Joung Soo Kim, MD, PhD; Min Won Lee, MD; Jun Oh Paek, MD; Ho Song Kang, MD; Hee Joon Yu, MD, PhD From the Department of Dermatology, Hanyang University, Guri Hospital, South Korea. The authors report no conflict of interest. Correspondence: Hee Joon Yu, MD, PhD, Department of Dermatology, Hanyang University, Guri Hospital, Guri, Gyeonggi-do 471-701, South Korea. Bhattacharjee P, Fatteh SM, Lloyd KL. Squamous cell carcinoma arising in long-standing lichen sclerosus et atrophicus. J Am Geriatr Soc. Lichen sclerosus: a review and practical approach. In: Wolff K, Goldsmith L, Katz S, et al, eds. New York, NY: McGraw Hill; 2007:546-550. J Am Acad Dermatol. Cooper SM, Gao XH, Powell JJ, et al. Does treatment of vulvar lichen sclerosus influence its prognosis? Kagie MJ, Kenter GG, Hermans J, et al. The relevance of various vulvar epithelial changes in the early detection of squamous cell carcinoma of the vulva. Int J Gynecol Cancer. Thomas RH, Ridley CM, McGibbon DH, et al. Anogenital lichen sclerosus in women. J R Soc Med. Walkden V, Chia Y, Wojnarowska F. The association of squamous cell carcinoma of the vulva and lichen sclerosus: implications for follow-up. Tasker GL, Wojnarowska F. Wang SH, Chi CC, Wong YW, et al. Genital verrucous carcinoma is associated with lichen sclerosus: a retrospective study and review of the literature. J Eur Acad Dermatol Venereol. Vulvar intraepithelial neoplasia: historical aspects and current status. Int J Gynecol Pathol. Vulvar squamous cell carcinoma development after diagnosis of VIN increases with age. Taube JM, Badger J, Kong CS, et al. Differentiated simplex vulvar intraepithelial neoplasia: a case report and review of the literature. Derrick EK, Ridley CM, Kobza-Black A, et al. A clinical study of 23 cases of female anogenital carcinoma. Crum C, McLachlin CM, Tate JE, et al. Pathobiology of vulvar squamous neoplasia. Ansink AC, Krul MRL, De Weger RA, et al. Human papillomavirus, lichen sclerosus, and squamous cell carcinoma of the vulva: detection and prognostic significance. Hillemanns P, Wang X. Integration of HPV-16 and HPV-18 DNA in vulvar intraepithelial neoplasia. Human papillomaviruses and cervical neoplasia: a model for carcinogenesis. Int J Gynecol Pathol. To the Editor: Lichen sclerosus LS is a chronic inflammatory disorder of unknown etiology that most commonly affects the anogenital region. Progressive sclerosis results in scarring with distortion of the normal epithelial architecture. We report a case of VIN and SCC developing in a region of preexisting LS. Solitary, reddish,erosive nodule on the clitorissurrounded by a pearly white,smooth, glistening area. A 76-year-old woman presented with a 7-mm nodule on the clitoris that was surrounded by a pearly white, smooth, glistening area Figure 1. The patient reported pain and tenderness associated with the nodule. No regional lymphadenopathy was evident. We performed an excisional biopsy of the entire nodule and a small part of the whitish patch Figure 2A. On histologic examination, the presence of hyperkeratosis, epidermal atrophy, a swollen dermal collagen bundle, and prominent edema was consistent with LS Figure 2B. The presence of dysplastic changes with mild disturbance of the epithelial architecture as well as acanthosis and dyskeratosis in the same tissue confirmed VIN Figure 2C. Dermal invasion and transition to SCC were seen in the part of the tissue verified as VIN. The presence of dermal tumor nests and an irregular border between the epidermis and dermis pointed to the existence of fully developed SCC Figure 2D. To prevent the recurrence of SCC, the patient returned for follow-up periodically. There was no recurrence within 6 months after excision. Left, center, and right boxes indicate areas shown in Figures 2B, 2C and 2D, respectively. Although LS is considered a premalignant condition, only a small portion of patients with LS ultimately develop vulvar SCC. First, in the majority of cases of vulvar SCC, LS, squamous cell hyperplasia, or VIN is present in the adjacent epithelium. Lichen sclerosus is found in adjacent regions in up to 62% of vulvar SCC cases. Third, in a series of LS patients who underwent long-term follow-up, 4% to 6% were reported to have developed vulvar SCC. Its identification is hindered by a high degree of cellular differentiation combined with the absence of widespread architectural disorder, nuclear pleomorphism, and diffuse nuclear atypia. Vulvar SCC can be divided into 2 patterns. The first is found in older women, which is unrelated to human papillomavirus HPV. The second is predominantly found in younger women, which is related to high-risk HPV. This type of vulvar SCC frequently is associated with the histologic subtypes of warty and basaloid differentiations and is referred to as undifferentiated VIN. There is no association with LS in these cases. E6 and E7 have the ability to bind and inactivate the protein p53 and retinoblastoma protein, which promotes rapid progression through the cell cycle without p53-mediated control of DNA integrity. Bhattacharjee P, Fatteh SM, Lloyd KL. Squamous cell carcinoma arising in long-standing lichen sclerosus et atrophicus. J Am Geriatr Soc. Lichen sclerosus: a review and practical approach. In: Wolff K, Goldsmith L, Katz S, et al, eds. New York, NY: McGraw Hill; 2007:546-550. J Am Acad Dermatol. Cooper SM, Gao XH, Powell JJ, et al. Does treatment of vulvar lichen sclerosus influence its prognosis? Kagie MJ, Kenter GG, Hermans J, et al. The relevance of various vulvar epithelial changes in the early detection of squamous cell carcinoma of the vulva. Int J Gynecol Cancer. Thomas RH, Ridley CM, McGibbon DH, et al. Anogenital lichen sclerosus in women. J R Soc Med. Walkden V, Chia Y, Wojnarowska F. The association of squamous cell carcinoma of the vulva and lichen sclerosus: implications for follow-up. Tasker GL, Wojnarowska F. Wang SH, Chi CC, Wong YW, et al. Genital verrucous carcinoma is associated with lichen sclerosus: a retrospective study and review of the literature. J Eur Acad Dermatol Venereol. Vulvar intraepithelial neoplasia: historical aspects and current status. Int J Gynecol Pathol. Vulvar squamous cell carcinoma development after diagnosis of VIN increases with age. Taube JM, Badger J, Kong CS, et al. Differentiated simplex vulvar intraepithelial neoplasia: a case report and review of the literature. Derrick EK, Ridley CM, Kobza-Black A, et al. A clinical study of 23 cases of female anogenital carcinoma. Crum C, McLachlin CM, Tate JE, et al. Pathobiology of vulvar squamous neoplasia. Ansink AC, Krul MRL, De Weger RA, et al. Human papillomavirus, lichen sclerosus, and squamous cell carcinoma of the vulva: detection and prognostic significance. Hillemanns P, Wang X. Integration of HPV-16 and HPV-18 DNA in vulvar intraepithelial neoplasia. Human papillomaviruses and cervical neoplasia: a model for carcinogenesis. Int J Gynecol Pathol. MDedge: Your Source for Clinical Advantage © 2018 , Parsippany, NJ, USA. The information provided is for educational purposes only. Use of this Web site is subject to the and.