Noninvasive vulvar lesions: An illustrated guide to diagnosis and treatment
Dystrophies, vulvodynia, and other noncancerous lesions
IN THIS ARTICLE
CASE Postmenopausal dyspareunia
A 60-year-old widow who recently remarried complains of dyspareunia. Examination of the vulva reveals firm but thin white skin over the periclitoral area and labia minora and shrinking of the vulvar skin.
What is the likely diagnosis?
Lichen sclerosus is the probable diagnosis, given her age and the appearance of the vulva, although it is impossible to assure the diagnosis without a biopsy. The preferred treatment is clobetasol, an ultrapotent steroid, applied daily.
True, powerful steroids can cause atrophy if applied regularly to other areas of the skin, but clobetasol does not cause atrophy of vulvar skin. After several weeks of nightly application, the skin should be softer and more pliable, and dyspareunia should be resolved. The patient can then reduce the clobetasol application to twice weekly—but she must continue the treatment indefinitely.
The vulva over the lifespan
The vulva is sensitive to both physiologic and pathologic changes, as well as to the sex hormones that govern the menstrual cycle. The mucosa on the inner aspects of the labia minora is very similar to the skin of the vagina and thus very sensitive to estrogen. The skin of the labia majora and the outer surface of the labia minora is more consistent with hair-bearing skin in the perineal area and more sensitive to androgens, which help thicken the skin. At menopause, the loss of estrogen leads to atrophy, and the vulvar epithelium is reduced to a few layers of mostly intermediate and parabasal cell types. The labia minora and majora as well as the clitoris gradually become less prominent with age.
The skin of the vulva consists of both dermis and epidermis, which interact with each other and respond to different nutritional and hormonal influences. For example, estrogen has little effect on vulvar epidermis, but considerable effect on the dermis, thickening the skin and preventing atrophy.
Postmenopausal atrophic changes can become a clinical problem when a woman resumes sexual intercourse after a long period of abstinence, as in the opening case. If atrophy is the main complaint, estrogen replacement therapy will alleviate symptoms of tightness, irritation, and dyspareunia, but it may take 6 weeks to 6 months to achieve optimal results. In the interim, women need to be reassured that reasonable function can be achieved.
With any vulvar irritation, the patient should discontinue the use of synthetic undergarments in favor of cotton panties, which permit more adequate circulation and do not trap moisture.
Sitz baths often help relieve local discomfort, but should be followed by thorough drying.
ACOG opinion on vulvodynia
The new ACOG Committee Opinion reflects recommendations of the American Society for Colposcopy and Cervical Pathology.17
Vulvodynia. American College of Obstetricians and Gynecologists Committee Opinion No. 345. Obstet Gynecol. October 2006;108:1049–1052.
Vulvar dystrophies: Think “white”
In the past, these diseases have been defined as non-neoplastic epithelial disorders of the vulva. Although there have been many attempts to more accurately define vulvar dystrophies, none have completely described the wide variety of clinical presentations.
In general, dystrophies are disorders of epithelial growth and nutrition that often result in a white surface color change. This definition includes intraepithelial neoplasia and Paget’s disease of the vulva. The International Society for the Study of Vulvovaginal Disease has proposed multiple classifications since 1975. I prefer the clarity of the 1987 classification system.1 I also consider these terms out-of-date: lichen sclerosus et atrophicus, carcinoma simplex, leukoplakic vulvitis, leukoplakia, hyperplastic vulvitis, neurodermatitis, kraurosis vulvae, leukokeratosis, erythroplasia of Queyrat, and Bowen’s disease.
What makes the lesions white?
The white appearance of dystrophic lesions is due to excessive keratin, at times deep pigmentation, and relative avascularity. All 3 of these characteristics are present in the spectrum of vulvar dystrophies. Biopsy of the affected skin is the key to accurate diagnosis and successful therapy.
Does not raise risk of carcinoma
The most common of the 3 groups of white lesions described in the 1987 classification of dystrophies, lichen sclerosus usually occurs in postmenopausal women, but can appear at any age, including childhood (FIGURE 1). Despite claims to the contrary, there is no good evidence that women with lichen sclerosus face a higher risk for vulvar carcinoma.
Lichen sclerosus affects all ages
3-year-old child. Note the inflammation secondary to excoriations.
20-year-old woman. The glans clitoris has begun the hooding process.
70-year-old woman. The introitus has shrunk, making intercourse impossible.
Signs and symptoms
In lichen sclerosus, the skin of the vulva appears very thin, atrophic, and dry, resembling parchment. It is also white, with loss of pigmentation.
Pruritus is the most common symptom and is usually the presenting symptom. Scratching during sleep may create ulcerations and areas of ecchymosis, and there is generalized shrinking of the vulvar skin, with eventual loss of the labia minora.
The edema and shrinking that occur around the clitoris cause a “hooding” of the glans clitoris. If the process continues unchecked, it can involve the labia majora as well as the skin of the inner thigh and anal region.
Prescribe clobetasol ointment
The patient should be instructed to use clobetasol 0.05% ointment on a continuing basis. This drug is so successful it has eclipsed the use of testosterone propionate for this indication. Lorenz and colleagues2 found very high success rates in 81 symptomatic patients with biopsy-proven disease who had failed previous therapy.
For reasons that are unknown, persistent use of this steroid on vulvar skin does not cause the atrophy commonly seen with prolonged use of high-potency steroids on other areas of the skin.
Start with twice-daily application and taper to less frequent use as the symptoms come under control. Most patients in remission can be maintained with twice-weekly application. Pruritus should disappear completely, and the skin itself will become less “leathery.”
Surgical treatment is not advised
Surgery does not appear to have a role because lichen sclerosus often recurs outside excised areas. Several reports have even described the return of disease in skin grafts used to replace large diseased areas.
I do not recommend surgery except in dire circumstances, when symptom relief is essential to the patient’s quality of life and all other therapies have failed.
Squamous cell hyperplasia
This disease is probably the same entity as lichen simplex chronicus. Changes in vulvar skin appear to result from chronic scratching secondary to intense pruritus. This complaint often involves a vicious cycle of scratching, increased pruritus, and more scratching, until excoriations occur. The aim of therapy is to eliminate the pruritus (FIGURE 2).
Squamous cell hyperplasia
75-year-old woman. The skin is thickened and may be leathery.
Intense pruritus and aggressive scratching lead to excoriations.
Signs and symptoms
Vulvar skin is typically white or pink. Biopsy will confirm the diagnosis, revealing a markedly thickened keratin layer (hyperkeratosis) and irregular thickening of the Malpighian ridges (acanthosis).
Inflammatory changes are also present, especially when there are areas of excoriation.
Treatment is similar to therapy for lichen sclerosus
Potent topical corticosteroids are the backbone of treatment; clobetasol is the preferred drug. The frequency of application is identical to that described for lichen sclerosus, and response to therapy usually takes 2 months. In the interim, it is advisable to prescribe other medications for the pruritus.
Lichen sclerosus and squamous cell hyperplasia sometimes coincide. Fortunately, the therapies are quite similar and both conditions tend to respond.
This disorder consists of chronic vulvar discomfort due to itching, burning, and/or pain that causes physical, sexual, and psychological distress.3,4 Once referred to as essential vulvodynia, it now is defined as generalized vulvar dysesthesia.
Signs and symptoms
Women with this condition tend to have difficulty localizing their pain. They often present with a complaint of recurrent yeast infection or constant irritation at the introitus. Dyspareunia may or may not be a presenting symptom, although intercourse often triggers this condition. Tight pants or rough undergarments also may trigger symptoms.
Common symptoms. In a study by Sadownik,5 women with vulvar dysesthesia reported the following symptoms:
Women with vulvar dysesthesia who appear to have urinary tract symptoms should undergo a urine culture, though it will often be negative and antibiotic therapy will have little effect.
A diagnosis of exclusion
The pain of dysesthesia appears to be neuropathic in origin in that it mimics pain of the sensory nervous system. It may be diffuse or focal, unilateral or bilateral, constant or sporadic. Thus, it is a diagnosis of exclusion.
Vulvar dysesthesia should be regarded as a chronic pain syndrome and treated accordingly, with emphasis on generalized improvements in health and attitude rather than single-therapy approaches.
Potent topical corticosteroids are usually of no benefit. Nor does topical estrogen produce long-term relief.
Once all possible causes of symptoms are excluded, refer the patient for education, support, and treatment of depression, if present. Occasionally, topical anesthetics will provide short-term relief.
At least 2 vulvar pain societies—the National Vulvodynia Association and the Vulvar Pain Foundation—have newsletters, outreach programs, and Web sites.
A more readily definable condition in the same category as vulvodynia is so-called vulvar vestibulitis, also known as localized vulvodynia (as classified by the new ACOG Committee Opinion on vulvodynia).
Signs and symptoms
The defining presentation is severe pain on vestibular touch (eg, entry at intercourse), with tenderness to pressure localized within the vulvar vestibule in a horseshoe distribution pattern encompassing 3, 6, and 9 o’clock on the vestibule. Erythema is often present, especially at the 5 and 7 o’clock positions (FIGURE 3).
Patients have no symptoms during normal daily activities, but complain of dyspareunia and an inability to use tampons.
Intense erythema at the 5 o’clock position in the vestibule.
Vulvar vestibulitis can be diagnosed using a moistened cotton-tip applicator. Pressure applied in the area of the urethral meatus will result in minimal discomfort, but pressure in the horseshoe area of the vestibule will cause exquisite discomfort.
Careful inspection at 5 and 7 o’clock in the vestibule usually uncovers intense erythema over an area of 4 or 5 mm. To distinguish vestibulitis from dysesthesia, see the comparison in TABLE 1.
Distinguishing vulvar vestibulitis and dysesthesia
ESSENTIAL VULVAR DYSESTHESIA
Pain is usually not constant
Pain is a constant burning sensation
Erythema in sensitive areas
No erythema or abnormal appearance
Lidocaine quells sensitivity
Lidocaine has no effect
Cause is dermal inflammation
Cause is allodynia (heightened nerve sensitivity)
Treatment of vulvar vestibulitis is complex. It is important to see the patient often to ensure that this syndrome is truly present rather than vulvar dysesthesia. Xylocaine jelly should be given in an attempt to relieve symptoms; topical steroid ointments are another option.
Women with persistent symptoms are difficult to treat medically. Earlier theories pointing to infection as the cause of vestibulitis have been discounted.