Lichen sclerosus is a chronic inflammatory

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1 DERMATOLOGY Lichen Sclerosus et Atrophicus in a Young Girl YS Marfatia*, SoNIA JAIN** Abstract Lichen sclerosus et atrophicus is a chronic inflammatory dermatosis that results in white plaques and epidermal atrophy. The condition has both genital and extragenital presentations. Here we describe the case of a 12-year-old girl who presented to us with white plaques over her genitals and no manifestation of extragenital disease. Keywords: Lichen sclerosus et atrophicus, lichen albus, white spot disease Lichen sclerosus is a chronic inflammatory dermatosis that most commonly affects the anogenital region and leads to intractable pruritus and soreness. The condition is more common in females 1 as in the present case also the patient is a young girl who presented with complaints of white plaques with itching over the genitals since her prepubertal years. The condition involves the risk of malignant transformation more so over the genital lesions but the precise incidence has not been defined. Pathophysiologically, the condition is associated with the presence of autoantibodies to glycoprotein extracellular matrix protein 1 (ECM-1). 2 Several risk factors have also been proposed including autoimmune diseases, infections and genetic predisposition. 3 There is evidence of its association with thyroid disease. 4 Figure 1. Photograph showing labial atrophy. CASE SUMMARY A 12-year-old young girl presented to us with depigmented patches over the genitals, which had an insidious onset and were gradually progressive over a period of one year (Fig. 1). She had moderate itching over the site and she had seen many doctors for her complaints but had no relief. On dermatological examination, labia majora showed atrophy along with depigmentation of the labia minora (Fig. 2). The *Professor and Head Dept. of Skin and VD, Baroda Medical College SSG Hospital, Raopura, Vadodara, Gujarat **Professor Dept. of Skin and VD MGIMS, Sewagram, Wardha, Maharashtra Address for correspondence Dr Sonia Jain A-14, Dhanvantri Nagar MGIMS, Sewagram, Wardha, Maharashtra soniapjain@rediffmail.com Figure 2. Photograph showing depigmented patch over the labia. depigmented patches extended from the fourchette to the vestibule and she had no oral or cutaneous lesions elsewhere. There was no history of sexual abuse or any high-risk behavior and none of the family members Indian Journal of Clinical Practice, Vol. 24, No. 6, November

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4 DERMATOLOGY abuse associated with a higher incidence of LSA in pediatric population. Besides, they also described the association of LSA with infection, autoimmunity and trauma. There has been a case on the records wherein Virdi and Kanwar reported the co-existence of localized cutaneous morphea with LSA and submucosal fibrosis in a middle-aged man in his late-thirties. 5 Another citation of a similar association between the aforesaid conditions has been mentioned by Prasad and Padmavathy et al, wherein they reported a case of a young male in his mid-twenties who presented with generalized morphea and LSA along with osteolytic bone changes. 6 However, our patient showed no signs of morphea or any other systemic disease. Figure 3. Histology showing homogenization of the collagen and inflammatory infiltrate in the dermis. had a similar clinical picture. She had no urinary or bowel complaints. We performed a labial biopsy from the depigmented patch after taking a written informed consent and the histopathological findings were consistent with Lichen sclerosus et atrophicus (LSA) showing homogenization of the collagen and inflammatory infiltrate in the dermis (Fig. 3). DISCUSSION Lichen sclerosus is also known as LSA, balanitis xerotica obliterans (BXO) in men, Csillag s disease, White spot disease, Lichen albus and Krauosis vulvae. LSA was first described in 1887 by Dr Hallopeau. A case series of 42 children (all females) suffering from LSA has been reported by Shirley A Warrington and Camille de San Lazaro where they found a high incidence of sexual REFERENCES 1. Tasker GL, Wojnarowska F. Lichen sclerosus. Clin Exp Dermatol 2003;28(2): 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 2004;29(5): Yesudian PD, Sugunendran H, Bates CM, O Mahony C. Lichen sclerosus. Int J STD AIDS 2005;16(7):465-73, test Birenbaum DL, Young RC. High prevalence of thyroid disease in patients with lichen sclerosus. J Reprod Med 2007;52(1): Virdi SK, Kanwar AJ. Generalized morphea, lichen sclerosis et atrophicus associated with oral submucosal fibrosis in an adult male. Indian J Dermatol Venereol Leprol 2009;75(1): Prasad PV, Padmavathy L, Sethurajan S, Kumar P, Rao L. Generalised morphoea with lichen sclerosus et atrophicus and unusual bone changes. Indian J Dermatol Venereol Leprol 1995;61(2): Cont d from page De La Blanchardiere A, Rozenberg F, Caumes E, Picard O, Lionnet F, Livartowski J, et al. Neurological complications of varicellazoster virus infection in adults with human immunodeficiency virus infection. Scand J Infect Dis 2000;32(3): Vafai A, Berger M. Zoster in patients infected with HIV: a review. Am J Med Sci 2001;321(6): Gershon AA, Mervish N, LaRussa P, Steinberg S, Lo SH, Hodes D, et al. Varicella-zoster virus infection in children with underlying human immunodeficiency virus infection. J Infect Dis 1997;176(6): Burke DG, Kalayjian RC, Vann VR, Madreperla SA, Shick HE, Leonard DG. Polymerase chain reaction detection and clinical significance of varicella-zoster virus in cerebrospinal fluid from human immunodeficiency virusinfected patients. J Infect Dis 1997;176(4): Friedman-Kien AE, Lafleur FL, Gendler E, Hennessey NP, Montagna R, Halbert S, et al. Herpes zoster: a possible early clinical sign for development of AIDS in high risk individual. J Am Acad Dermatol 1986;14(6): Blank LJ, Polydefkis MJ, Moore RD, Gebo KA. Herpes zoster among persons living with HIV in the current antiretroviral therapy era. J Acquir Immune Defic Syndr 2012;61(2): De Castro N, Carmagnat M, Kernéis S, Scieux C, Rabian C, Molina JM. Varicella-zoster virus-specific cell-mediated immune responses in HIV-infected adults. AIDS Res Hum Retroviruses 2011;27(10): Noonan L, Gunson T, Ellis-Pegler R, Thomas M, Briggs S. Short-course intravenous aciclovir treatment for cutaneous herpes zoster in patients with HIV infection. Int J STD AIDS 2012;23(5): Indian Journal of Clinical Practice, Vol. 24, No. 6, November 2013

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6 GASTROENTEROLOGY 536 Indian Journal of Clinical Practice, Vol. 24, No. 3, August 2013 EDGE India reprint/galvus/cvm/097/11/13