A Pediatric Case of Disseminated Lichen Sclerosus – a Case Report

Abstract

Lichen sclerosus (LS) is an uncommon chronic inflammatory skin disorder with a predilection for the anogenital area, characterized by porcelain white papules, plaques and atrophic patches. We report a prepubertal, 12-year-old girl who presented with chronic, disseminated pearly, flat-topped papules, plaques and atrophic patches located on the trunk, limbs and in the anogenital area, consistent with LS based on clinical and histologic findings. Potent and ultrapotent topical corticosteroids should be considered as first-line treatment. The ultraviolet A1 (UVA1) and calcipotriol for extragenital lesions, as well as calcineurin inhibitors for anogenital lesions, are other treatment options for pediatric LS. Lichen sclerosus is an uncommon chronic inflammatory skin disorder with a predilection for the anogenital area, characterized by porcelain white papules, plaques or atrophic patches. It was first described in 1887, by Hallopeau. Since then, many synonyms have been in use, notably 'guttate scleroderma,' 'white spot disease,' 'dermatitis lichenoides chronica atrophicans" or Csillag's disease,' 'kraurosis vulvae,' 'vulvar dystrophy, 'balanitis xerotica obliterans', 'lichen albus or Von Zumbusch Disease', or 'lichen sclerosus et atrophicus'. Since not all cases of LS et atrophicus exhibit atrophic tissue, 'et atrophicus' was dropped and replaced by 'lichen sclerosus,' which is now used for genital and extragenital lesions (1). LS is mostly seen in females with two peak ages of presentation: prepubertal girls and postmenopausal women. The prevalence rate of vulvar LS ranges between 1:70 to 1:1000 in women and 1:900 in children (2, 3).The patogenesis of LS has not yet been completly elucidatet; however, genetic factors and autoimmunity have been implicated. The most common autoimmune diseases associated with LS were autoimmune thyroiditis (12 %), alopecia areata (9%), vitiligo (6%), and pernicious anemia (2%) (4, 5). Hormonal, environmental, and infectious factors have also been implicated as possible causes of this disease.Initially, the lesions are pearly, flat-topped papules that coalesce into plaques, over time becoming atrophic patches. Telangiectasias, follicular plugging and bullous lesions may also be seen. The most common location is the anogenital area, although extragenital lesions can be present, mostly affecting buttocks, breasts, submammary area, neck, back, chest, axillae and wrists. Oral mucosal involvement has also been reported. Extragenital lesions are rare, especially in children.We report a prepubertal female child with LS with genital and disseminated extragenital lesions. Case ReportA 12-year-old prepubertal girl with a 12-month history of numerous mildly pruritic hypochromic lesions distributed on the trunk, limbs, and genital area was referred to the University Clinic of Dermatology, Skopje, Macedonia. She had visited several physicians due to these complaints which were misdiagnosed as vitiligo, tinea, psoriasis and verrucae planae. She used various ointments such as