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lichen sclerosus

Etiology: - obesity & phimosis in males [8] - idiopathic in women Epidemiology: 1) most common cause of vulvar burning & itching in elderly women 2) causes 1/3 of benign vulvar lesions [3] 3) rare in men circumcised at birth - documented in cases of urostomy, ileostomy, hypospadias, & hypospadias repair [8] Pathology: - irritation from urinary occlusion may play a role in men [8] - precursor to vulvar intraepithelial neoplasia [3] Clinical manifestations: - may appear alone or superimposed lichen simplex chronicus. - vulvitis - may be symptomatic or asymptomatic [10] - vulvar burning & itching, vulvar pain - dysuria, dyspareunia - anal pruritus & pain [10] - may appear as white macules or ivory pallor - circumferentially involves vaginal introitus & perirectal area - ivory patches & plaques extending to the perineal body - telangiectasias involving the entire vulva may be present - vulvar skin appears thin & wrinkled 'cigarette paper' - loss of landmarks, labia majora & labia minora may fuse - flattening & disappearance of clitoral hood - introitus may be stenotic - extends to perirectal areas (hourglass or butterfly appearance) - perirectal skin may be thin & white [1] - without vaginal involvement [1] * images [5] Laboratory: - punch biopsy confirms diagnosis Differential diagnosis: - lichen planus (70% with vaginal involvement) - lichen simplex chronicus Complications: - scarring can progress around the introitus, exacerbating sexual dysfunction [10] - associated with squamous cell carcinoma (4-5%) [3] Management: 1) high potency topical glucocorticoids (most effective) a) clobetasol 0.05% QD-BID for 4 weeks [1] (8-12 weeks [3]), then gradually taper over 2-3 months [1,3] b) halobetasol c) clobetasol 0.05% QD for 1-3 months for men 2) follow-up in 3 & 6 months, then ever 6-12 months until resolution [8] 3) alternatives less effective than clobetasol [8] a) topical testosterone propionate (2%) BID for 1-2 weeks b) tacrolimus 0.1% BID, topical, for 16 weeks [2] 4) treat obesity & phimosis in males [8]

Related

lichen planus lichenification lichenoid eruption

Specific

lichen sclerosus et atrophicus (LSA)

General

vulvar dystrophy (vulvar disorder) lichenoid dermatitis chronic gynecologic disease

References

  1. Geriatrics Review Syllabus, American Geriatrics Society, 5th edition, 2002-2004; 7th edition 2010 - Geriatric Review Syllabus, 9th edition (GRS9) Medinal-Walpole A, Pacala JT, Porter JF (eds) American Geriatrics Society, 2016 - Geriatric Review Syllabus, 10th edition (GRS10) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2019 - Geriatric Review Syllabus, 11th edition (GRS11) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2022
  2. Hengge UR 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, 155:1021 PMID: 17034535
  3. Geriatrics at your Fingertips, 13th edition, 2011 Reuben DB et al (eds) American Geriatric Society
  4. Murphy R. Lichen sclerosus. Dermatol Clin. 2010 Oct;28(4):707-15 PMID: 20883914
  5. DermNet NZ: Lichen sclerosus (images) http://www.dermnetnz.org/immune/lichen-sclerosus.html
  6. Virgili A, Borghi A, Minghetti S, Corazza M. Mometasone fuoroate 0.1% ointment in the treatment of vulvar lichen sclerosus: a study of efficacy and safety on a large cohort of patients. J Eur Acad Dermatol Venereol. 2014 Jul;28(7):943-8. PMID: 23879234
  7. McPherson T, Cooper S. Vulval lichen sclerosus and lichen planus. Dermatol Ther. 2010 Sep-Oct;23(5):523-32 PMID: 20868406
  8. Akel R, Fuller C. Updates in lichen sclerosus: British Association of Dermatologists guidelines for the management of lichen sclerosus 2018. Br J Dermatol. 2018 Apr;178(4):823-824. PMID: 29668094 https://onlinelibrary.wiley.com/doi/full/10.1111/bjd.16445
  9. Medical Knowledge Self Assessment Program (MKSAP) 18, American College of Physicians, Philadelphia 2018
  10. Welch K, Saunders N, Parker-Featherstone E et al Clinical Profile of Vulvar Lichen Sclerosus NEJM Journal Watch. Nov 13, 2020 Massachusetts Medical Society (subscription needed) http://www.jwatch.org - Lee A, Fischer G. Diagnosis and treatment of vulvar lichen sclerosus: An update for dermatologists. Am J Clin Dermatol 2018 Oct; 19:695 PMID: 29987650 https://link.springer.com/article/10.1007/s40257-018-0364-7 - Lewis FM et al. British Association of Dermatologists guidelines for the management of lichen sclerosus, 2018. Br J Dermatol 2018 Apr; 178:839 PMID: 29313888 https://onlinelibrary.wiley.com/doi/full/10.1111/bjd.16241 - American College of Obstetricians and Gynecologists' Committee on Practice Bulletins - Gynecology. Diagnosis and Management of Vulvar Skin Disorders: ACOG Practice Bulletin, Number 224. Obstet Gynecol 2020 Jul; 136:e1. PMID: 32590724 https://journals.lww.com/greenjournal/Abstract/2020/07000/Diagnosis_and_Management_of_Vulvar_Skin_Disorders_.55.aspx - Funaro D. Lichen sclerosus: A review and practical approach. Dermatol Ther 2004; 17:28 PMID: 14756888 https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1396-0296.2004.04004.x - Welch KC et al. How to perform a vulvar biopsy. OBG Manage 2020 Jun; 32:14. Not indexed in PubMed https://www.mdedge.com/obgyn/article/223325/gynecology/how-perform-vulvar-biopsy - Regauer S et al. Early vulvar lichen sclerosus: A histopathological challenge. Histopathology 2005 Oct; 47:340. PMID: 16178888 Review. - Dalziel KL et al. The treatment of vulval lichen sclerosus with a very potent topical steroid (clobetasol propionate 0.05%) cream. Br J Dermatol 1991 May; 124:461. PMID: 2039723 - Chibnall R. Vulvar pruritus and lichen simplex chronicus. Obstet Gynecol Clin North Am 2017 Sep; 44:379. PMID: 28778638 https://www.sciencedirect.com/science/article/abs/pii/S0889854517300700
  11. NIAMS: Lichen Sclerosus https://www.niams.nih.gov/health-topics/lichen-sclerosus