Vulvar lichen planus
- Susan M Cooper, MB ChB, MRCGP, FRCP, MD
Susan M Cooper, MB ChB, MRCGP, FRCP, MD
- Consultant Dermatologist and Honorary Senior Clinical Lecturer
- Oxford University Hospitals
- Stephanie J Arnold, FACD, MBBS (Hons), BSc (Hons)
Stephanie J Arnold, FACD, MBBS (Hons), BSc (Hons)
- Consultant Dermatologist
- Churchill Hospital Department of Dermatology, Oxford
- Section Editors
- Robert L Barbieri, MD
Robert L Barbieri, MD
- Editor-in-Chief — Obstetrics, Gynecology and Women's Health
- Section Editor — General Gynecology and Female Reproductive Endocrinology
- Kate Macy Ladd Professor of Obstetrics, Gynecology and Reproductive Biology
- Harvard Medical School
- Robert P Dellavalle, MD, PhD, MSPH
Robert P Dellavalle, MD, PhD, MSPH
- Section Editor — General Dermatology
- Professor of Dermatology and Public Health
- University of Colorado School of Medicine
- Colorado School of Public Health
- Chief, Dermatology Service
- US Department of Veterans Affairs
- Eastern Colorado Health Care System
Lichen planus is a relatively uncommon inflammatory dermatologic condition with subtypes that can affect the skin, mucosa, nails, and scalp. Vulvar lichen planus is a subtype of lichen planus that is characterized by erosive, papular, or hypertrophic lesions on the vulva, with or without concomitant vaginal involvement.
Erosive lichen planus (also known as mucosal lichen planus) can result in severe tissue destruction that leads to vulvar pain and urinary and sexual impairment. Erosive lichen planus also can affect other mucosal sites, such as the oral cavity, nasal mucosa, esophagus, larynx, conjunctiva, and urethra .
High quality studies to guide treatment of vulvar lichen planus are lacking. Superpotent topical corticosteroids are well accepted as the first-line treatment for erosive disease.
Vulvar lichen planus will be discussed here. Other clinical presentations of lichen planus are reviewed separately. (See "Lichen planus", section on 'Clinical features' and "Oral lichen planus: Pathogenesis, clinical features, and diagnosis".)
Lichen planus is estimated to affect 0.5 to 2 percent of the population; estimates have varied based upon geographic location and diagnostic criteria . The incidence and prevalence of vulvar lichen planus has not been clearly established, but vulvar disease may be a common manifestation of lichen planus in women. In one series of 37 women diagnosed with lichen planus, vulvar lesions were present in 51 percent .
- Eisen D. The evaluation of cutaneous, genital, scalp, nail, esophageal, and ocular involvement in patients with oral lichen planus. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999; 88:431.
- Schlosser BJ. Lichen planus and lichenoid reactions of the oral mucosa. Dermatol Ther 2010; 23:251.
- Lewis FM, Shah M, Harrington CI. Vulval involvement in lichen planus: a study of 37 women. Br J Dermatol 1996; 135:89.
- Cooper SM, Ali I, Baldo M, Wojnarowska F. The association of lichen sclerosus and erosive lichen planus of the vulva with autoimmune disease: a case-control study. Arch Dermatol 2008; 144:1432.
- Ball SB, Wojnarowska F. Vulvar dermatoses: lichen sclerosus, lichen planus, and vulval dermatitis/lichen simplex chronicus. Semin Cutan Med Surg 1998; 17:182.
- Kennedy CM, Galask RP. Erosive vulvar lichen planus: retrospective review of characteristics and outcomes in 113 patients seen in a vulvar specialty clinic. J Reprod Med 2007; 52:43.
- Cooper SM, Wojnarowska F. Influence of treatment of erosive lichen planus of the vulva on its prognosis. Arch Dermatol 2006; 142:289.
- Cooper SM, Haefner HK, Abrahams-Gessel S, Margesson LJ. Vulvovaginal lichen planus treatment: a survey of current practices. Arch Dermatol 2008; 144:1520.
- Simpson RC, Thomas KS, Leighton P, Murphy R. Diagnostic criteria for erosive lichen planus affecting the vulva: an international electronic-Delphi consensus exercise. Br J Dermatol 2013; 169:337.
- 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 2006; 86:433.
- 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 2006; 155:994.
- Chew A, Stefanato CM, Savarese I, et al. Clinical patterns of lichen planopilaris in patients with vulval lichen planus. Br J Dermatol 2014; 170:218.
- Lewis FM. Vulval lichen planus. Br J Dermatol 1998; 138:569.
- Neill SM. Erosive lichen planus:diagnosis and management. Syllabus of the post-graduate course, International Society for the Study of Vulvovaginal Disease, October 1999.
- Grunwald MH, Zvulunov A, Halevy S. Lichen planopilaris of the vulva. Br J Dermatol 1997; 136:477.
- Pecoraro V, Romano Boix E. [Anal manifestations of lichen planus]. Med Cutan Ibero Lat Am 1984; 12:339.
- Ridley CM. Chronic erosive vulval disease. Clin Exp Dermatol 1990; 15:245.
- Zendell K, Edwards L. Lichen sclerosus with vaginal involvement: report of 2 cases and review of the literature. JAMA Dermatol 2013; 149:1199.
- Eisen D. The vulvovaginal-gingival syndrome of lichen planus. The clinical characteristics of 22 patients. Arch Dermatol 1994; 130:1379.
- Pelisse M. Erosive vulvar lichen planus and desquamative vaginitis. Semin Dermatol 1996; 15:47.
- Pelisse M, Leibowitch M, Sedel D, Hewitt J. [A new vulvovaginogingival syndrome. Plurimucous erosive lichen planus]. Ann Dermatol Venereol 1982; 109:797.
- Pelisse M. The vulvo-vaginal-gingival syndrome. A new form of erosive lichen planus. Int J Dermatol 1989; 28:381.
- Rogers RS 3rd, Eisen D. Erosive oral lichen planus with genital lesions: the vulvovaginal-gingival syndrome and the peno-gingival syndrome. Dermatol Clin 2003; 21:91.
- Setterfield JF, Neill S, Shirlaw PJ, et al. The vulvovaginal gingival syndrome: a severe subgroup of lichen planus with characteristic clinical features and a novel association with the class II HLA DQB1*0201 allele. J Am Acad Dermatol 2006; 55:98.
- Ridley CM, Neill SM. Non-infective cutaneous conditions of the vulva. In: The Vulva, Ridley CM, Neill SM (Eds), Blackwell Science, Oxford 1999. p.121.
- Byrd JA, Davis MD, Rogers RS 3rd. Recalcitrant symptomatic vulvar lichen planus: response to topical tacrolimus. Arch Dermatol 2004; 140:715.
- Weedon D. The lichenoid reaction pattern (interface dermatitis). In: Weedon's Skin Pathology, 3rd ed, Elsevier Limited, Edinburgh 2010. p.35.
- Helander SD, Rogers RS 3rd. The sensitivity and specificity of direct immunofluorescence testing in disorders of mucous membranes. J Am Acad Dermatol 1994; 30:65.
- Cheng H, Oakley A, Rowan D, Lamont D. Diagnostic criteria in 72 women with erosive vulvovaginal lichen planus. Australas J Dermatol 2015.
- Murphy GM, Cronin E. Lichen planus pemphigoides. Clin Exp Dermatol 1989; 14:322.
- Woodruff JD, Sussman J, Shakfeh S. Vulvitis circumscripta plasmocellularis. A report of four cases. J Reprod Med 1989; 34:369.
- Davis J, Shapiro L, Baral J. Vulvitis circumscripta plasmacellularis. J Am Acad Dermatol 1983; 8:413.
- Virgili A, Levratti A, Marzola A, Corazza M. Retrospective histopathologic reevaluation of 18 cases of plasma cell vulvitis. J Reprod Med 2005; 50:3.
- Edwards L. Vulvar lichen planus. Arch Dermatol 1989; 125:1677.
- Sobel JD. Desquamative inflammatory vaginitis: a new subgroup of purulent vaginitis responsive to topical 2% clindamycin therapy. Am J Obstet Gynecol 1994; 171:1215.
- Neill SM, Lewis FM. Vulvovaginal lichen planus: a disease in need of a unified approach. Arch Dermatol 2008; 144:1502.
- Friedrich EG. Vulvar disease, WB Saunders, Philadelphia 1983. p.174.
- Martin J, Holdstock G. Isolated vulval oedema as a feature of Crohn's disease. J Obstet Gynaecol 1997; 17:92.
- Ridley CM, Neill SM. Genital Dermatology, Edwards L (Ed), Lippincott Williams & Wilkins, Philadelphia 2004. p.14.
- Silverman S Jr, Gorsky M, Lozada-Nur F. A prospective follow-up study of 570 patients with oral lichen planus: persistence, remission, and malignant association. Oral Surg Oral Med Oral Pathol 1985; 60:30.
- Fitzpatrick SG, Hirsch SA, Gordon SC. The malignant transformation of oral lichen planus and oral lichenoid lesions: a systematic review. J Am Dent Assoc 2014; 145:45.
- Jänner M, Muissus E, Rohde B. [Lichen planus as a possible precancerous condition]. Dermatol Wochenschr 1967; 153:513.
- Wallace HJ. Lichen sclerosus et atrophicus. Trans St Johns Hosp Dermatol Soc 1971; 57:9.
- Lewis FM, Harrington CI. Squamous cell carcinoma arising in vulval lichen planus. Br J Dermatol 1994; 131:703.
- Dwyer CM, Kerr RE, Millan DW. Squamous carcinoma following lichen planus of the vulva. Clin Exp Dermatol 1995; 20:171.
- Zaki I, Dalziel KL, Solomonsz FA, Stevens A. The under-reporting of skin disease in association with squamous cell carcinoma of the vulva. Clin Exp Dermatol 1996; 21:334.
- Kennedy CM, Peterson LB, Galask RP. Erosive vulvar lichen planus: a cohort at risk for cancer? J Reprod Med 2008; 53:781.
- Simpson RC, Murphy R. Is vulval erosive lichen planus a premalignant condition? Arch Dermatol 2012; 148:1314.
- Regauer S, Reich O, Eberz B. Vulvar cancers in women with vulvar lichen planus: a clinicopathological study. J Am Acad Dermatol 2014; 71:698.
- Cheng H, Oakley A, Conaglen JV, Conaglen HM. Quality of Life and Sexual Distress in Women With Erosive Vulvovaginal Lichen Planus. J Low Genit Tract Dis 2017; 21:145.
- Al-Hashimi I, Schifter M, Lockhart PB, et al. Oral lichen planus and oral lichenoid lesions: diagnostic and therapeutic considerations. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007; 103 Suppl:S25.e1.
- McPherson T, Cooper S. Vulval lichen sclerosus and lichen planus. Dermatol Ther 2010; 23:523.
- Savage NW. Oral lichenoid drug eruptions. Oral Dis 1997; 3:55.
- Bradford J, Fischer G. Management of vulvovaginal lichen planus: a new approach. J Low Genit Tract Dis 2013; 17:28.
- Voûte AB, Schulten EA, Langendijk PN, et al. Fluocinonide in an adhesive base for treatment of oral lichen planus. A double-blind, placebo-controlled clinical study. Oral Surg Oral Med Oral Pathol 1993; 75:181.
- Santegoets LA, Helmerhorst TJ, van der Meijden WI. A retrospective study of 95 women with a clinical diagnosis of genital lichen planus. J Low Genit Tract Dis 2010; 14:323.
- Simpson RC, Littlewood SM, Cooper SM, et al. Real-life experience of managing vulval erosive lichen planus: a case-based review and U.K. multicentre case note audit. Br J Dermatol 2012; 167:85.
- Kirtschig G, Van Der Meulen AJ, Ion Lipan JW, Stoof TJ. Successful treatment of erosive vulvovaginal lichen planus with topical tacrolimus. Br J Dermatol 2002; 147:625.
- Lotery HE, Galask RP. Erosive lichen planus of the vulva and vagina. Obstet Gynecol 2003; 101:1121.
- Jensen JT, Bird M, Leclair CM. Patient satisfaction after the treatment of vulvovaginal erosive lichen planus with topical clobetasol and tacrolimus: a survey study. Am J Obstet Gynecol 2004; 190:1759.
- Lonsdale-Eccles AA, Velangi S. Topical pimecrolimus in the treatment of genital lichen planus: a prospective case series. Br J Dermatol 2005; 153:390.
- Edwards L. Erosive and vesiculobullous diseases. In: Genital Dermatology Atlas, 2nd ed, Edwards L, Lynch PJ (Eds), Wolters Kluwer, Philadelphia 2011. p.125.
- Bowen AR, Vester A, Marsden L, et al. The role of vulvar skin biopsy in the evaluation of chronic vulvar pain. Am J Obstet Gynecol 2008; 199:467.e1.
- Deen K, McMeniman E. Mycophenolate mofetil in erosive genital lichen planus: a case and review of the literature. J Dermatol 2015; 42:311.
- Mann MS, Kaufman RH. Erosive lichen planus of the vulva. Clin Obstet Gynecol 1991; 34:605.
- Anderson M, Kutzner S, Kaufman RH. Treatment of vulvovaginal lichen planus with vaginal hydrocortisone suppositories. Obstet Gynecol 2002; 100:359.
- Helgesen AL, Warloe T, Pripp AH, et al. Vulvovaginal photodynamic therapy vs. topical corticosteroids in genital erosive lichen planus: a randomized controlled trial. Br J Dermatol 2015; 173:1156.
- CLINICAL MANIFESTATIONS
- Erosive lichen planus
- Papulosquamous lichen planus
- Hypertrophic lichen planus
- Lichen planopilaris
- Clinical evaluation
- Proposed diagnostic criteria
- DIFFERENTIAL DIAGNOSIS
- NATURAL HISTORY
- Associated malignancy
- Erosive vulvar lichen planus
- - Adjunctive measures
- - First-line therapy
- Topical corticosteroids
- - Efficacy
- - Administration
- - Second-line therapy
- Topical tacrolimus
- - Efficacy
- - Administration
- - Severe erosive lichen planus
- - Refractory disease
- - Vaginal involvement
- Intravaginal corticosteroids
- Other therapies
- - Adhesions and scarring
- - Emerging therapy
- Treatment of hypertrophic and papulosquamous lichen planus
- Treatment of vulvo-vaginal-gingival syndrome
- Treatment of concurrent infection
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS