Lynette J. Margesson, MD, FRCPC
Section of Dermatology, Department of Surgery & Department of Obstetrics and Gynecology, Dartmouth Medical School, Hanover, NH, USA
Vulvovaginal diseases commonly are inadequately diagnosed and treated. Most are dermatologic, but can be atypical in presentation in the warm, moist genital area. There is limited training or education for medical caregivers for these conditions. The first step is correct diagnosis, which requires time and knowledge of the normal anatomy, and careful examination. Dermatologists are invaluable for management as they recognize skin problems and can correct barrier function, control inflammation, and address itching and pain.
examination, lichen planus, lichen sclerosus, pruritus, vagina, vulva
The consequential effects of vulvovaginal diseases being rarely taught is that they are frequently missed or mismanaged by medical and surgical caregivers, leaving both patients and physicians floundering. Women hide and scratch, enduring pain, engendering significant debility and sexual dysfunction, and wasting millions of dollars on “yeast treatments”. The general unfamiliarity with the normal anatomy and the atypical appearance of common dermatological conditions represent a considerable clinical challenge. Dermatologists, who are accustomed to instant visual diagnosis, need to take extra time to apply their knowledge of morphology and recognize the normal anatomy when treating vulvovaginal disorders. We are uniquely qualified to help1,2 in assessing the pathology, identifying etiology, correcting barrier function, limiting inflammation, and addressing cutaneous itching and pain.
Recognizing normal variants is essential. The appearance of the vulva varies depending on age, ethnicity, and hormonal factors. A good diagram is invaluable – anatomical familiarity is important not only for the caregiver, but also for the patient. A suitable figure is available on the website of the International Society for the Study of Vulvovaginal Disease at www.ISSVD.org.
The vulva becomes flattened with loss of normal architecture in lichen sclerosus. Without an understanding of the normal architecture, subtle scarring in this condition may be unrecognized. The prepuce may be slightly swollen and adherence to the clitoris is easily missed. Scarring is a nonspecific sign associated with many erosive and inflammatory skin conditions in the vulvar area. It is important to make sure all architectural features are intact. Both digital and speculum examinations of the vagina are important to rule out erosions, synechiae, and scarring in the vagina, as can be seen in erosive lichen planus.3
Because common variations in vulvar anatomy may be unfamiliar, recognizing these is important. Vulvar papillomatosis shows small monomorphous papules on the vulvar trigone that can be easily confused with genital warts. Normal sebaceous glands (Fordyce spots) are at times quite prominent and can appear worrisome. These lesions are soft, asymptomatic, and harmless. Also, common benign blood vessel growths on the labia majora (angiokeratomas) can appear black and sinister.3
A thorough and accurate patient history is essential, including details of previous treatments and response to therapy. Inquire about all the various products the patient is using, particularly cleansers, lubricants, and menstrual products. Do not always accept the chief complaint at face value. The patient may complain of itching or burning, but her real concern is about infidelity, cancer, or sexually transmitted infection. Always ask about incontinence, both urinary and fecal, as 10% of women over 50 years of age have urinary incontinence.4 Women seldom, if ever, volunteer this information to their treating physician. Furthermore, fecal incontinence is very common and almost never mentioned. Schlosser et al. 3 provide a detailed approach.
A thorough examination requires good visualization with proper lighting, avoiding glare. Proper exposure is mandatory. Examination can be in the frog-leg position or in stirrups. The latter is preferred as one can then visualize the entire area including the anus. A close look, preferably with magnification, is mandatory. Not infrequently a patient will complain bitterly of pain and burning from an apparently trivial lesion. Painful fissures and erosions can be very subtle or invisible in poor light. Concomitant vulvar conditions are common. It is not unusual to see the patient with lichen sclerosus, contact dermatitis from incontinence, and atrophy from lack of estrogen. Occasionally, squamous cell carcinoma may also be seen. Examine the rest of the skin and the oral cavity. About 60% of patients with oral lichen planus have vulvar or vaginal disease.5,6 Always investigate the possibility of more than one problem. Iatrogenic disease is common. One can see steroid atrophy with thigh striae or herpes simplex flaring in an area of lichen sclerosus being treated with a superpotent topical steroid. Vaginal disease must be considered to be associated with the vulvar condition or contributing to it. It is worth noting that about 60% of vulvar lichen planus cases have vaginal disease. Chronic vaginitis discharge from any vaginitis can cause or worsen a vulvar condition.1,3
Biopsies are always important, especially if the patient will need lifetime treatment as in lichen sclerosus. However, a biopsy of lichen sclerosus is not generally recommended for children. A pain-free biopsy is ideal, and can be accomplished using a topical anesthetic (e.g., 2.5% lidocaine with 2.5% prilocaine in a cream base). This is applied liberally to the modified mucous membrane area for 20 minutes, or for 60 minutes for keratinized skin. Local anesthesia with 2% lidocaine with adrenaline is then injected slowly using a 30-gauge needle. If there is any question, do multiple biopsies at a single sitting. A typical problem is vulvar melanosis. There are often several sites that look suspect and it is best to biopsy all of them at one visit. Biopsies, particularly from vulvar or vaginal lichen planus, can be nonspecific. Differentiated squamous cell carcinoma of the vulva can be read as lichen simplex chronicus. Therefore, a dermatopathologist who is clinically familiar with these conditions is invaluable.
Education and Support
Before starting therapy it is important to understand that for almost all vulvovaginal conditions there are psychological, social, and sexual repercussions. Loss of intimacy not uncommonly results in low self-esteem, frustration, depression, anger, or hostility. Frequently, some combination of patient ignorance, guilt, embarrassment, and anxiety further complicate vulvovaginal problems. Consequently, good education, support, and counseling are imperative. More than with any other area of the body, the vulvar patient needs to be recognized as a person and treated gently and with respect. Take extra time for patient education by addressing the disease process, discussing available therapies, and managing expectations. Precise treatment details must be conveyed. As well, use the encounter for an educational vulvar examination. Handouts are very helpful to demystify the disorder and improve compliance. High-quality clinical photographs are essential for patient education and documentation.
The goal of therapy is to correct barrier function, thereby reducing or eliminating inflammation, itching, and pain.
Common causes of altered skin barrier in the vulvovaginal area are contact dermatitis (particularly from overzealous hygiene), atopic dermatitis, atrophy from lack of estrogen, psoriasis, or other ‘rashes’ and, less commonly, tumors. To provide the optimal environment for maintenance of a functioning skin barrier, it is important to limit the exposure to harmful factors (e.g., excessive hygiene, heat, sweat, vaginal secretion, urine, feces, clothing washed in enzyme-containing detergents, and friction) that can cause or exacerbate any skin condition. Sweat, heat, and moisture promote maceration, epithelial breakdown, and infection. Women have a tendency to over wash using facecloths and caustic cleansers. For cleansing, little to no soap is best. Dove for Sensitive Skin® (fragrance free) or Cetaphil Gentle Cleanser® can be used for cleansing with bare hands only. A hand-held shower on a gentle setting can be a good choice, especially for women with physical limitations. The area is patted dry and hairdryers should never be used. Clothing should be ventilated, fit well, and be laundered in enzyme-free detergent. Avoid thongs, girdles, and tight jeans. Urinary and fecal incontinence need to be addressed. For urinary incontinence, an appropriate incontinence pad (not a menstrual panty liner) should be used. For fecal cleansing, consider Cetaphil Gentle Cleanser® or mineral oil. Pelvic floor rehabilitation and/or help from a urologist should be considered.
The concept of ‘soak and seal’ is the same on the vulva as elsewhere. Soaks provide symptomatic relief, gentle debridement, and restore a moist environment for healing. A plain water soak in a tub or sitz bath for 5-10 minutes can be used. Occasionally, water will sting very raw skin. Normal saline does not sting, so it can be used by mixing 1 teaspoon of salt in
4 cups of water. After the soak, to seal in the moisture, a petrolatum-based product is best, but this can be messy, sticky, and can even trap sweat. A hypoallergenic product, such as Vanicream™, can be very useful. For very raw skin, plain white petrolatum is recommended. Always perform cultures and treat any associated infection caused by Candida and bacteria, usually Staphylococcus and Streptococcus. For acute severe candidiasis use fluconazole 150mg on day 1, 3 and 7. For suppression use fluconazole 150mg per week for up to 6 months. If the patient exhibits a poor response, re-culture to rule out an azole resistant Candida (e.g., Candida Glabrata), which would require treatment with 600mg boric acid vaginal suppositories nightly for 14 days.
Too often topical corticosteroids are not effectively used in the vulvovaginal area. It must be appreciated that the vulvar vestibule is relatively treatment resistant to topical corticosteroids, in contrast to the labiocrural folds, perineum, perianal area, and thighs (which can easily be thinned and develop striae). For thick, scaly vulvar conditions, such as lichen sclerosus, lichen planus, or lichen simplex chronicus, a superpotent steroid (e.g., clobetasol or halobetasol 0.05% in an ointment base) is advisable. Daily treatment may be needed for 8-12 weeks to gain adequate control. Education is very important here. The patient needs to know exactly where to apply the ointment and how much. A diagram is very useful and a clinical photograph of the female patient’s affected area is even better. Limit the use of superpotent corticosteroids in the steroid sensitive areas to 2-3 weeks. Limit the amount prescribed to 15g. For long-term use consider intermittent application, such as treatment on Monday, Wednesday and Friday, or switch to a low potency steroid. A typical therapeutic regimen for vulvar lichen sclerosus would be clobetasol 0.05% ointment daily for 12 weeks, then decrease to 3 times a week. If there is concern about recurrent yeast infections prescribe oral fluconazole 150mg weekly for suppression.
Topical Calcineurin Inhibitors (TCIs)
TCIs can be used to avoid corticosteroid-induced side-effects. Pimecrolimus 1% cream and tacrolimus 0.03% and 0.1% ointments have been reported to be very helpful for lichen sclerosus, lichen planus, lichen simplex chronicus, and a number of the bullous diseases, or even Crohn’s disease.7 Unfortunately, because TCIs can cause localized burning they are often poorly tolerated. Overall, both TCIs are less effective than topical potent and superpotent steroids for treating vulvovaginal skin disorders. There is controversy regarding their safety in lichen sclerosus and lichen planus. In addition, their cost can be prohibitive.
For a nonresponsive area of lichen sclerosus or lichen planus, triamcinolone acetonide 10mg/mL diluted to a concentration of 3.3-5mg/mL can overcome the failure of topicals. The area injected will depend on the individual case. To avoid pain, preanesthetize the area. Treatment can cause atrophy and must be used intermittently with caution.8
Systemic steroids can be very useful for intractable itching and inflammation. Classically, prednisone is recommended, but it too often causes gastrointestinal upset, anxiety, and agitation. Systemic prednisone is very useful for a quick burst without a taper for 7-10 days when treating an acute, limited skin condition, such as simple contact dermatitis. For longer-term management of inflammation, intramuscular (IM) triamcinolone (Kenalog®-40) can be an excellent choice. It is very well tolerated and best given deep into the muscle of the mid-anterior thigh. In obese women, injection into the fat results in subcutaneous atrophy, slow absorption, and a poor response. One milligram per kilogram, up to 80mg/dose, is recommended. IM triamcinolone does not have the side-effects of anxiety and agitation that are common with prednisone. Its main side-effect is occasional irregular bleeding in premenopausal women. It is an ideal therapeutic option for lichen simplex chronicus and lichen planus, administered as one dose monthly for 1-3 months, limiting the number of treatments to four times a year. Although the list of generic side-effects of triamcinolone include pituitary axis suppression, infection, cataracts and worsening glaucoma, irregular menses, and rarely allergy, these are much less prevalent than with prednisone.8
Vaginal corticosteroids are imperative for the management of vaginal lichen planus and bullous diseases, however, there are no commercially available products. The simplest treatment is with clobetasol or halobetasol 0.05% ointment or cream using a Premarin® applicator and inserting 1-2g in the vagina at night. Commonly, hydrocortisone acetate is used. A 25mg suppository is available, but the dose is usually too low for effective treatment of significant disease. A 100mg suppository can be compounded. For more severe disease 10% hydrocortisone acetate is compounded in a vaginal cream and 3-5g (300-500mg) are inserted nightly for 2 weeks and then decreased to Monday, Wednesday and Friday. There are no safety data on these products and local atrophy may occur. Yeast infection must be suppressed using fluconazole 150mg weekly. Adrenal axis suppression can occur.8
Up to 10% of women present with vulvar pruritus.9 Itching is one of the most distressing vulvar symptoms and patients can find it more difficult to manage than pain. Start by identifying the underlying cause or disease for targeted treatment. Pruritus is often an ongoing clinical challenge. Management involves not only pharmacologic intervention, but also nonspecific measures, such as patient support and education. All potential irritants, including excessive body hygiene (over washing), must be stopped. Infection with Candida and bacteria must be eliminated. Cooling the area can be helpful. Use cool gel packs, not ice packs that can further injure the skin. Cool soaks or sitz baths and bland emollients can soothe fissured or eroded skin. Inflammation can be controlled with topical and systemic steroids. Sedation is often imperative to stop scratching. For nighttime sedation, hydroxyzine or doxepin starting at 10mg/dose can be slowly increased to 100mg. During the day a selective serotonin reuptake inhibitor (citalopram 20-40mg in the morning), can be helpful. For neuropathic pruritus, a tricyclic antidepressant (amitriptyline, doxepin, or nortriptyline) can be considered. Begin with a low dose and increase gradually. Gabapentin can be of benefit, start at 300mg/dose up to a maximum of 3600mg/day.10,11
Vulvar pain may be due to any one of a number of vulvar disorders or attributable to idiopathic pain (i.e., vulvodynia). Topical anesthetics are commonly recommended (e.g., 2-5% lidocaine in a gel or ointment base or 2.5% lidocaine with 2.5% prilocaine in a cream base). These can be applied several times a day if the treatment is not too irritating. Never use benzocaine as it is very caustic and allergenic. The range of pain medications is beyond the scope of this article. Typically, tricyclic agents (e.g., amitriptyline or nortriptyline), anticonvulsants (e.g., gabapentin or pregabalin), and/or antidepressants (e.g., duloxetine or venlafaxine) are used. For these medications, start low and go slow. Treatment for vulvodynia is most effective with a multidisciplinary approach using medications, pelvic floor physiotherapy, cognitive pain therapy, nerve blocks, and more.12,13
For patients not responding consider noncompliance, an incorrect diagnosis, infection, trauma due to aggressive hygiene, contact dermatitis, or squamous cell carcinoma. Factors that can contribute to noncompliance include fear of steroids, vulvar ignorance, miscommunication, secondary gain (e.g., to avoid sexual activity), and physical impairment, such that the obese or arthritic patient cannot reach the area. Always look for concomitant conditions (e.g., lichen sclerosus plus contact dermatitis plus infection). Patients showing a poor response to treatment should be reassessed, biopsied, and re-biopsied.
Dermatologists can play an important role in the management of vulvovaginal disease. We are ideally trained to recognize any skin changes and the multiple, often confusing, combinations of these conditions. In addition, familiarity with managing chronic and complex cutaneous conditions requiring long-term maintenance therapy provides an invaluable clinical advantage.
- Margesson LJ. Vulvar disease pearls. Dermatol Clin 24(2):145-55, v (2006 Apr).
- Edwards L. Vulvovaginal dermatology. Preface. Dermatol Clin 28(4):xi-xii (2010 Oct).
- Schlosser BJ, Mirowski GW. Approach to the patient with vulvovaginal complaints. Dermatol Ther 23(5):438-48 (2010 Sep-Oct).
- Nygaard I, Barber MD, Burgio KL, et al. Prevalence of symptomatic pelvic floor disorders in US women. JAMA 300(11):1311-6 (2008 Sep 17).
- Belfiore P, Di Fede O, Cabibi D, et al. Prevalence of vulval lichen planus in a cohort of women with oral lichen planus: an interdisciplinary study. Br J Dermatol 155(5):994-8 (2006 Nov).
- Di Fede O, Belfiore P, Cabibi D, et al. Unexpectedly high frequency of genital involvement in women with clinical and histological features of oral lichen planus. Acta Derm Venereol 86(5):433-8 (2006).
- Goldstein AT, Thaci D, Luger T. Topical calcineurin inhibitors for the treatment of vulvar dermatoses. Eur J Obstet Gynecol Reprod Biol 146(1):22-9 (2009 Sep).
- McPherson T, Cooper S. Vulval lichen sclerosus and lichen planus. Dermatol Ther 23(5):523-32 (2010 Sep-Oct).
- Margesson LJ, Danby FW. Anogenital pruritus. In: Bope E, Rakel R, Kellerman R (eds). Conn’s Current Therapy 2010. 2010 ed. Philadelphia: Elsevier, p882-4 (2010).
- Lynch PJ. Lichen simplex chronicus (atopic/neurodermatitis) of the anogenital region. Dermatol Ther 17(1):8-19 (2004).
- Stewart KM. Clinical care of vulvar pruritus, with emphasis on one common cause, lichen simplex chronicus. Dermatol Clin 28(4):669-80 (2010 Oct).
- Danby CS, Margesson LJ. Approach to the diagnosis and treatment of vulvar pain. Dermatol Ther 23(5):485-504 (2010 Sep-Oct).
- Groysman V. Vulvodynia: new concepts and review of the literature. Dermatol Clin 28(4):681-96 (2010 Oct).