Int J Biol Sci 2019; 15(7):1429-1439. doi:10.7150/ijbs.34613

Review

Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets

Davis A. Tran1, Xiaohui Tan1, Charles J. Macri2, Andrew T. Goldstein2,3✉, Sidney W. Fu1✉

1. Department of Medicine (Division of Genomic Medicine), and Department of Microbiology, Immunology and Tropical Medicine, The George Washington University School of Medicine and Health Sciences, Washington, DC
2. Department of Obstetrics and Gynecology, The George Washington University School of Medicine and Health Sciences, Washington, DC
3. Center for Vulvovaginal Disorders, Washington, DC

Abstract

Lichen sclerosus (LS) is an inflammatory dermatosis with a predilection for anogenital skin. Developing lesions lead to vulvar pain and sexual dysfunction, with a significant loss of structural anatomical architecture, sclerosis, and increased risk of malignancy. Onset may occur at any age in both sexes, but typically affects more females than males, presenting in a bimodal fashion among pre-pubertal children and middle-aged adults. A definitive cure remains elusive as the exact pathogenesis of LS remains unknown. A general review of LS, histologic challenges, along with amounting support for LS as an autoimmune disease with preference for a Th1 immune response against a genetic background is summarized. In addition to the classically referenced ECM1 (extracellular matrix protein 1), a following discussion of other immune and genetic targets more recently implicated as causative or accelerant agents of disease, particularly miR-155, downstream targets of ECM1, galectin-7, p53, and epigenetic modifications to CDKN2A, are addressed from the viewpoint of their involvement in three different, but interconnected aspects of LS pathology. Collectively, these emerging targets serve not only as inherently potential therapeutic targets for treatment, but may also provide further insight into this debilitating and cryptic disease.

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How to cite this article:
Tran DA, Tan X, Macri CJ, Goldstein AT, Fu SW. Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets. Int J Biol Sci 2019; 15(7):1429-1439. doi:10.7150/ijbs.34613. Available from http://www.ijbs.com/v15p1429.htm