Guideline sources The following summarized guidelines for the evaluation and management of lichen sclerosus (LS) are prepared by our editorial team based on guidelines from the American College of Obstetricians and Gynecologists (ACOG 2020), the British Association of Dermatologists (BAD 2018), and the European Academy of Dermatology and Venereology (EADV 2017; 2015). 1 2 3 4 Guidelines 1. Diagnostic investigations History and physical examination: As per ACOG 2020 guidelines, elicit a comprehensive medical history, perform a physical examination and obtain evaluation of abnormal vaginal discharge, if an infectious etiology is suspected, in the initial evaluation of patients with vulvovaginal symptoms. B As per BAD 2018 guidelines: https://web.pathway.md/diseases/recdyyjrnUjOzKIug 1/8 6/29/23, 5:52 AM Lichen sclerosus Pathway As per BAD 2018 guidelines: Elicit a full history including dyspareunia and psychosexual issues and document urinary symptoms in all patients with LS. E Perform a detailed physical examination documenting architectural changes at baseline using a diagram or photograph, according to the patient preference. E Evaluation for comorbidities: evaluate for autoimmune diseases if clinically indicated, as some diseases such as thyroid disease, pernicious anemia, vitiligo, diabetes mellitus are associated with LS in female patients. B 2. Diagnostic procedures Vulvar biopsy: Perform vulvar biopsy of visible lesions in any of the following circumstances: atypical lesions (such as new pigmentation, indurated, affixed to underlying tissue, bleeding, or ulcerated) concern for malignancy immunocompromised conditions (including human immunodeficiency virus) diagnostic uncertainty lesions not responding to standard therapy disease worsening during treatment. B 3. Medical management Indications for treatment: As per BAD 2018 guidelines: Manage all patients with LS by a healthcare professional experienced in treating the condition (secondary care specialist or general practitioner with specific training). E Initiate treatment in all patients with LS following a firm clinical diagnosis or with histological confirmation, where necessary. E As per EADV 2017 guidelines, treat patients with LS. Treat also patients not having itch but having clinical signs of LS. B Topical corticosteroids: As per ACOG 2020 guidelines: Administer medium-potency or high-potency topical corticosteroid ointment for the initial treatment of patients with LS. A Offer long-term, individualized topical corticosteroids to maintain normality of skin color and texture and to prevent scarring in patients with LS. B As per BAD 2018 guidelines, discuss the amount of topical treatment to be used, the site of application and the safe use of an ultra-potent topical corticosteroid with patients with LS. E Show 5 more https://web.pathway.md/diseases/recdyyjrnUjOzKIug 2/8 6/29/23, 5:52 AM Lichen sclerosus Pathway As per EADV 2017 guidelines: Administer potent or ultra-potent topical corticosteroids such as mometasone furoate or clobetasol propionate as first-line therapy in patients with genital LS. B Apply mometasone furoate 0.1% ointment twice weekly for maintaining remission, recognizing that it may help to prevent malignant changes. B As per EADV 2015 guidelines, administer very potent or potent topical corticosteroids in patients with genital LS. Consider administering proactive maintenance therapy for remission, often in female patients. B Show 2 more Topical calcineurin inhibitors: As per ACOG 2020 guidelines, consider offering topical calcineurin inhibitors (such as tacrolimus or pimecrolimus) in patients with confirmed LS not responding to topical or intralesional corticosteroids or in patients at risk of skin atrophy. C As per BAD 2018 guidelines, insufficient evidence to support the use of topical calcineurin inhibitors in patients with LS. I As per EADV 2017 guidelines, consider offering topical calcineurin inhibitors in patients with LS recognizing that these agents are not licensed for the treatment of LS: tacrolimus 0.1% ointment for genital B and extragenital LS both in males and females C tacrolimus 0.03% ointment for anogenital LS in pediatric patients C pimecrolimus 1% cream for vulvar LS. B As per EADV 2015 guidelines, consider offering topical tacrolimus in some patients with LS. B Show 2 more Topical hormone therapy: As per EADV 2017 guidelines, insufficient evidence to support the use of topical hormone therapy (progesterone or testosterone) in patients with vulvar LS. I As per EADV 2015 guidelines, insufficient evidence to support the use of topical testosterone, dihydrotestosterone or progesterone in patients with genital LS. I Retinoids: As per BAD 2018 guidelines, insufficient evidence to support the use of systemic retinoids in patients with LS. I As per EADV 2017 guidelines: Consider offering retinoids in patients with LS failed standard therapy, given only by a dermatologist experienced in the use of these agents. B Initiate effective contraception 1 month before treatment and continued throughout oral retinoid treatment and after until the retinoids left the patient's system: at least 1 month after stopping isotretinoin or alitretinoin at least 2 years after stopping acitretin. B https://web.pathway.md/diseases/recdyyjrnUjOzKIug 3/8 6/29/23, 5:52 AM Lichen sclerosus Pathway As per EADV 2015 guidelines, insufficient evidence to support the use of topical retinoids in patients with vulval LS. I Antihistamines: consider offering oxatomide for relieving pruritus in patients with vulvar LS. C Agents with no evidence for benefit: Insufficient evidence to support the use of the following topical treatments in patients with LS: topical vitamin E in vulval LS I human fibroblast lysate cream I topical TRPM8 agonist icilin. I Insufficient evidence to support the use of the following systemic treatments in patients with LS: hydroxycarbamide/hydroxyurea I cycloferon I fumarate I hydroxychloroquine I potassium para-aminobenzoate I oral vitamin A, E and D. I Management of co-occurring infections: consider administering a combination of corticosteroids with antibiotics and antifungals, such as gentamicin or fusidic acid and nystatin or azole antifungals or an alternative preparation, for a short period of time for the treatment of secondary infections in patients with vulvar LS. C 4. Nonpharmacologic interventions Skin care: As per BAD 2018 guidelines, advise avoiding irritant and fragrance products in all patients with LS. E As per EADV 2015 guidelines: Consider offering emollients for symptom relief in patients with LS after an initial treatment with topical corticosteroids. C Advise wearing silk rather than cotton briefs in patients with LS. B 5. Therapeutic procedures Ultraviolet phototherapy: As per EADV 2017 guidelines, consider offering phototherapy in selected patients with LS. B Show 2 more As per EADV 2015 guidelines: Consider offering UV A1 phototherapy in patients with vulvar LS, if topical corticosteroids fail. C https://web.pathway.md/diseases/recdyyjrnUjOzKIug 4/8 6/29/23, 5:52 AM Lichen sclerosus Pathway Recognize that the well-documented development of carcinomas after PUVA therapy and UV B gives cause of concern, particularly at the genital site. B Photodynamic therapy: Insufficient evidence to recommend photodynamic therapy in patients with LS. I Consider offering photodynamic therapy in patients with vulvar LS if standard therapy fails, recognizing that it is painful and time consuming. C Carbon dioxide laser therapy: consider offering CO laser treatment in patients with cutaneous and genital LS. C Intralesional corticosteroid injections: consider administering intralesional corticosteroid injections in patients with LS with treatment-resistant itch or in patients unable to apply topical corticosteroids. C Intralesional adalimumab injections: consider administering intralesional adalimumab injection in patients with LS failed other treatments. C Therapies with no evidence for benefit: Insufficient evidence to recommend the following therapies in patients with LS: focused ultrasound I stem cells I platelet rich plasma. I 6. Surgical interventions Indications for surgery, female: As per EADV 2017 guidelines: Perform surgery in patients with vulvar LS only for the treatment of coexistent VIN/SCC or fusion. B Recognize that vulvar LS tends to recur around the scar in female patients. B As per EADV 2015 guidelines, reserve vulvectomy for female patients with LS associated with vulvar malignancy. B Show 2 more Indications for surgery (male): consider performing circumcision with complete removal of the foreskin in male patients with genital LS limited to foreskin and glans. C Show 5 more 7. Specific circumstances Patients with extragenital lichen sclerosus: As per BAD 2018 guidelines, consider offering potent topical corticosteroids, acitretin, methotrexate and phototherapy in patients with extragenital LS. C https://web.pathway.md/diseases/recdyyjrnUjOzKIug 5/8 6/29/23, 5:52 AM Lichen sclerosus Pathway As per EADV 2017 guidelines: Consider offering potent topical corticosteroids and topical calcipotriol, possibly under occlusion, in patients with extragenital LS. C Consider offering UV A1 phototherapy as potential first-line therapy in patients with extragenital LS. B As per EADV 2015 guidelines, consider offering topical calcipotriol, possible under occlusion, in patients with extragenital LS if first-line treatments fail. C Show 2 more Pediatric patients, female: As per BAD 2018 guidelines, refer pediatric and young female patients with LS to specialized vulval services, such as vulval clinic, pediatric dermatologist or urologist experienced in the management of LS. E Show 4 more As per EADV 2015 guidelines: Consider offering topical tacrolimus 0.03% ointment in pediatric female patients with anogenital LS, and as a safe maintenance treatment (twice weekly) for reducing recurrences. C Consider offering pimecrolimus as an alternative to topical corticosteroids in pediatric and young female patients with vulvar LS. C Pediatric patients, male: As per BAD 2018 guidelines, offer a trial of an ultra-potent topical corticosteroid once daily for 1- 3 months combined with emollients and barrier preparations in pediatric male patients with phimosis caused by LS. A Show 3 more As per EADV 2015 guidelines: Consider performing circumcision with complete removal of the foreskin in most pediatric male patients with LS unless LS is present in hypospadias repair or in obese patients. C Perform surgery of the meatus if necessary (stenosis). Obtain follow-up at 6 months after the circumcision carefully investigating the meatus and glans. Reserve urethral surgery for special cases (such as hypospadia). B 8. Patient education General counseling: Provide all patients with LS with up-to-date patient information on the condition. E Advise obese male patients with a buried penis to lose weight. Consider referring to a urologist and bariatric services. E 9. Follow-up and surveillance https://web.pathway.md/diseases/recdyyjrnUjOzKIug 6/8 6/29/23, 5:52 AM Lichen sclerosus Pathway Indications for referral (gynecology): Consider referring all female patients with LS to a specialist vulval clinic if: not responding to a topical corticosteroid surgical management is being considered. E Indications for referral (urology): Consider referring male patients with LS to a urologist in the following situations with according purposes: Situation Guidance Urology opinion and further evaluation and management of LUTS E Urinary symptoms Circumcision B Phimosis not responding to an ultra-potent topical corticosteroid Clobetasol propionate, introduced into the urethra via a urinary catheter or meatal dilator depending on stricture length, before proceeding to surgical treatment options E Urethral stricture Urology opinion on other surgical treatment options, such as total or partial glans resurfacing and split-skin grafting. B Not responding to topical corticosteroids and/or circumcision Assessment of treatment response: As per ACOG 2020 guidelines, follow-up patients at 3 months following initial therapy to assess response to therapy and to ensure proper application of the medication. B As per BAD 2018 guidelines, follow-up all patients treated for LS to assess response to treatment and to advise on long-term control. E Patients with refractory disease: As per ACOG 2020 guidelines, consider administering intralesional corticosteroid injections in patients with LS poorly controlled or resistant to topical corticosteroids. C As per BAD 2018 guidelines: Consider offering a repeated course of topical treatment for 1-3 months in male patients with relapsed LS. E Consider administering intralesional triamcinolone 1-2 mg both in female and male patients with LS having topical corticosteroid-resistant hyperkeratotic areas, after the exclusion of intra- epithelial neoplasia or malignancy by biopsy. C As per EADV 2015 guidelines, consider offering retinoids in patients with LS if standard therapy fails. C Show 4 more https://web.pathway.md/diseases/recdyyjrnUjOzKIug 7/8 6/29/23, 5:52 AM Lichen sclerosus Pathway Clinical findings Symptoms Past medical history Constipation Alopecia areata Dyspareunia Hashimoto's disease Dysuria Localized scleroderma Erectile dysfunction Phimosis Inability to retract the foreskin Rheumatoid arthritis Pain with bowel movements SLE Penile itching Gynecologic exam Penile pain Vulvar itching Vulvar excoriations Vulvar pain Vulvar fissures Genitourinary exam Penile erosions Penile fissures Prepuce cannot be retracted over the glans penis Preputial scarring Vulvar erythema Vulvar hyperkeratosis Vulvar leukoderma Vulvar lichenification References 1. F M Lewis, F M Tatnall, S S Velangi et al. British Association of Dermatologists guidelines for the management of lichen sclerosus, 2018. Br J Dermatol. 2018 Apr;178 4 839 853. Open 2. G Kirtschig, K Becker, A G nthert et al. Evidence-based S3 Guideline on (anogenital) Lichen sclerosus. J Eur Acad Dermatol Venereol. 2015 Oct;29 10):e1 43. Open 3. 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 Jun;31 6 925 941. Open 4. No authors listed. Diagnosis and Management of Vulvar Skin Disorders: ACOG Practice Bulletin, Number 224. Obstet Gynecol. 2020 Jul;136 1 222 225. Open https://web.pathway.md/diseases/recdyyjrnUjOzKIug 8/8