Noninvasive Vulvar Lesions an Illustrated Guide to Diagnosis and Treatment
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OBG M ANAGEMENT Philip J. DiSaia, MD Professor and Director Division of Gynecologic Oncology Dorothy Marsh Chair in Reproductive Biology University of California, Irvine Dr. DiSaia is also Chairman of the Gynecologic Oncology Group and Past President and Chairman of the Board of the American Board of Obstetrics and Gynecology Atrophic labia minora, pale and scarified from scratching, may be caused by lichen sclerosus. The microscopic view shows a hypertrophic keratin layer with an excoria- tion penetrating the epithelium. ® Dowden Health Media NoninvasiveCopyright vulvar lesions An illustratedFor personal guide use only to diagnosis and treatment IN THIS ARTICLE Dystrophies, vulvodynia, and other noncancerous lesions S Distinguishing vulvar vestibulitis CASE of nightly application, the skin should be and dysesthesia Postmenopausal dyspareunia softer and more pliable, and dyspareunia should be resolved. The patient can then Page 73 A 60-year-old widow who recently remarried reduce the clobetasol application to twice S complains of dyspareunia. Examination of weekly—but she must continue the treat- Paget disease: the vulva reveals firm but thin white skin ment indefinitely. Not just a breast over the periclitoral area and labia minora complaint and shrinking of the vulvar skin. The vulva over the lifespan Page 76 What is the likely diagnosis? The vulva is sensitive to both physiologic and pathologic changes, as well as to the ichen sclerosus is the probable diag- sex hormones that govern the menstrual nosis, given her age and the appear- cycle. The mucosa on the inner aspects of L ance of the vulva, although it is the labia minora is very similar to the skin impossible to assure the diagnosis without of the vagina and thus very sensitive to I M A a biopsy. The preferred treatment is clobe- estrogen. The skin of the labia majora and G tasol, an ultrapotent steroid, applied daily. the outer surface of the labia minora is E: K I M B True, powerful steroids can cause atro- more consistent with hair-bearing skin in E R L Y phy if applied regularly to other areas of the perineal area and more sensitive to M A R T the skin, but clobetasol does not cause androgens, which help thicken the skin. At E atrophy of vulvar skin. After several weeks menopause, the loss of estrogen leads to NS 62 OBG MANAGEMENT • December 2006 For mass reproduction, content licensing and permissions contact Dowden Health Media. atrophy, and the vulvar epithelium is reduced to a few layers of mostly interme- ACOG opinion on vulvodynia diate and parabasal cell types. The labia The new ACOG Committee Opinion reflects recommendations minora and majora as well as the clitoris of the American Society for Colposcopy and Cervical Pathology.17 gradually become less prominent with age. The skin of the vulva consists of both • Vulvodynia may be localized or generalized. Pain localized to the vulvar vestibule, formerly termed vulvar dermis and epidermis, which interact with vestibulitis, is now classified as localized vulvodynia. each other and respond to different nutri- • The cause is unknown, and therefore it cannot be tional and hormonal influences. For determined whether localized and generalized vulvodynia example, estrogen has little effect on vul- are different manifestations of the same disease or var epidermis, but considerable effect on completely different entities. the dermis, thickening the skin and pre- • Treatment can be difficult, and improvement can take venting atrophy. weeks or months, even with appropriate therapy. Postmenopausal atrophic changes can – No single agent is successful in all women become a clinical problem when a woman – Pain may never subside completely resumes sexual intercourse after a long peri- – Patients may need us to help them develop od of abstinence, as in the opening case. If realistic expectations for improvement atrophy is the main complaint, estrogen • Some women need psychological support, such as replacement therapy will alleviate symptoms sex therapy and counseling. of tightness, irritation, and dyspareunia, but Vulvodynia. American College of Obstetricians and Gynecologists Committee it may take 6 weeks to 6 months to achieve Opinion No. 345. Obstet Gynecol. October 2006;108:1049–1052. optimal results. In the interim, women need to be reassured that reasonable function can International Society for the Study of be achieved. Vulvovaginal Disease has proposed multi- ple classifications since 1975. I prefer the Hygienic considerations clarity of the 1987 classification system.1 I With any vulvar irritation, the patient also consider these terms out-of-date: should discontinue the use of synthetic lichen sclerosus et atrophicus, carcinoma undergarments in favor of cotton panties, simplex, leukoplakic vulvitis, leukoplakia, FAST TRACK which permit more adequate circulation hyperplastic vulvitis, neurodermatitis, Vulvar dystrophies and do not trap moisture. kraurosis vulvae, leukokeratosis, erythro- S Sitz baths often help relieve local dis- plasia of Queyrat, and Bowen’s disease. Lichen sclerosus comfort, but should be followed by thor- S Squamous cell ough drying. What makes the lesions white? hyperplasia The white appearance of dystrophic Formerly “hyperplastic lesions is due to excessive keratin, at times dystrophy” T Vulvar dystrophies: deep pigmentation, and relative avascular- S Other dermatosis ity. All 3 of these characteristics are present Think “white” in the spectrum of vulvar dystrophies. S Squamous cell In the past, these diseases have been Biopsy of the affected skin is the key to carcinoma in situ defined as non-neoplastic epithelial disor- accurate diagnosis and successful therapy. May present clinically ders of the vulva. Although there have at any age as papules been many attempts to more accurately or macules, coalescent or define vulvar dystrophies, none have com- discrete, single or multiple T Lichen sclerosus pletely described the wide variety of clini- S Paget’s disease cal presentations. Does not raise risk of carcinoma of the vulva In general, dystrophies are disorders of The most common of the 3 groups of Clinicopathologic entity epithelial growth and nutrition that often white lesions described in the 1987 classi- with a pathognomonic result in a white surface color change. This fication of dystrophies, lichen sclerosus histologic appearance definition includes intraepithelial neoplasia usually occurs in postmenopausal women, and Paget’s disease of the vulva. The but can appear at any age, including child- Source: Modified from Voet RL1 www.obgmanagement.com December 2006 • OBG MANAGEMENT 63 L Noninvasive vulvar lesions: An illustrated guide FIGURE 1 Lichen sclerosus affects all ages 3-year-old child. Note the inflam- 20-year-old woman. The glans 70-year-old woman. The introitus mation secondary to excoriations. clitoris has begun the hooding has shrunk, making intercourse process. impossible. hood (FIGURE 1). Despite claims to the con- ent use of this steroid on vulvar skin does trary, there is no good evidence that not cause the atrophy commonly seen with women with lichen sclerosus face a higher prolonged use of high-potency steroids on risk for vulvar carcinoma. other areas of the skin. Start with twice-daily application and taper Signs and symptoms to less frequent use as the symptoms come In lichen sclerosus, the skin of the vulva under control. Most patients in remission appears very thin, atrophic, and dry, can be maintained with twice-weekly resembling parchment. It is also white, application. Pruritus should disappear with loss of pigmentation. completely, and the skin itself will become FAST TRACK Pruritus is the most common symptom and less “leathery.” There is no good is usually the presenting symptom. Scratching during sleep may create ulcera- Surgical treatment is not advised evidence that tions and areas of ecchymosis, and there is Surgery does not appear to have a role women with lichen generalized shrinking of the vulvar skin, because lichen sclerosus often recurs out- sclerosus face with eventual loss of the labia minora. side excised areas. Several reports have The edema and shrinking that occur even described the return of disease in skin a higher risk for around the clitoris cause a “hooding” of grafts used to replace large diseased areas. vulvar carcinoma the glans clitoris. If the process continues I do not recommend surgery except in unchecked, it can involve the labia majora dire circumstances, when symptom relief is as well as the skin of the inner thigh and essential to the patient’s quality of life and anal region. all other therapies have failed. Prescribe clobetasol ointment The patient should be instructed to use clo- betasol 0.05% ointment on a continuing T Squamous cell hyperplasia basis. This drug is so successful it has This disease is probably the same entity as eclipsed the use of testosterone propionate lichen simplex chronicus. Changes in vul- for this indication. Lorenz and colleagues2 var skin appear to result from chronic found very high success rates in 81 symp- scratching secondary to intense pruritus. tomatic patients with biopsy-proven dis- This complaint often involves a vicious ease who had failed previous therapy. cycle of scratching, increased pruritus, and For reasons that are unknown, persist- more scratching, until excoriations occur. CONTINUED 64 OBG MANAGEMENT • December 2006 Noninvasive vulvar lesions: An illustrated guide L The aim of therapy is to eliminate the pru- FIGURE 2 ritus (FIGURE 2). Squamous cell hyperplasia Signs and symptoms