t anagemen M obg or the lower vagina and vulva are richly supplied with peripheral nerves and are, therefore, sensitive to pain, particularly the region of the hymeneal ring. Although the pudendal nerve (arrow) courses through the area, it is an uncommon source of vulvar pain. ALEXANDRA BAKER f 46 OBG Management | September 2011 | Vol. 23 No. 9 obgmanagement.com first of 3 parts VulVar pain syndromes your first challenge is making the correct diagnosis Many cases of generalized vulvodynia and localized vulvodynia (vestibulodynia) are mistakenly attributed to yeast infection, pudendal neuralgia, and other entities. Avoid those pitfalls by using a reliable roadmap for evaluation, differentiation, and identification of the various forms of vulvar pain. neal m. lonky, md, mpH, moderator; libby edwards, md, Jennifer Gunter, md, and Hope K. Haefner, md, panelists lthough the incidence of vulvar pain treatment. To address the lack of guidance, has increased over the past decade— OBG Management Contributing Editor Neal In thIs Article A thanks to both greater awareness and M. Lonky, MD, assembled a panel of experts increasing numbers of affected women—the on vulvar pain syndromes and invited them What needs phenomenon is not a recent development. to share their considerable knowledge. The to be ruled out As early as 1874, T. Galliard Thomas wrote, ensuing discussion, presented in three parts, for a diagnosis “[T] his disorder, although fortunately not offers a gold mine of information. of vulvodynia? 1 very frequent, is by no means very rare.” He In this opening article, the panel focuses page 52 went on to express “surprise” that it had not on causes, symptomatology, and diagnosis of been “more generally and fully described.” this common complaint. In Part 2, which will Conditions other Despite the focus Thomas directed to appear in the October issue of this journal, the issue, vulvar pain did not get much atten- the focus is the bounty of treatment options. than vulvodynia tion until the 21st century, when a number Part 3 follows in November, when the discus- that are associated of studies began to gauge its prevalence. For sion shifts to vestibulodynia. with vulvar pain example, in a study in Boston of about 5,000 page 54 women, the lifetime prevalence of chronic vulvar pain was 16%.2 And in a study in Texas, Common diagnoses— How common is the prevalence of vulvar pain in an urban, and misdiagnoses pudendal neuralgia? largely minority population was estimated Dr. Lonky: What are the most common diag- page 56 to be 11%.3 The Boston study also reported noses when vulvar pain is the complaint? that “nearly 40% of women chose not to seek Dr. Gunter: The most common cause of treatment, and, of those who did, 60% saw chronic vulvar pain is vulvodynia, although three or more doctors, many of whom could lichen simplex chronicus, chronic yeast infec- not provide a diagnosis.”2 tions, and non-neoplastic epithelial disorders, Clearly, there is a need for compre- such as lichen sclerosus and lichen planus, hensive information on vulvar pain and can also produce irritation and pain. In post- its causes, symptoms, diagnosis, and menopausal women, atrophic vaginitis can obgmanagement.com Vol. 23 No. 9 | September 2011 | OBG Management 47 Vulvar pain syndromes were found to have a noninfectious entity the OBG ManaGeMent expert panel instead—most commonly lichen simplex chronicus and vulvodynia.4 Neal m. lonky, md, mpH, moderator of this discussion, Dr. Edwards: The most common “diagnosis” is Clinical Professor of Obstetrics and Gynecology at the for vulvar pain is vulvodynia. However, the University of California–Irvine and a member of the Board of Directors of Southern California Permanente Medical Group. definition of vulvodynia is pain—i.e., burn- He serves as an OBG ManageMent Contributing Editor. ing, rawness, irritation, soreness, aching, or stabbing or stinging sensations—in the ab- sence of skin disease, infection, or specific neurologic disease. Therefore, even though Libby edwards, md, is Adjunct Clinical Associate Professor the usual cause of vulvar pain is vulvodynia, of Dermatology at the University of North Carolina in Chapel it is a diagnosis of exclusion, and skin disease, Hill, NC, and Chief of Dermatology at Carolinas Medical infection, and neurologic disease must be Center in Charlotte, NC. Dr. Edwards is a Past President and Past Secretary General of the International Society for the ruled out. Study of Vulvovaginal Disease. In regard to infection, Candida albicans and bacterial vaginosis (BV) are usually the first conditions that are considered when a Jennifer Gunter, md, is Director of Pelvic Pain and patient complains of vulvar pain, but they Vulvovaginal Disorders for Kaiser Permanente in San are not common causes of vulvar pain and francisco, Calif. are never causes of chronic vulvar pain. Very rarely they may cause recurrent pain that clears, at least briefly, with treatment. Candida albicans is usually primar- ily pruritic, and BV produces discharge and Hope K. Haefner, md, is Professor of Obstetrics and odor, sometimes with minor symptoms. Non- Gynecology at the University of Michigan Hospitals and albicans Candida (e.g., Candida glabrata) is Co-Director of the University of Michigan Center for Vulvar nearly always asymptomatic, but it occasion- Diseases in Ann Arbor, Mich. ally causes irritation and burning. Group B streptococcus is another in- fectious entity that very, very occasionally causes irritation and dyspareunia but is usu- ally only a colonizer. Herpes simplex virus is a cause of recur- also cause a burning pain, although symp- rent but not chronic pain. toms are typically more vaginal than vulvar. Chronic pain is more likely to be caused Yeast and lichen simplex chronicus typically by skin disease than by infection. Lichen sim- produce itching, although sometimes they plex chronicus causes itching; any pain is due can present with irritation and pain, so they to erosions from scratching. must be considered in the differential diag- Dr. Haefner: Several other infectious con- nosis. It is important to remember that many ditions or their treatments can cause vulvar women with vulvodynia have used multiple pain. For example, herpes (particularly topical agents and may have developed com- primary herpes infection) is classically associ- plex hygiene rituals in an attempt to treat their ated with vulvar pain. The pain is so great that, symptoms, which can result in a secondary li- at times, the patient requires admission for chen simplex chronicus. pain control. Surprisingly, despite the known That said, there is a high frequency of pain of herpes, approximately 80% of patients misdiagnosis with yeast. For example, in a who have it are unaware of their diagnosis. study by Nyirjesy and colleagues, two thirds Although condyloma is generally a pain- of women who were referred to a tertiary less condition, many patients complain of pain clinic for chronic vulvovaginal candidiasis following treatment for it, whether treatment continued on page 52 48 OBG Management | September 2011 | Vol. 23 No. 9 obgmanagement.com Vulvar pain syndromes TABLE 1 terminology and classification Some diseases that are associated with of vulvar pain from the international society vulvar pain do not qualify for the diagnosis of for the study of Vulvovaginal disease vulvodynia (table 2, page 54) because they are associated with an abnormal appearance a. Vulvar pain related to a specific disorder of the vulva. 1. Infectious (including candidiasis, herpes) 2. Inflammatory (lichen planus, immunobullous disorders) What needs to be ruled out for 3. Neoplastic (Paget’s disease, squamous cell carcinoma) a diagnosis of vulvodynia? 4. Neurologic (herpes neuralgia, spinal nerve compression) Dr. Lonky: What skin diseases need to be b. Vulvodynia ruled out before vulvodynia can be diag- nosed? 1. Generalized Dr. Edwards: Skin diseases that affect the a. Provoked (sexual, nonsexual, or both) vulva are usually pruritic—pain is a later sign. b. Unprovoked Lichen simplex chronicus (also known as ec- c. Mixed (provoked and unprovoked) zema) is pruritus caused by any irritant; any 2. Localized (including vestibulodynia, clitorodynia, hemivulvodynia) pain that arises is produced by visible exco- a. Provoked (sexual, nonsexual, or both) riations from scratching. b. Unprovoked Lichen sclerosus manifests as white epi- thelium that has a crinkling, shiny, or waxy c. Mixed (provoked and unprovoked) texture. It can produce pain, especially dys- SOURCE: Moyal-Barracco and Lynch.5 Reproduced with permission from the Journal of Reproduc- pareunia. The pain is caused by erosions that tive Medicine. arise from fragility and introital narrowing and inelasticity. Vulvovaginal lichen planus is usually involves topical medications or laser surgery. erosive and preferentially affects mucous “skin diseases that Chancroid is a painful vulvar ulcer. membranes, especially the vestibule; it some- affect the vulva are Trichomonas can sometimes be associated times affects the vagina and mouth, as well. usually pruritic—pain with vulvar pain. Desquamative inflammatory vaginitis is a later sign.” Dr. Lonky: What terminology do we use is most likely a skin disease that affects only —libby edwards, MD when we discuss vulvar pain? the vagina. It involves introital redness and a Dr. Haefner: The current terminology used clinically and microscopically purulent vagi- to describe vulvar pain was published in nal discharge that also reveals parabasal cells 2004, after years of debate over nomenclature and absent lactobacilli. within the International Society for the Study Dr. Lonky: You mentioned that neurologic of Vulvovaginal Disease.5 The terminology diseases can sometimes cause vulvar pain. lists two major categories of vulvar pain: Which ones? • pain related to a specific disorder.This Dr. Edwards: Pudendal neuralgia, diabetic category encompasses numerous condi- neuropathy, and post-herpetic neuralgia tions that feature an abnormal appearance are the most common specific neurologic of the vulva (table 1). causes of vulvar pain. Multiple sclerosis can • vulvodynia, in which the vulva appears also produce pain syndromes.