ORIGINAL RESEARCH ARTICLE:VULVA AND VAGINA Clinicopathologic Diagnostic Criteria for Vulvar Lichen Planus Tania Day, MD, PhD,1,2 Edward Wilkinson, MD,3 Darion Rowan, FACD,4 and James Scurry, FRCPA,1,5 for the ISSVD Difficult Pathologic Diagnoses Committee* Lichen planus at any site is estimated to affect 2% of women, Objective: The aim of the study was to describe the clinical and histopath- with the oral cavity most commonly involved.5 Vulvovaginal LP ologic features required for a clinicopathologic diagnosis of vulvar lichen occurs in 25% to 57% of women with oral LP, causes 6% of planus (LP), which is divided into 3 types: erosive, classic, and hypertrophic. chronic vaginal complaints in postmenopausal women, and is his- Materials and Methods: The International Society of the Study of tologically confirmed in 3.7% of women attending a multidisciplin- Vulvovaginal Diseases tasked the Difficult Pathologic Diagnoses commit- ary vulvar clinic.6–8 Multiple factors contribute to underestimation of tee with development of a consensus document for the clinicopathologic prevalence: some cases are asymptomatic, women defer care seeking, 08/17/2020 on BhDMf5ePHKbH4TTImqenVBMw8cOsTrAm31mBmE+A1czBq2ga3kGGqBufqZk6iwZpO93TnKa60V8= by http://journals.lww.com/jlgtd from Downloaded diagnosis of vulvar LP,lichen sclerosus, and differentiated vulvar intraepi- and medical practitioners fail to make the diagnosis.8,9 thelial neoplasia. The LP subgroup reviewed the literature and formulated Three types of LP occur on the vulva: erosive, classic, and Downloaded diagnostic criteria, then approved by the International Society of the Study hypertrophic.10–12 The most common is likely to be the erosive of Vulvovaginal Diseases membership. form, followed by hypertrophic, and then classic. However, this is from Results: The clinicopathologic diagnosis of erosive LP incorporates 5 difficult to ascertain given previous studies' lack of clinical and his- http://journals.lww.com/jlgtd criteria: (a) a well-demarcated, glazed red macule or patch at labia minora, topathologic discernment between the 3 types.8 The aims of this vestibule, and/or vagina, (b) disease affects hairless skin, mucocutaneous consensus statement are to describe the clinical features and histo- junction, and/or nonkeratinized squamous epithelium, (c) evidence of basal pathologic findings that yield a diagnosis of vulvar LP and to pro- layer damage, categorized as degenerative or regenerative, (d)acloselyap- vide recommendations that facilitate clinicopathologic correlation. plied band-like lymphocytic infiltrate, and (e) absent subepithelial sclero- by sis. The clinicopathologic diagnoses of classic and hypertrophic LP each BhDMf5ePHKbH4TTImqenVBMw8cOsTrAm31mBmE+A1czBq2ga3kGGqBufqZk6iwZpO93TnKa60V8= require a characteristic clinical appearance accompanied by hyperkeratosis, METHODS hypergranulosis, acanthosis, basal layer degeneration, a closely applied The International Society of the Study of Vulvovaginal Dis- lymphocytic infiltrate, and absent dermal sclerosis, with hypertrophic LP eases (ISSVD) tasked the Difficult Pathologic Diagnoses commit- showing marked epithelial abnormality compared with classic LP. tee with development of a consensus document for the diagnosis Conclusions: Clinicopathological correlation yields the most reliable di- of vulvar LP, lichen sclerosus (LS), and differentiated vulvar in- agnosis of vulvar LP.Disease appearance overlaps with other physiologic, traepithelial neoplasia (dVIN). The LP subgroup performed a dermatologic, infectious, and neoplastic entities; a low threshold for biopsy literature review using the search terms “vulvar,”“vulval,” at all morphologically distinct areas is recommended. Use of the histopath- “vulvovaginal,”“vulvovaginal-gingival,” and “lichen planus,” ologic criteria described in this document may reduce the nondiagnostic bi- restricted to articles published during or after 1990. There were opsy rate for clinically diagnosed LP. 286 publications, of which 68 (24%) were case reports or letters to Key Words: vulva, vagina, erosive lichen planus, classic lichen planus, the editor, 85 (30%) were focused on a different condition, and 46 hypertrophic lichen planus, regenerative, degenerative (16%) were reviews or guidelines. Thirty-one (11%) original re- search articles addressed the clinical and/or pathologic diagno- – (J Low Genit Tract Dis 2020;24: 317 329) sis of LP; the remainder focused on etiology, epidemiology, comorbidities, management, and outcomes. The committee ap- ichen planus (LP) is a T-cell–mediated chronic inflammatory 1 praised the pertinent publications, synthesized them into a critical L skin disorder. The pathophysiology involves epitopic alteration review, then generated diagnostic criteria and recommendations. of epithelial basal cells, leading to lymphocytic attack and a cycle of 2 After the LP group reached agreement, the article was disseminated on cellular damage and repair. The reason for epitopic alteration is within the committee and underwent further revisions to achieve 08/17/2020 unknown, but a similar phenomenon occurs in graft-versus-host dis- 3,4 consensus. The document then was approved by vote of the ISSVD ease (GVHD) and lichenoid drug reactions. The histopathologic membership. Signed written consents were obtained for use of the — manifestation of this process is a lichenoid tissue reaction a clinical photographs. band of lymphocytes adjacent to damaged epithelium. Epidemiology and symptoms 1Faculty of Health and Medicine, University of Newcastle, New South Wales, Vulvar LP usually affects postmenopausal women. The mean Australia; 2Maternity and Gynaecology, John Hunter Hospital, Newcastle, 3 age in retrospective cohort studies restricted to erosive disease is New South Wales, Australia; Department of Pathology, Immunology, and 8,13–17 Lab Medicine, University of Florida College of Medicine, Gainesville, FL; 57 to 67 years. The diagnosis is occasionally made in 4Omnicare Women's Health, Auckland, New Zealand; and 5Pathology NSW reproductive-age women, with lower ages of 26 to 34 years in pub- Hunter New England, Newcastle, New South Wales, Australia lished ranges.13–17 Women with vulvovaginal-gingival syndrome Reprint requests to: Tania Day, MD, PhD, Maternity and Gynaecology John of LP seem to have an earlier age of onset, and disease may mani- Hunter Hospital, 2 Lookout Rd, New Lambton Heights, NSW 2305, 18–20 Australia. E-mail: [email protected] fest in adolescence. The mean age of women with nonerosive The authors have declared that there are no conflicts of interest. LP is 63 years, with a wide range of 21 to 88 years.11 * Jill Albritton, Tania Day, Debra S. Heller, Claudia Perrera, Gianluigi Radici, The clinical presentation of vulvar LP is described primarily Darion Rowan, Maria Angelica Selim, James Scurry, Kathryn Welch, Edward Wilkinson, and Mario Preti. in single-center retrospective cohorts based on a clinical diagno- sis. Some studies combine erosive and nonerosive LP; these do Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on – behalf of the ASCCP. This is an open-access article distributed under the not uniformly report location and type.6,21 23 Symptoms most terms of the Creative Commons Attribution-Non Commercial-No Derivatives commonly attributed to erosive LP are pain (50%–92%) and License 4.0 (CCBY-NC-ND), where it is permissible to download and share – 13,14,16,18,21,22 the work provided it is properly cited. The work cannot be changed in any pruritus (42% 65%). Dyspareunia is reported in 52%–91%, but a case note audit found less than half of women way or used commercially without permission from the journal. – DOI: 10.1097/LGT.0000000000000532 had documentation of sexual impacts.14,15,18,21 23 Other symptoms Journal of Lower Genital Tract Disease • Volume 24, Number 3, July 2020 317 Day et al. Journal of Lower Genital Tract Disease • Volume 24, Number 3, July 2020 FIG 1. Erosive LP: bilateral well-demarcated glazed red patch over vestibule and inner labia minora with a variable white edge and a white plaque on the right lateral border. include vaginal discharge (8%–24%) and dysuria (8%–23%).14,21,22 plaques, a white papules or plaques surrounded by the red patch, In 2 cohorts that combine erosive and nonerosive disease, 10% to and white delineation at the junction between normal and abnor- 34% of women were asymptomatic.6,8 A study restricted to nonerosive mal epithelium (see Figures 1–3). LP found pruritus as the main symptom in 81% of women, with Vaginal disease is reported in 20% to 85% of women with the remainder reporting pain (13%) or no symptoms (6%).11 vulvar erosive LP, but there is limited information on its appearance.14–16,18,21–23 Studies report a spectrum of findings: “telangiectasias and patchy erythema,”“superficial erosions,” Evaluation “painful friable hemorrhagic mucosa,” and “a variable discharge, Vulvovaginal Examination—Erosive LP. Awell- which is often serosanguinous.”10,20 Most studies do not identify demarcated, glazed red macule or patch at the labia minora, rates of vaginal synechia and obliteration; those that do report vestibule, and/or vagina is present in 81% to 97% of women adhesions in 10% to 50%.18,20,22 In absence of scarring, vaginal with a clinical diagnosis of erosive LP14,16,22 (see Figure 1). erythema and friability may be difficult to distinguish from The color is red-to-purple, rather than the orange-red associated desquamative inflammatory vaginitis (DIV) and severe vulvovaginal with plasma