Sexuality Matters The Vulvar Dermatoses Part of the Differential Diagnosis for Sexual Dysfunction Jill M. Krapf, MD; Andrew Goldstein, MD Vulvar complaints can be an uncomfortable discussion not only for the patient but also for the health care provider. Because vulvar dermatoses fall between two, often disparate, specialties—gynecology and dermatology—clinicians may feel poorly trained to identify and treat these disorders. Vulvar dermatoses must be considered as a part of the differential diagnosis for any woman with sexual dysfunction or pain. ulvar dermatoses can interfere with sexual function because of discomfort, pain, and embarrassment. Chronic vulvar conditions impact not only a woman’s sexual well-being but also her overall quality of life.1 As women become more comfortable with vulvar health, they will seek the advice of their physicians, especially about their sexual health. Gynecologists must be prepared to diagnose and treat vulvar conditions, including chronic vulvar skin disorders. This review will discuss the diagnosis and treatment of common vulvar dermatoses, with a focus on how these conditions affect sexual wellbeing in women. V An Approach to the Vulva Most women do not realize that vulvovaginal symptoms are common, and in turn, may feel isolated due to their condition. In this solitude, women might fear that their symptoms represent cancer or a sexually transmitted disease.2 Vulvovaginal disease can significantly affect sexual well-being, including sexual function and intimacy. Frustration and depression are common in most chronic pain conditions and should Follow The Female Patient on and be a consideration with chronic vulvar disease as well.3 A comprehensive history and physical examination are essential in the management of vulvar conditions. A full Jill M. Krapf, MD, is Adjunct Instructor, The George Washington University School of Medicine and Health Sciences, Washington, DC. Andrew Goldstein, MD, is Associate Clinical Professor, The George Washington University School of Medicine and Health Sciences; Director, Centers for Vulvovaginal Disorders, Washington, DC, and New York, NY; President, The International Society for the Study of Women’s Sexual Health. The Female Patient | VOL 37 APRIL 2012 1 The Vulvar Dermatoses in pigmentation, and scarring. A cotton swab may be used to detect tenderness or decreased sensation. Examination with a colposcope can be very useful, and we highly recommend its use. A speculum examination is necessary to detect vaginal findings such as ulceration, synechiae, loss of rugae, pallor, and petechiae. Vaginal discharge should be obtained for cultures, wet mount, and pH assessment. Abnormalities warrant a vulvar biopsy; many vulvar dermatologic conditions cannot be diagnosed by mere observation. A general examination, including skin, eyes, and mouth, should be performed as some vulvar dermatoses are associated with autoimmune conditions and extra-genital lesions. A systematic approach to physical examination is essential when considering the differential diagnosis of vulvar presentations. Irritant and Contact Dermatitis FIGURE 1. Contact dermatitis occurs when an irritant or allergen causes inflammation, presenting clinically in a range from mild erythema and swelling to severe erythema, fissures, skin thickening, erosion, and ulceration. sexual history, including current sexual practices and infections, is crucial. Eliciting use of topical or over-the-counter medications is important. A history of genital surgery, including labiaplasty, which is becoming much more common, should also be noted. It is also important to ask about vulvar trauma. Lastly, exercise regimens and vulvovaginal care habits should be discussed. Examination of the vulva should be systematic and thorough, performed in dorsal lithotomy position with proper lighting. A mirror may be helpful to allow the patient to communicate the location of concern. Observation of the vulva includes identification of atrophy, erythema, induration, fissures, lichenification, ulceration, erosions, changes 2 The Female Patient | VOL 37 APRIL 2012 Contact dermatitis is one of the most common and often avoidable problems. The incidence is approximately 15% to 30%; however, with increased use of over-the-counter vulvar products, the incidence is rising.4 Exogenous agents cause inflammation of the skin. Common irritants and allergens include body fluids, menstrual pads, heat, soaps and detergents, antibiotics, douches, fragrances, nickel (from piercings), rubber, and spermicides.5 In addition, semen can also act as an irritant or allergen. Clinical examination may reveal a range of fi ndings from mild erythema and swelling to severe erythema, fissures, skin thickening, erosion and ulceration (Figure 1).6 A detailed history and physical examination are keys to diagnosis; however, physicians should have a low threshold for biopsy to rule out coexisting conditions. With continued exposure or chronic scratching, lichen simplex chronicus may develop. Identification and removal of the causative agent is the main goal of treatment. It is essential to counsel patients on proper vulvar hygiene. Inflammation may be alleviated with topical steroids, All articles are available online at www.femalepatient.com Krapf and Goldstein including triamcinolone 0.1% ointment twice daily for moderate cases and clobetasol 0.05% ointment once daily for severe cases. Ice packs and antihistamines, such as hydroxyzine, are helpful for vulvar pruritus. Scratching during sleep can be especially difficult to treat. Low-dose tricyclic antidepressants, such as amitriptyline, may be given at bedtime for this purpose. Patients should be examined 1 month after initiating treatment, with steroids and antidepressants tapered with resolution of symptoms. Superimposed fungal and bacterial infections are common and should be treated. Patients who do not respond to treatment will need reevaluation and a biopsy to exclude other conditions. Lichen Simplex Chronicus Lichen simplex chronicus of the vulva is a chronic eczematous condition characterized by intense and unrelenting pruritus, leading to scratching and lichenification (Figure 2). The disorder represents an end-stage response to a causative process. Excoriation and fissures can become infected with yeast or bacteria. A biopsy is often necessary to exclude lichen sclerosus, lichen planus, or vulvar intraepithelial neoplasia.3 Lichen simplex chronicus affects quality of life, impacting both psychological and sexual well-being. The condition has been associated with psychological problems, including demoralization, depression, anxiety, obsessive-compulsive disorder, and sleep disturbances. Compared to matched controls, women with lichen simplex chronicus demonstrated significantly lower scores on the Female Sexual Function Index, especially in the domain scores of desire, arousal, lubrication, orgasm, and sexual satisfaction.1 Identifying and eliminating all irritant and allergen exposure is the first step in treatment of lichen simplex chronicus. It is also essential to break the itchscratch-itch cycle, which can be difficult as women may scratch in their sleep. Nighttime pruritus may be alleviated with oral amitriptyline at bedtime and FIGURE 2. Lichen simplex chronicus is a chronic eczematous condition characterized by intense and unrelenting pruritus, leading to scratching and lichenification. application of ice. Inflammation may be treated with topical application of high potency corticosteroids. Concomitant infections should be treated accordingly. Lichen Sclerosus Lichen sclerosus is a chronic inflammatory skin disorder that affects approximately 1 in 70 women, usually presenting in premenarchal girls and menopausal women.3 Vulvar irritation is the most common presenting symptom, although lichen sclerosus may also be entirely asymptomatic and found incidentally on examination. With disease progression, scratching and sclerotic changes lead to erosions and fissures; progressive scarring results in narrowing of the introitus, resorption of the labia minora, and phimosis of the clitoris (Figure 3). Although the exact pathogenesis of lichen sclerosus is unclear, the condition is generally accepted as The Female Patient | VOL 37 APRIL 2012 3 The Vulvar Dermatoses formed before treatment with topical steroids is initiated. First-line treatment is clobetasol propionate 0.05% ointment applied once daily at night for 4 weeks, followed by alternate nights for 4 weeks, then twice weekly for 4 weeks. Patients should follow up 2 to 3 months after initiating treatment, followed by 6 months, and then seen annually if disease is well controlled.7 Approximately 60% of patients will experience complete remission of their symptoms with this regimen.10 The topical calcineurin inhibitors tacrolimus and pimecrolimus have been studied, but given their unclear long-term safety profiles, they are not considered first-line treatment.11,12 Lichen Planus FIGURE 3. Lichen sclerosus is a chronic inflammatory skin disorder that has a waxy or “cigarette paper” appearance. With disease progression, scratching and sclerotic changes may lead to erosions, fissures, and narrowing of the introitus. an autoimmune disorder. There is a 5% associated risk of vulvar squamous cell carcinoma, and it is unclear if treatment decreases this risk.7 Chronic vulvar pain has been reported by 79% of women with lichen sclerosus.3 Of all quality of life domains, sexual function is most impacted. Lichen sclerosus can cause sexual dysfunction, with introital dyspareunia and decreased sexual activity. This disorder has been shown to cause sexual distress by affecting desire, arousal, lubrication, orgasm, satisfaction, and pain.8 However, treatment of lichen sclerosus does improve sexual dysfunction.9 Physical examination reveals ivory white atrophic plaques with a “cigarette paper” appearance. Although vulvar lichen sclerosus can be a clinical diagnosis, skin changes may be difficult to differentiate from vulvar intraepithelial neoplasia, and a biopsy should be per- 4 The Female Patient | VOL 37 APRIL 2012 Lichen planus, an autoimmune inflammatory mucocutaneous disorder, affects approximately 1% of women with a peak incidence from age 30 to 60 years.12 Patients may present with pruritus, burning, dyspareunia, postcoital bleeding, or vaginal discharge. This disease severely affects sexual interaction. Nearly 8% of women examined for evaluation of vulvar pain were found to have lichen planus. Ninety-five percent of women reported sexual dysfunction, with dyspareunia in 60% and apareunia in 35% of women.13 Erosive lichen planus presents as glassy, brightly erythematous erosions accompanied by white striae (Wickham’s striae). The disease may markedly alter the vulvovaginal anatomy resulting in loss of the labia minora, narrowing of the introitus, and obliteration of the vagina (Figure 4). Patients frequently report a copious yellow vaginal discharge. Lichen planus can be misdiagnosed as lichen sclerosus. Unlike lichen planus, lichen sclerosus has a waxy or “cigarette paper” appearance and rarely displays vaginal involvement. It is important to remember that lichen sclerosus and lichen planus may coexist in the same patient. Vulvar and vaginal lichen planus are difficult to treat; lesions are relatively resistant to available therapies. Firstline treatment is topical clabetasol pro- Krapf and Goldstein pionate 0.05%. Daily treatment should be continued until lesions have resolved and then slowly tapered, with a limit of 3 months. Soaking in warm water may aid in penetration of the topical through heavily keratinized lesions.14 Vaginal lichen planus may be treated with intravaginal hydrocortisone suppositories to prevent obliteration of the vagina. Calcineurin inhibitors have also been used for vulvovaginal lichen planus with good success. When topical medications fail, the next step is systemic treatment with an oral corticosteroid, 40 to 60 mg per day for 2 to 4 weeks. Although not recommended for active lichen planus, in cases of severe scarring, surgery to lyse vulvovaginal adhesions may be necessary to restore a woman’s sexual function.14 The Challenge Vulvar pain and dyspareunia are common presenting complaints in the office setting. Vulvar dermatoses must be considered as a part of the differential diagnosis for any woman with sexual dysfunction or pain. A detailed history and physical examination, backed by a confident knowledge of the vulvar dermatoses, will aid in diagnosis and treatment. The authors report no actual or potential conflicts of interest in relation to this article. References 1. Ermertcan AT, Gencoglan G, Temeltas G, Horasan GD, Deveci A, Ozturk F. Sexual dysfunction in female patients with neurodermatitis. J Androl. 2011;32(2):165-169. 2. Margesson LJ. Vulvar disease pearls. Dermatol Clin. 2006;24(2):145-155. 3. Burrows LJ, Shaw HA, Goldstein AT. The vulvar dermatoses. J Sex Med. 2008;5(2):276-283. 4. Beecker J. Therapeutic principles in vulvovaginal dermatology. Dermatol Clin. 2010;28(4):639-648. 5. Bhate K, Landeck L, Gonzalez E, Neumann K, Schalock P. Genital contact dermatitis: a retrospective analysis. Dermatitis. 2010;21(6):317-320. 6. American College of Obstetricians and Gynecologists. ACOG Practice Bulletin No. 93: diagnosis and management of vulvar skin disorders. Obstet Gynecol. 2008;111(5):1243-1253. 7. Murphy R. Lichen sclerosus. Dermatol Clin. 2010;28(4):707-715. FIGURE 4. Lichen planus is an autoimmune inflammatory mucocutaneous disorder targeting oral and vulvovaginal mucosa. Erosive lichen planus presents as glassy, brightly erythematous erosions accompanied by white striae (Wickham’s striae). Loss of the labia minora, narrowing of the introitus, and obliteration of the vagina occur with disease progression. 8. Van de Nieuwenhof HP, Meeuwis KA, Nieboer TE, Vergeer MC, Massuger LF, De Hullu JA. The effect of vulvar lichen sclerosus on quality of life and sexual functioning. J Psychosom Obstet Gynaecol. 2010;31(4):279-284. 9. Burrows LJ, Creasey A, Goldstein AT. The treatment of vulvar lichen sclerosus and female sexual dysfunction [published online ahead of print October 18, 2010]. J Sex Med. 2011;8(1):219-222. doi: 10.1111/j.17436109.2010.02077.x. 10. Neill SM, Lewis FM, Tatnall FM, Cox NH. British Association of Dermatologists’ guidelines for the management of lichen sclerosus 2010. Br J Dermatol. 2010;163(4):672-682. 11. Hengge UR, Krause W, Hofmann H, et al. Multicentre, phase II trial on the safety and efficacy of topical tacrolimus ointment for the treatment of lichen sclerosus. Br J Dermatol. 2006;155(5):1021-1028. 12. Goldstein AT, Creasey A, Pfau R, Phillips D, Burrows LJ. A double-blind, randomized controlled trial of clobetasol versus pimecrolimus in patients with vulvar lichen sclerosus. J Am Acad Dermatol. 2011;4(6):e99-104. 13. Cooper SM, Haefner HK, Abrahams-Gessel S, Margesson LJ. Vulvovaginal lichen planus treatment: a survey of current practices. Arch Dermatol. 2008;144(11):1520-1521. 14. Goldstein AT, Metz A. Vulvar lichen planus. Clin Obstet Gynecol. 2005;48(4):818-823. The Female Patient | VOL 37 APRIL 2012 5 The Vulvar Dermatoses Coding for The Vulvar Dermatoses: Part of the Differential Diagnosis for Sexual Dysfunction Philip N. Eskew Jr, MD This important article discusses a clinical condition that requires a great deal of time discussing the impact on a patient’s life. If seen during an annual examination, you should request that the patient return for a visit where you can not only obtain a comprehensive history but also perform indicated biopsies. You may need to wait on the biopsy results before you submit your claim. Several of the ICD-9 codes mentioned in this article are: 692 693 625.0 625.7 625.9 697.0 698.1 Contact dermatitis and other eczema (692.0 – 692.9) Dermatitis due to substances taken internally (693.0 – 693.9) Dyspareunia Vulvodynia Unspecified symptom associated with female genital organs (chronic vulvar pain) Lichen planus (lichen planopilaris, ruber planus) Pruritus of genital organs 698.3 701.0 Lichenification and lichen simplex chronicus (Hyde’s disease, neurodermatitis [circumscripta] [local], prurigo nodularis) Circumscribed scleroderma (lichen sclerosus et atrophicus) As mentioned in the article, the use of the colposcope can be very helpful. The CPT codes for its use in examining the vulva are: 56820 Colposcopy of the vulva 56821 with biopsy(s) Philip N. Eskew Jr, MD, is past member, Current Procedural Terminology (CPT) Editorial Panel; past member, CPT Advisory Committee; past chair, ACOG Coding and Nomenclature Committee; and instructor, CPT coding and documentation courses and seminars. 6 The Female Patient | VOL 37 APRIL 2012
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