Lichen Sclerosus et Atrophicus Workup
- Author: Jeffrey Meffert, MD; Chief Editor: Dirk M Elston, MD more...
Laboratory Studies
Skin biopsy (punch preferred) is the primary study to perform. Despite the presence of autoantibodies described in several studies, an autoimmune (AI) workup (eg, antinuclear antibody, vitamin B-12 levels, thyroid function tests) is still not generally recommended because the frequency of multiple AI diseases associated with lichen sclerosus is not high enough to justify the expense of screening all patients. Work is now underway to identify any patient subsets or particular presentations of lichen sclerosus that would warrant AI screening. For the same reason, Borrelia antibody titers are not recommended, as they would not clearly influence therapy and, in most studies, are not strongly associated with lichen sclerosus, especially in the United States.
Imaging Studies
Imaging studies are not needed unless urinary obstruction secondary to severe, stenosing genital lichen sclerosus is present. Intravenous pyelogram might be appropriate in this situation.
Procedures
Punch biopsy in the most mature area of the lesion usually is diagnostic. In genital biopsies, snip excisions may suffice. Suturing the wound, especially when using braided suture, which is less likely to poke sensitive skin, leads to more rapid healing than allowing self-granulation. Ulcerative or vegetative genital lesions may need to be biopsied more than once to screen for squamous cell carcinoma. Epidermal hyperplasia and/or dysplasia associated with lichen sclerosus on vulvar biopsy specimens is associated with an increased risk of malignant transformation. Overexpression of wild-type p53 is also associated with increased cancer risk as is a human papillomavirus–associated increase in p16INK4A.[15]
Histologic Findings
Classic lichen sclerosus demonstrates a lichenoid infiltrate in the dermal-epidermal junction, compact hyperkeratosis with stratum corneum, which often is thicker than the greatly effaced epidermis. Remarkable edema in the papillary (upper) dermis is replaced by a dense, homogenous fibrosis as the lesion matures. Extensive and deeper biopsies may show areas more consistent with scleroderma than classic lichen sclerosus. Note the images below:
Typical lichen sclerosus histology demonstrating homogenized edematous papillary (upper) dermis and effaced epidermis.
Late lichen sclerosus may show less edema in the upper dermis and more sclerosis throughout the dermis. Involvement of the lower dermis or fat may occur in lichen sclerosus/scleroderma overlap presentations. Chan I, Oyama N, Neill SM, Wojnarowska F, Black MM, McGrath JA. Characterization of IgG autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Clin Exp Dermatol. Sep 2004;29(5):499-504. [Medline].
Regauer S, Liegl B, Reich O, Beham-Schmid C. Vasculitis in lichen sclerosus: an under recognized feature?. Histopathology. Sep 2004;45(3):237-44. [Medline].
Kowalewski C, Kozlowska A, Chan I, et al. Three-dimensional imaging reveals major changes in skin microvasculature in lipoid proteinosis and lichen sclerosus. J Dermatol Sci. Jun 2005;38(3):215-24. [Medline].
Li YZ, Wu Y, Zhang QH, Wang Y, Zhen JH, Li SL. Hypoxia-ischaemia is involved in the pathogenesis of vulvar lichen sclerosus. Clin Exp Dermatol. Dec 2009;34(8):e531-6. [Medline].
Hunger RE, Bronnimann M, Kappeler A, Mueller C, Braathen LR, Yawalkar N. Detection of perforin and granzyme B mRNA expressing cells in lichen sclerosus. Exp Dermatol. May 2007;16(5):416-20. [Medline].
Smith SD, Fischer G. Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series. Pediatr Dermatol. Nov-Dec 2009;26(6):725-9. [Medline].
Sherman V, McPherson T, Baldo M, Salim A, Gao XH, Wojnarowska F. The high rate of familial lichen sclerosus suggests a genetic contribution: an observational cohort study. J Eur Acad Dermatol Venereol. Feb 25 2010;[Medline].
De Vito JR, Merogi AJ, Vo T, et al. Role of Borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus: a PCR study of thirty-five cases. J Cutan Pathol. Aug 1996;23(4):350-8. [Medline].
Eisendle K, Grabner T, Kutzner H, Zelger B. Possible role of Borrelia burgdorferi sensu lato infection in lichen sclerosus. Arch Dermatol. May 2008;144(5):591-8. [Medline].
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. Nov 2008;144(11):1432-5. [Medline].
Zollinger T, Mertz KD, Schmid M, Schmitt A, Pfaltz M, Kempf W. Borrelia in granuloma annulare, morphea and lichen sclerosus: a PCR-based study and review of the literature. J Cutan Pathol. Dec 10 2009;[Medline].
Bjekic M, Sipetic S, Marinkovic J. Risk factors for genital lichen sclerosus in men. Br J Dermatol. Feb 2011;164(2):325-9. [Medline].
Gunthert AR, Faber M, Knappe G, Hellriegel S, Emons G. Early onset vulvar Lichen Sclerosus in premenopausal women and oral contraceptives. Eur J Obstet Gynecol Reprod Biol. Mar 2008;137(1):56-60. [Medline].
O'Mahony C, Yesudian PD, Stanley M. Imiquimod use in the genital area and development of lichen sclerosus and lichen planus. Int J STD AIDS. Mar 2010;21(3):219-21. [Medline].
Prowse DM, Ktori EN, Chandrasekaran D, Prapa A, Baithun S. Human papillomavirus-associated increase in p16INK4A expression in penile lichen sclerosus and squamous cell carcinoma. Br J Dermatol. Feb 2008;158(2):261-5. [Medline].
Stucker M, Grape J, Bechara FG, Hoffmann K, Altmeyer P. The outcome after cryosurgery and intralesional steroid injection in vulvar lichen sclerosus corresponds to preoperative histopathological findings. Dermatology. 2005;210(3):218-22. [Medline].
Kreuter A, Gambichler T. Narrowband UV-B phototherapy for extragenital lichen sclerosus. Arch Dermatol. Sep 2007;143(9):1213. [Medline].
Romero A, Hernandez-Nunez A, Cordoba-Guijarro S, Arias-Palomo D, Borbujo-Martinez J. Treatment of recalcitrant erosive vulvar lichen sclerosus with photodynamic therapy. J Am Acad Dermatol. Aug 2007;57(2 Suppl):S46-7. [Medline].
Vermaat H, Smienk F, Rustemeyer T, Bruynzeel DP, Kirtschig G. Anogenital allergic contact dermatitis, the role of spices and flavour allergy. Contact Dermatitis. Oct 2008;59(4):233-7. [Medline].
Fischer G, Rogers M. Treatment of childhood vulvar lichen sclerosus with potent topical corticosteroid. Pediatr Dermatol. May-Jun 1997;14(3):235-8. [Medline].
Kreuter A, Tigges C, Gaifullina R, Kirschke J, Altmeyer P, Gambichler T. Pulsed high-dose corticosteroids combined with low-dose methotrexate treatment in patients with refractory generalized extragenital lichen sclerosus. Arch Dermatol. Nov 2009;145(11):1303-8. [Medline].
Bornstein J, Heifetz S, Kellner Y, Stolar Z, Abramovici H. Clobetasol dipropionate 0.05% versus testosterone propionate 2% topical application for severe vulvar lichen sclerosus. Am J Obstet Gynecol. Jan 1998;178(1 Pt 1):80-4. [Medline].
Lindhagen T. Topical clobetasol propionate compared with placebo in the treatment of unretractable foreskin. Eur J Surg. Dec 1996;162(12):969-72. [Medline].
Jones RW, Sadler L, Grant S, Whineray J, Exeter M, Rowan D. Clinically identifying women with vulvar lichen sclerosus at increased risk of squamous cell carcinoma: a case-control study. J Reprod Med. Oct 2004;49(10):808-11. [Medline].
Olejek A, Kozak-Darmas I, Kellas-Sleczka S, Jarek A, Wiczkowski A, Krol W, et al. Chlamydia trachomatis infection in women with lichen sclerosus vulvae and vulvar cancer. Neuro Endocrinol Lett. 2009;30(5):671-4. [Medline].

