[Laryngeal pemphigus]. / Le pemphigus laryngé.

The pemphigus vulgaris is a serious bullous disease of the adult. The pharyngolaryngeal localization has rarely been described. The oral and genital localizations are often inaugural. We report the case of a 45 year old female who presented a laryngeal pemphigus which evolved during several years before its cure. The bullous dermatosis are serious affections capable of being stressfull to life. Their diagnosis and treatment must be done quickly. The discovery of painful pharyngolaryngeal lesions, erosive or bullous, must lead to a biopsy with immunofluorescence histological examination in order to diagnose the pemphigus vulgaris and conduct quickly the treatment.

[The follow-up of anogenital warts in a specialized consultation: study of patients lost to follow-up]. / Condylomes anogénitaux externes suivis en consultation spécialisée: étude des malades perdus de vue.

INTRODUCTION: The treatment of condylomata acuminata relies on prolonged management, partly related to the need for repeated applications with regard to destructive therapeutic measures and the clinical supervision because of the high prevalence of relapses. This implies that patients are ready to consult their doctor at regular intervals during a relatively prolonged period of time. We wanted to assess the patients lost to follow-up in a population of patients exhibiting anogenital warts together with the factors associated with the loss to follow-up. PATIENTS AND METHODS: This was a prospective observational study conducted in France among dermatologists and gynecologists between May and December 2000. The physicians were free to prescribe the treatment of their choice. The social data of the patients and the classical characteristics of the condylomata acuminata were assessed. A patient was considered as lost to follow-up when he/she no longer came in for the consultation assessing the treatment. The data of the patients lost to follow-up were compared to those of the other patients. RESULTS: Two hundred ninety-seven cases, with 57 p. 100 men, were included during the study period. The mean age of the patients was of 33.8 +/- 11.4 years, with 43 p. 100 of patients aged under 30. In 40 p. 100 of cases, the lesions had existed for more than 3 months and were symptomatic in 30 p. 100 of cases. At the end of the study, 28.3 p. 100 of patients were considered as lost to follow-up. With multivariate analysis, the loss to follow-up appeared related to the patients' age (more frequent, the younger the patient; p<0.04) and not to the fixing of a precise appointment at the end of treatment (p<0.001). The asymptomatic nature of the lesions, recognized as a loss to follow-up risk factor in univariate analysis, was at the limit of significance in multivariate analysis (p=0.06). DISCUSSION: Clinicians should be particularly attentive with young and asymptomatic patients that they treat for condylomata acuminata, and attempt to reduce the rate of lost to follow-up and improve the quality of their management.

Failure of isotretinoin and interferon-alpha combination therapy for HPV-linked severe vulvar dysplasia. A report of two cases.

BACKGROUND: Retinoids (RA) and interferon (IFN) have been reported to be active against a variety of tumors and human papillomavirus (HPV)-related lesions. Because chronic and recurrent HPV-linked vulvar intraepithelial neoplasia 3 (VIN 3) have a high risk of invasion, we evaluated combined therapy of IFN-alpha with 13-cis-retinoic acid (13 cRA) in the treatment of two VIN 3 cases of this type. CASE: Two patients with chronic and recurrent VIN 3 were treated with combined therapy of IFN-alpha (4.5 x 10(6) five times a week) and 13 cRA (1 mg/kg/d) for six months. Clinical regression was observed at the end of treatment in both cases, but histologic features of VIN 3 were still present. CONCLUSION: These data demonstrate the ineffectiveness of the combined regimen of IFN-alpha and 13 cRA with this schedule for a period of six months in recurrent and chronic VIN 3.

[Vulvar lichen sclerosus]. / Lichen scléreux de la vulve.

The most common form of vulvar dermatose is the lichen sclerosus. More frequent after menopause, it can be observed at all ages, even in childhood. Vulvar lichen sclerosus manifests usually by a vulvar pruritus and (or) orificial dyspareunia, however in 15% of cases it remains asymptomatical. The lesions can extend to all the vulva, giving it a very particular pearly white aspect, or remain localized as a "leucoplasia", requiring biopsy to confirm the diagnosis. It is mostly the hyperplastic lichen sclerosus (consisting of a marked epithelial hyperplasia) which risks degeneration. In case of non-response to a strong local steroid therapy, high risk clinical cases must be pointed out, treated and biopsied. A biopsy must be done in thick leucoplastic areas which are coarse, steady and rebellious to treatment, specially on ulcered zones without any tendency to healing or palpable infiltrated lesions.

Vulvar vestibulitis. Lack of evidence for a human papillomavirus etiology.

Prompted by contradictory papers on the subject, we performed a prospective study to assess the possible human papillomavirus (HPV) etiology of vulvar vestibulitis. Eleven patients with periglandular vestibulitis, as well as a control group of 11 patients with condylomata acuminata, were selected. Biopsy specimens were taken for histologic and virologic evaluation. One specimen of vestibulitis showed koilocytosis. Using the polymerase chain reaction, none of the vestibulitis specimens, including the one with koilocytosis, were found to harbor HPV DNA, whereas all the condylomata acuminata contained HPV 6/11 DNA. Our results do not support an HPV etiology of vestibulitis.

[Paget disease of the vulva. 36 cases]. / Maladie de Paget vulvaire: 36 cas.

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.

The epithelial changes associated with squamous cell carcinoma of the vulva: a review of the clinical, histological and viral findings in 78 women.

Seventy-eight excised specimens of squamous cell carcinoma of the vulva were reviewed retrospectively for the presence of lichen sclerosus or vulvar intraepithelial neoplasia (VIN) at sites proximal to the tumour or more distant. Lichen sclerosus was evident in 61% and VIN alone in 31%. VIN III (differentiated) was associated with over 50% of the specimens with lichen sclerosus. HPV 16 was found in six of the 11 VIN lesions, investigated but in none of the six with lichen sclerosus.

The vulvo-vaginal-gingival syndrome. A new form of erosive lichen planus.

The vulvo-vaginal-gingival syndrome, a new form of erosive lichen planus, has recently been described. The clinical features of this condition are reviewed and summarized based on the personal examination of 19 affected patients. Each of these patients demonstrated erosive or desquamative disease on some combination of vulvar, vaginal, or gingival mucosal surfaces, but often, at a single point in time, only one or two of the three areas were involved. Clinical features supporting the diagnosis of lichen planus included the regular presence of a narrow white reticulated border at the periphery of the eroded vulvar lesions and the occasional presence of nonerosive lichen planus lesions elsewhere on the skin or mucous membranes. Biopsy specimens taken from the white reticulated border generally revealed the typical histology of lichen planus, whereas biopsy specimens taken from the central eroded surface more often showed only a nonspecific inflammatory reaction. Recognition of the vulvo-vaginal-gingival syndrome as part of the lichen planus spectrum will likely reduce the frequency of otherwise unclassifiable cases of vulvo-vaginal desquamative and erosive mucositis.

An atypical case of penile carcinoma in situ associated with human papillomavirus DNA type 18.

We report an unusual case of penile carcinoma in situ in a heterosexual man. The histologic study showed a severe dysplasia, and the typing of human papillomavirus DNA, performed at the Pasteur Institute, disclosed a type 18, which is usually associated with penile and cervical carcinomas. The identification of an oncogenic human papillomavirus type in the penile lesions, such as type 18 found in this case, should lead to a search for cervical carcinoma in the sexual partner.

[Vulvovaginogingival syndrome. New characteristic grouping of plurimucous erosive lichen planus]. / Le syndrome vulvo-vagino-gingival. Nouveau groupement caractéristique du lichen plan érosif plurimuqueux.

The triple association of a chronic painful erosive vulvitis, an erosive or desquamative vaginitis and an erosive vestibular gingivitis constitutes a hitherto unreported syndrome. The first 19 cases of this affection seen in the Hôpital Tarnier over the last three years are presented and analyzed, the etiology of these erosive mucosal lesions, limited to three body regions, being lichen planus in each case. Detection of mucosal erosion at one of these three sites now requires clinical investigation of the other two, and biopsy of least one of them from the edge of an eroded zone, as well as search for other-possible mucocutaneous areas of lichen planus. Clinical onset is often asynchronous, one lesion appearing before the others, the simplest to recognize being gingival erosive lichen. In one case, however, peri-erosive lamellar detachments suggested chronic desquamative gingivitis of possible benign pemphigoid origin. Erosive lichen planus of vulva and vagina has not been reported previously. Knowledge of this syndrome allows correlation between lichen planus and certain cases of erosive gingivitis, erythroplastic vulvitis and desquamative vaginitis.