Clinicopathologic Diagnostic Criteria for Vulvar Lichen Planus.

OBJECTIVE: The aim of the study was to describe the clinical and histopathologic features required for a clinicopathologic diagnosis of vulvar lichen planus (LP), which is divided into 3 types: erosive, classic, and hypertrophic. MATERIALS AND METHODS: The International Society of the Study of Vulvovaginal Diseases tasked the Difficult Pathologic Diagnoses committee with development of a consensus document for the clinicopathologic diagnosis of vulvar LP, lichen sclerosus, and differentiated vulvar intraepithelial neoplasia. The LP subgroup reviewed the literature and formulated diagnostic criteria, then approved by the International Society of the Study of Vulvovaginal Diseases membership. RESULTS: The clinicopathologic diagnosis of erosive LP incorporates 5 criteria: (a) a well-demarcated, glazed red macule or patch at labia minora, vestibule, and/or vagina, (b) disease affects hairless skin, mucocutaneous junction, and/or nonkeratinized squamous epithelium, (c) evidence of basal layer damage, categorized as degenerative or regenerative, (d) a closely applied band-like lymphocytic infiltrate, and (e) absent subepithelial sclerosis. The clinicopathologic diagnoses of classic and hypertrophic LP each require a characteristic clinical appearance accompanied by hyperkeratosis, hypergranulosis, acanthosis, basal layer degeneration, a closely applied lymphocytic infiltrate, and absent dermal sclerosis, with hypertrophic LP showing marked epithelial abnormality compared with classic LP. CONCLUSIONS: Clinicopathological correlation yields the most reliable diagnosis of vulvar LP. Disease appearance overlaps with other physiologic, dermatologic, infectious, and neoplastic entities; a low threshold for biopsy at all morphologically distinct areas is recommended. Use of the histopathologic criteria described in this document may reduce the nondiagnostic biopsy rate for clinically diagnosed LP.

Diagnostic criteria in 72 women with erosive vulvovaginal lichen planus.

BACKGROUND: Erosive vulvovaginal lichen planus (EVLP) is a chronic, painful dermatosis affecting mucocutaneous sites. Clinicopathological diagnostic criteria have been described on the basis of expert consensus. The aim of this study was to review the presentation of EVLP, particularly assessing the frequency of suggested diagnostic criteria. METHODS: Clinical signs, symptoms and histological features of women with a clinical diagnosis of EVLP were identified from clinical records and photographs. RESULTS: In all, 72 women with an average age of 67 years were included. Pain or burning were documented in 66/72 cases (92%) and itch in 36 (50%). Clinical images showed well-demarcated red shiny areas or erosions at the vaginal introitus (96%), scarring with loss of normal architecture (88%) and hyperkeratotic border and Wickham striae (46%). A total of 27 women had mucosal disease at another site (38%) and 24 had vaginal involvement (33%). Vulval histology was available for 45/72 cases (63%). The most prevalent histological finding was a band of inflammation with predominant lymphocytes (35/72, 49%). Overall, 97% of cases had at least three of nine suggested diagnostic criteria. CONCLUSIONS: The most frequent findings in women with EVLP were symptoms of pain or burning, well-demarcated red shiny areas or erosions at the introitus and scarring with loss of architecture. Our findings support the recently described diagnostic criteria for EVLP.

Vaginal stenosis after toxic epidermal necrolysis.

A 17-year-old adolescent girl developed severe toxic epidermal necrolysis after inhalation of methamphetamine. Recovery was complicated by vaginal synechiae and occlusion necessitating surgery.

Women's perception of the outcome of the surgical management of vulvar intraepithelial neoplasia.

OBJECTIVE: To assess women's perception of the effects of vulvar surgery for vulvar intraepithelial neoplasia (VIN). STUDY DESIGN: Women attending a vulvar clinic for routine follow-up after surgical treatment of VIN were invited to respond to a brief questionnaire relating to their perception of vulvar appearance and sexual function. Clinical details, including the patients' age, parity, the focality and size of the lesion, types of treatment and length of follow-up, were extracted from the medical records. The diagnosis of VIN was made according to the histologic criteria of the International Society for the Study of Vulvovaginal Disease. RESULTS: Only 2 of 70 (3%) women expressed dissatisfaction following surgical treatment. One woman described vulvar disfigurement and the second had diminished sexual satisfaction following surgery. CONCLUSION: There is a high level of patient satisfaction following conservative surgery for VIN.

Lipedematous scalp in a child.

A nine year old Maori girl was noted to have a spongy consistency to her posterior scalp. The overlying skin appeared normal, as was hair growth in that area. The abnormal findings became more extensive over the subsequent year. A computerized tomography scan showed diffuse thickening of subcutaneous tissues posteriorly on the scalp. A skin biopsy specimen showed a normal epidermis with a thick layer of mature subcutaneous fat and some disruption of the subcutaneous architecture. The clinical features and investigations were consistent with a diagnosis of lipedematous scalp, which has been described predominantly in adult women and is usually associated with alopecia. This condition has not been reported previously in the pediatric literature.

Vulvar intraepithelial neoplasia: aspects of the natural history and outcome in 405 women.

OBJECTIVE: To determine the clinical characteristics, outcome following surgical treatment, and natural history of cases of untreated vulvar intraepithelial neoplasia (VIN). METHODS: Four hundred five cases of VIN 2-3 seen between 1962 and 2003 were reviewed. RESULTS: The mean age of women with VIN decreased from 50.0 years before 1980 to 39.0 in subsequent years. After treatment of VIN by excision and/or laser vaporization, half the women required at least one further treatment by the 14th year. It is estimated that 50% of women with positive surgical margins had at least one further treatment within 5 years, but only 15% of women with negative surgical margins required further treatment. Invasive vulvar, perianal, or urethral carcinoma occurred in 17 (3.8%) women (mean age 42 years) after treatment. Nine (2%) cases represented treatment failure, with a median treatment-to-invasion interval of 2.4 years. Eight (1.8%) cases represented new "field" carcinomas, with a median initial treatment-to-invasion interval of 13.5 years. Ten untreated cases, aged 32-76 years (mean age 55 years), progressed to invasion in 1.1-7.3 years (mean 3.9 years). Age, previous pelvic radiotherapy, unifocality, immunosuppression, and association with multicentric neoplasia were not risk factors for the development of invasive vulvar carcinoma in this study. Spontaneous regression of VIN occurred in 47 women (mean age 24.6 years), with a median interval to complete regression of 9.5 months. CONCLUSION: Surgically treated VIN has a high rate of recurrence. Untreated VIN in women over 30 years has an appreciable invasive potential.

Clinically identifying women with vulvar lichen sclerosus at increased risk of squamous cell carcinoma: a case-control study.

OBJECTIVE: To identify clinical factors that might identify women with vulvar lichen sclerosus who are at increased risk of developing squamous cell cacinoma. STUDY DESIGN: A retrospective, case-control study compared 46 women presenting between 1992 and 2000 with clinical and histologic evidence of squamous cell carcinoma of the vulva arising in a background of lichen sclerosus and 213 new referrals with vulvar lichen sclerosus during the same period. RESULTS: The ages of the patients and presence of clinical hyperplasia were the only differences between the 2 groups. CONCLUSION: Women presenting with vulvar cancer arising within a background of lichen sclerosus are significantly older than women presenting with lichen sclerosus. In addition, clinical evidence of squamous hyperplasia is independently associated with vulvar carcinoma. Neither the presence nor duration of symptoms nor the loss of vulvar architecture is a useful indicator of potential cancer risk.

Idiopathic granulomatous vulvitis.

A 20-year-old woman initially presented with clinical evidence of an acute vulval inflammatory disorder incorrectly diagnosed as a Bartholin's abscess. Prolonged healing associated with infection followed attempts to incise and biopsy the area. Over a decade the condition has followed a chronic course with exacerbations and remissions and the vulva has become progressively more swollen and distorted. Lymphangiomas have developed. Histology showed non-necrotizing granulomas. Investigations for Crohn's disease and sarcoidosis were negative. Prednisone resulted in improvement during acute inflammatory episodes.