O&G · Gynaecological health — vulval disorders
Vulval dermatoses: lichen sclerosus & lichen planus — figure-of-eight distribution, the diagnostic biopsy rule, SCC risk and lifelong surveillance
Also known as Lichen sclerosus · Lichen planus (erosive) · Vulval lichen sclerosus · Vulvo-vaginal-gingival syndrome · Vulval dermatoses · ISSVD vulvar dermatoses
Exam-exhaustive FRANZCOG fellowship topic on the chronic inflammatory vulval dermatoses: lichen sclerosus (the figure-of-eight distribution, porcelain-white atrophic plaques, the autoimmune associations, the approximately 4 percent squamous cell carcinoma transformation rate, the ultrapotent topical corticosteroid clobetasol first-line treatment, and the lifelong surveillance), and erosive lichen planus (the vulvo-vaginal-gingival syndrome, vaginal adhesions, the lower but elevated SCC risk). The diagnostic biopsy rule, the re-biopsy triggers, and the ISSVD / 2016 European classification framework. Anchored to the British Association of Dermatologists 2018 guideline, the EuroGuiderm 2024 treatment guideline, and the Vieira-Baptista 2022 systematic review of vulvar cancer risk. RANZCOG-primary, globally tagged to MRCOG, ABOG, FRCSC and MRCPI.
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Target exams
Red flags
- A focal ulcer, raised erythematous or hyperkeratotic plaque, a non-healing erosion, a new lump or a treatment-resistant area in known lichen sclerosus or lichen planus is vulvar neoplasia until biopsy proves otherwise
- Postmenopausal bleeding or any bleeding from a vulval lesion is invasive squamous cell carcinoma or vulvar intraepithelial neoplasia until proven otherwise
- An acute exacerbation with superimposed pain, fissuring or discharge mandates reassessment for candidiasis, herpes simplex or a supervening carcinoma
- Coalesced labial adhesion obstructing urine flow is an acute complication of undertreated lichen sclerosus and needs urgent division
- Vaginal adhesions and synechiae are erosive lichen planus until proven otherwise and progress to permanent stenosis if untreated
A 62-year-old woman sits in your clinic describing ten years of vulval itch, splitting on defecation and dyspareunia. She has seen three doctors, been told it is thrush, and been given four courses of clotrimazole. On examination you see the porcelain-white atrophic plaques in a figure-of-eight distribution, with labial fusion, clitoral burying and an introitus narrowed to a finger. Your job is not to treat another imagined thrush — it is to confirm the diagnosis with a vulval biopsy, exclude neoplasia, treat with an ultrapotent topical corticosteroid ointment, and put her on lifelong surveillance. The biopsy rule, the clobetasol regimen, and the 4 percent SCC number are the three things an examiner wants you to defend.[1][3]
Overview and definition
The vulval dermatoses are a group of chronic inflammatory skin conditions affecting the vulva and (frequently) the perianal skin. The 2016 European guideline on vulval conditions groups them as dermatoses (lichen sclerosus, lichen planus, lichen simplex chronicus, eczema, psoriasis), and explicitly separates them from infective conditions, vulvar intraepithelial neoplasia, and the pain syndromes.[5]
Two dermatoses dominate the fellowship exam because they carry a measurable squamous cell carcinoma (SCC) risk and have specific first-line steroid treatment:[1][5]
- Lichen sclerosus (LS) — a chronic inflammatory mucocutaneous disease producing white atrophic plaques in a figure-of-eight distribution; spares the vaginal mucosa; carries an approximately 4 percent lifetime SCC risk.
- Erosive lichen planus (LP) — a chronic inflammatory mucocutaneous disease producing erosions, vaginal adhesions and the vulvo-vaginal-gingival syndrome; carries a lower but elevated SCC risk.
Why this matters — the burden in numbers
References12ShowHide
- [1]Lewis FM, Tatnall FM, Velangi SS, et al. British Association of Dermatologists guidelines for the management of lichen sclerosus, 2018. Br J Dermatol, 2018.PMID 29313888
- [2]Kirtschig G, Kinberger M, Kreuter A, et al. EuroGuiderm guideline on lichen sclerosus-Treatment of lichen sclerosus. J Eur Acad Dermatol Venereol, 2024.PMID 38822598
- [3]Vieira-Baptista P, Pérez-López FR, López-Baena MT, et al. Risk of Development of Vulvar Cancer in Women With Lichen Sclerosus or Lichen Planus: A Systematic Review. J Low Genit Tract Dis, 2022.PMID 35285455
- [4]Cooper SM, Gao XH, Powell JJ, Wojnarowska F Does treatment of vulvar lichen sclerosus influence its prognosis? Arch Dermatol, 2004.PMID 15210461
- [5]van der Meijden WI, Boffa MJ, Ter Harmsel WA, et al. 2016 European guideline for the management of vulval conditions. J Eur Acad Dermatol Venereol, 2017.PMID 28164373
- [6]Kirtschig G, Wakelin SH, Wojnarowska F Mucosal vulval lichen planus: outcome, clinical and laboratory features. J Eur Acad Dermatol Venereol, 2005.PMID 15857455
- [7]Mauskar MM, Marathe K, Venkatesan A, Schlosser BJ, Edwards L Vulvar diseases: Conditions in adults and children. J Am Acad Dermatol, 2020.PMID 31712170
- [8]Fergus KB, Lee AW, Baradaran N, et al. Pathophysiology, Clinical Manifestations, and Treatment of Lichen Sclerosus: A Systematic Review. Urology, 2020.PMID 31605681
- [9]De Luca DA, Papara C, Vorobyev A, et al. Lichen sclerosus: The 2023 update. Front Med (Lausanne), 2023.PMID 36873861
- [10]Kirtschig G Lichen Sclerosus-Presentation, Diagnosis and Management. Dtsch Arztebl Int, 2016.PMID 27232363
- [11]Wallace HJ Lichen sclerosus et atrophicus. Trans St Johns Hosp Dermatol Soc, 1971.PMID 5570266
- [12]Dunselman GA, Vermeulen N, Becker C, et al. ESHRE guideline: management of women with endometriosis. Hum Reprod, 2014.PMID 24435778