Vulvar diseases: Conditions in adults and children.

The most problematic vulvovaginal conditions are familiar to dermatologists but may exhibit distinct clinical features or medication management because of the anatomic location. The second article in this continuing medical education series focuses on management pearls for treating vulvar diseases. We highlight key conditions, such as lichen sclerosus, erosive lichen planus, and vulvodynia. In addition, we review conditions that dermatologists may be less familiar with, such as plasma cell vulvitis, desquamative inflammatory vaginitis, vulvar aphthae, and low estrogen states. Nearly 1 in 6 women experience undiagnosed and untreated vulvovaginal discomfort at some point in their lives. Physicians who treat vulvar disorders will improve the quality of life of countless women.

Vulvar diseases: Approach to the patient.

Patients with vulvar dermatoses often delay seeking medical treatment because of anxiety and embarrassment. Moreover, women frequently self-treat with various home remedies and see multiple clinicians before presenting to a dermatologist. Despite serving as the primary providers for patients with vulvovaginal symptoms, gynecologists typically receive limited training in the causes and management of these conditions. Dermatologists are experts in the evaluation and management of cutaneous disease and should be the caretakers of all skin, including the genitalia. Vulvar disorders are underrecognized by dermatologists for numerous reasons: inadequate training, lack of comfort with both interview and examination techniques, and unfamiliarity with normal anatomic variations. The first article in this continuing medical education series on vulvar dermatoses reviews the fundamentals, approach, and techniques that can be used to ensure a successful visit for both patient and provider.

Vulvodynia.

Vulvodynia is a genital pain syndrome occurring in 7% to 8% of women. Although common, most practitioners are uncomfortable with the diagnosis and management of these women's pain, and many believe this is psychologically based. Multifactorial in origin, ubiquitous factors include pelvic floor muscle abnormalities, neuropathic pain, anxiety, and primary or secondary sexual dysfunction. Although there are many published studies on vulvodynia, quality trials that evaluate therapy are lacking. However, experience suggests that most patients are significantly improved with pelvic floor physical therapy, medication for neuropathic pain, psychological support, and attention to sexual function.

Noninfectious genital ulcers.

Noninfectious genital ulcers are much more common than ulcers arising from infections. Still, it is important to take a thorough history of sexual activity and a sexual abuse screen. A physical exam should include skin, oral mucosa, nails, hair, vulva, and vaginal mucosa if needed. The differential diagnosis of noninfectious genital ulcers includes: lipschütz ulcers, complex aphthosis, Behçet's syndrome, vulvar metastatic Crohn's disease, hidradenitis suppurativa, pyoderma gangrenosum, pressure ulcers, and malignancies. It is important to come to the correct diagnosis to avoid undue testing, stress, and anxiety in patients experiencing genital ulcerations.

Vulvodynia.

Vulvodynia is a pain syndrome affecting the vulva. It occurs in about 16% of women at some time of their lives. The etiology of vulvodynia is still enigmatic and is probably multifactorial-including physiological concerns (eg, pelvic floor muscle dysfunction, neuropathic pain, and psychosocial) and sexual issues (eg, anxiety and sexual dysfunction). Although it is a common syndrome, most patients are neither correctly diagnosed nor treated. A diagnosis of vulvodynia is based upon patient history and lack of physical findings upon careful examination. No clinical or histological findings are present to aid in diagnosis. Most treatment options for vulvodynia are neither well studied nor have an evidence base, relying instead upon expert opinion, care provider experience, and use of data from other pain syndromes. However, many patients show marked improvement after physical therapy for the pelvic floor, medications for neuropathic pain, and psychosexual therapy.

Vulvar inflammatory dermatoses: an update and review.

Currently, urogenital complaints are among the most common problems encountered by family practitioners, gynecologists, and dermatologists. In response to the intricacy of vulvar disorders, the International Society for the Study of Vulvovaginal Disease was created to facilitate the exchange between clinicians and pathologists involved in the care of these patients. Recent classifications for inflammatory disorders and intraepithelial neoplasm have been proposed. In addition, vulvar skin biopsies are the most common source of intradepartmental consultation during dermatopathology sign-out. The purpose of this article is to review the various inflammatory dermatoses of the vulva and to update readers with new advances regarding these entities.

Imiquimod 3.75% cream applied daily to treat anogenital warts: combined results from women in two randomized, placebo-controlled studies.

OBJECTIVE: To evaluate if new imiquimod formulations using a shorter treatment duration are safe and efficacious to treat anogenital warts. METHODS: In two studies 534 women ≥12 years of age (mean 33.4) with 2-30 warts (mean 7.9) and total wart area ≥10 mm(2) (mean 166.3) were randomized (1:2:2) to placebo (106), imiquimod 2.5% (212) or 3.75% (216) creams applied once daily until complete clearance or a maximum of 8 weeks. RESULTS: For placebo, imiquimod 2.5% and 3.75%, respectively, complete clearance of all warts was achieved in 14.2%, 28.3%, and 36.6% of women (intent-to-treat, P = 0.008 imiquimod 2.5%, and P < 0.001 3.75% versus placebo). Mean changes in wart counts were -10.7%, -50.9%, and -63.5% (per-protocol, P < 0.001 each active versus placebo) and safety-related discontinuation rates 0.9%, 1.4%, and 2.3%. CONCLUSIONS: Imiquimod 3.75% applied daily for up to 8 weeks was well tolerated and superior to placebo in treating women with external anogenital warts.

Pigmented vulvar lesions.

Pigmented lesions represent an enormous range of conditions, from benign to malignant tumors, and from infectious to post-inflammatory. Pigmented lesions are much less easily diagnosed on anogenital skin, and clinicians should have a low threshold for biopsy confirmation of diseases not classic in appearance.

Desquamative inflammatory vaginitis: what is it?

OBJECTIVE: To record the inflammatory patterns found in desquamative inflammatory vaginitis and to investigate further the existence of an idiopathic subset of this condition. STUDY DESIGN: This was a retrospective case note study of 11 women over a 12-month period who presented with symptoms of painful sexual intercourse and increased vaginal discharge. RESULTS: Examination of the vulva was usually normal or showed mild erythema and a thin purulent discharge. Examination of the vaginal wall showed erythema consistent with inflammation. A biopsy from the vaginal wall showed essentially 2 patterns of inflammation: either a lichenoid or a nonspecific mixed inflammatory infiltrate. Therapy with clindamycin and clobetasone propionate was largely effective. CONCLUSION: While this study does not fully answer the histology of desquamative inflammatory vaginitis, it does highlight the need for further study to identify whether there is an idiopathic subset of desquamative inflammatory vaginitis or whether it is erosive lichen planus.

A survey on diagnosis and treatment of vulvodynia among vulvodynia researchers and members of the International Society for the Study of Vulvovaginal Disease.

OBJECTIVE: To survey members of the International Society for the Study of Vulvovaginal Disease and authors of recent related medical publications for whom e-mail addresses were available to assess current opinion and practices. STUDY DESIGN: In a cross-sectional online survey of potential participants, characteristics of respondents and their preferred diagnostic criteria and treatment modalities for vulvodynia were assessed using univariate and multivariate methods. RESULTS: Of recipients, 61.0% completed the survey. Of these, 86.7% were active in diagnosing or treating women with vulvodynia and 41.3% were currently active in vulvodynia research. Of respondents, > 80% include a history, a genital visual examination and vulvar pressure sensitivity testing in their evaluation of women with vulvar pain. Laboratory assessments were less likely to be rated essential or helpful. Of participants, > 80% rated as very effective or somewhat effective tricyclic antidepressants, pelvic floor physical therapy and psychologic counseling. Most indicated they have made the diagnosis of vulvodynia in conjunction with other vulvovaginal diagnoses and agreed that vulvodynia does remit for some women. CONCLUSION: Despite many diagnostic and treatment options available, some consensus on diagnostic and treatment preferences for vulvodynia was identified. Further research to develop the evidence base for diagnostic and treatment decisions is needed.

Diagnosis and management of vulvodynia in postmenopausal women.

Vulvodynia, defined as vulvar pain or burning sensation for more than 3 months, without an identifiable cause, can occur at any age. In this paper, the authors address the classification, epidemiology, etiology, diagnosis, and treatment of this condition, focusing on postmenopausal women. In postmenopausal women, vulvar pain and dyspareunia can often be attributed to low levels of estrogen resulting in vulvovaginal atrophy. While correction of vulvovaginal atrophy is an important part of the management of these patients, it will usually be insufficient to manage vulvodynia. The treatment of vulvodynia includes general care measures, topical, oral, or injectable agents, psychological approaches, pelvic floor rehabilitation and, in some cases, surgery. No particular intervention has been shown to be superior, so a "trial and error" strategy is usually used.

Maintenance fluconazole therapy for recurrent vulvovaginal candidiasis.

BACKGROUND: No safe and convenient regimen has proved to be effective for the management of recurrent vulvovaginal candidiasis. METHODS: After inducing clinical remission with open-label fluconazole given in three 150-mg doses at 72-hour intervals, we randomly assigned 387 women with recurrent vulvovaginal candidiasis to receive treatment with fluconazole (150 mg) or placebo weekly for six months, followed by six months of observation without therapy. The primary outcome measure was the proportion of women in clinical remission at the end of the first six-month period. Secondary efficacy measures were the clinical outcome at 12 months, vaginal mycologic status, and time to recurrence on the basis of Kaplan-Meier analysis. RESULTS: Weekly treatment with fluconazole was effective in preventing symptomatic vulvovaginal candidiasis. The proportions of women who remained disease-free at 6, 9, and 12 months in the fluconazole group were 90.8 percent, 73.2 percent, and 42.9 percent, as compared with 35.9 percent, 27.8 percent, and 21.9 percent, respectively, in the placebo group (P< 0.001). The median time to clinical recurrence in the fluconazole group was 10.2 months, as compared with 4.0 months in the placebo group (P<0.001). There was no evidence of fluconazole resistance in isolates of Candida albicans or of superinfection with C. glabrata. Fluconazole was discontinued in one patient because of headache. CONCLUSIONS: Long-term weekly treatment with fluconazole can reduce the rate of recurrence of symptomatic vulvovaginal candidiasis. However, a long-term cure remains difficult to achieve.

Subsets of vulvodynia: overlapping characteristics.

OBJECTIVE: To determine the characteristics of vulvar pain as to location and provocation by touch and pressure in order to confirm that current International Society for the Study of Vulvovaginal Disease definitions of vulvar vestibulitis (provoked vestibulodynia) and vulvodynia (generalized vulvodynia) effectively describe and differentiate these 2 subsets. STUDY DESIGN: The charts of all women diagnosed with vulvodynia at their initial clinic visit between November 2002 and June 2003 were reviewed for this study. Each patient was evaluated by questionnaire, interview in person and by physical examination to ascertain the location and provoked vs. spontaneous nature of the pain as primary criteria for the differentiation of provoked vestibulodyniafrom generalized vulvodynia. RESULTS: Sixty patients were included in the analysis. Four (6.7%) described provoked pain only in the vestibule (vulvar vestibulitis, provoked vestibulodynia), and 5 women (8.3%) experienced only unprovoked pain that was not confined to the vestibule (dysesthetic vulvodynia, unprovoked generalized vulvodynia). Other patients fell into patterns not specifically recognized or identified by a name. Sixteen (26.7%) experienced both provoked and unprovoked pain always limited to the vestibule, and 21 (35.0%) described provoked and unprovoked pain that extended beyond the vestibule at least occasionally. Six (10.0%) patients described only provoked pain primarily but not limited to the vestibule, and 8 patients each described a unique pattern. CONCLUSION: This study suggests that the criteria of location and only provoked vs. only unprovoked pain alone do not describe 2 distinct subsets of vulvodynia; rather, there is overlap in provoked vs. unprovoked pain and location.

Lichen sclerosus with vaginal involvement: report of 2 cases and review of the literature.

IMPORTANCE: Lichen sclerosus (LS) is an uncommon chronic inflammatory disease that most commonly affects anogenital skin of postmenopausal women. It typically manifests as atrophic white plaques, which may be accompanied by purpura or fissuring. Rarely, LS has been observed to affect mucosal tissues in the mouth and the penile urethra. It is generally taught that LS does not affect the vagina, unlike lichen planus. To our knowledge, only one case report of LS with vaginal involvement exists in the literature. OBSERVATIONS: Two cases of severe vulvar LS with vaginal involvement are reported. Both cases exhibited characteristic features of LS on vaginal biopsy, and both patients were followed up clinically without further treatment of the vagina. CONCLUSIONS AND RELEVANCE: Vaginal LS may be more common than previously thought and may be underdiagnosed. Patients with more severe disease or with significant vaginal atrophy may be more likely to have involvement of the vagina. In addition, patients with pelvic organ laxity may be at increased risk if their vaginal walls are chronically exposed because of prolapse. Physicians managing patients with vulvar LS should be aware of the possibility of vaginal involvement so that vaginal lesions may be diagnosed and followed up appropriately.

Dermatologic causes of vaginitis: a clinical review.

The concept of vaginitis is widely accepted. Most physicians assume that vaginitis represents an infection, with nearly all vaginal complaints diagnosed as Candidiasis, bacterial vaginosis, or trichomonas. However, like the mouth, the vagina is covered with squamous epithelium, and therefore affected by various dermatoses. Some dermatoses prominently affect mucous membranes, such as lichen planus, pemphigus vulgaris, cicatricial pemphigoid, and blistering forms of erythema multiforme. In addition, some dermatoses affect only the vagina, including desquamative inflammatory vaginitis and atrophic vaginitis. The diagnosis and management of these diseases are hampered by the difficulty of visualizing the vagina, and the lack of medications other than antimicrobials available for use in the vagina.