3 patients, vulvar intraepithelial neoplasia in 3, lichen sclerosus in 1 and cervical intraepithelial neoplasia with condylomata on the cervix and vagina in 3. All the patients underwent treatment of the vagina. Most underwent treatment of the cervix, and some underwent treatment of the vulva at various degrees of intensity and depth. During the posttreatment colposcopic follow-up examination, all the patients demonstrated lesions colposcopically consistent with adenosis of the vagina or vulva within the area treated with the CO2 laser. Biopsies of the lesions were performed, adenosis was confirmed histologically, and endometriosis was ruled out histologically. This entity has not been previously associated with CO2 laser vaporization, and its clinical significance is undetermined. Further follow-up is indicated.
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ranking = 2
keywords = intraepithelial neoplasia, intraepithelial, neoplasia
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7/8. Malignant potential of mixed vulvar dystrophy (lichen sclerosus associated with squamous cell hyperplasia).

    Fifty women whose clinical vulvar appearance was compatible with that of hyperplastic or mixed vulvar dystrophy were evaluated in the Vulvovaginal Referral Unit, University of florida, from 1980 through 1986. Histologic material from biopsies performed on these patients was reviewed. The histologic picture was consistent with the clinical diagnosis in 33 cases. Fifteen patients had lichen sclerosus with various degrees of hyperkeratosis, while one had human papillomavirus-associated vulvar intraepithelial neoplasia and another had only mild chronic inflammation. Three patients in the mixed dystrophy group developed squamous carcinoma of the vulva. women with squamous cell hyperplasia occurring in a background of lichen sclerosus (mixed dystrophy) constitute a distinct group at higher risk of developing invasive cancer and require histologic assessment.
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ranking = 1
keywords = intraepithelial neoplasia, intraepithelial, neoplasia
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8/8. Mucosal lichen sclerosus/lichen planus overlap syndromes.

    Lichen sclerosus and lichen planus affecting cutaneous sites are easily distinguishable clinical. Clinical signs on mucosal sites, however, may not allow differentiation between these diseases, and reliance is frequently placed on histopathological findings. We report a series of seven patients with clinical evidence of coexisting vulval lichen sclerosus and lichenoid oral lesions. All patients had vulval biopsies, and four had oral biopsies. histology of all the vulval biopsy specimens showed features consistent with lichen sclerosus. Two of these patients developed vulval intraepithelial neoplasia during the course of their disease, and one progressed to a well-differentiated squamous carcinoma. histology of the oral lesions showed features that were predominantly those of lichen planus. There are, however, few reports of histologically proven oral lichen sclerosus. Variations in histopathological descriptions of lichen sclerosus, depending on duration of disease, have been reported, particularly with regard to the position of the inflammatory infiltrate in relation to the dermo-epidermal junction. We believe that these patients may have oral lichen sclerosus, or at the very least make up a distinctive group who need to be identified and followed up, as their risk of oral dysplasia is unknown.
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ranking = 1
keywords = intraepithelial neoplasia, intraepithelial, neoplasia
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