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Lichen Sclerosus Arch Dis Child: first published as 10.1136/adc.64.8.1204 on 1 August 1989. Downloaded from Archives of Disease in Childhood, 1989, 64, 1204-1206 Current topic Lichen sclerosus J BERTH-JONES, R A C GRAHAM-BROWN, AND D A BURNS Department of Dermatology, Leicester Royal Infirmary Lichen sclerosus, previously termed 'lichen sclerosus but the latter may be spared. When extragenital et atrophicus', is a uncommon disease that presents sites are affected there is usually anogenital disease to a wide variety of medical disciplines. It is a cause also. of much distress, not only because of its symptoms, The appearance of the vulva is usually diagnostic. but also, increasingly, because of a potential to be There are characteristic shiny white papules and misdiagnosed as sexual abuse.1-3 For those familiar plaques, with a semitranslucent appearance likened with lichen sclerosus in childhood it is usually to mother of pearl. The affected skin usually shows a possible to make a firm clinical diagnosis. tendency to fine wrinkling giving an appearance The incidence of lichen sclerosus is hard to termed 'cigarette paper skin'. In the more severe estimate as patients present to different specialties. cases purpura, excoriation, blistering (usually Mild cases may never come to receive medical haemorrhagic), telangiectasia, erosion, and bleeding attention, and others may remain misdiagnosed. may be present. When both the vulva and perianal The disease is much less common in children than in region are affected the disease often takes on ancopyright. adults: in a series of 290 cases only 20 developed the 'hour glass' or 'figure of eight' pattern. (see figure). disease below the age of 16.4 We currently have 14 Common presenting complaints are vulval sore- girls aged 15 or under suffering from lichen sclerosus ness, pruritus, bleeding, and haemorrhagic blisters. under our care in a dermatology unit serving a Pain on defaecation and dysuria, often associated population of 866 000, but the incidence is probably with crying and considerable distress on defaecation higher than this figure would suggest. and micturition, are common symptoms in young The well established association with organ specific patients.1 12 The tendency to avoid micturition may autoimmune disease has resulted in lichen sclerosus cause nocturnal enuresis, nocturia, and appreciable http://adc.bmj.com/ being regarded as an autoimmune phenomenon.56 sleep disturbance. Pain on defaecation may lead to There may also be a genetic component as several severe constipation. Exudation and vaginal discharge familial cases and a pair of identical affected twins may be prominent features, particularly during have been described.3 4 7 8 episodes of superinfection. The severity of the disease varies widely. It may Clinical features be virtually asymptomatic but in severe cases exacerbations develop in which the vulva becomes on September 25, 2021 by guest. Protected Onset may occur as early as 6 months of age.9 severely eroded, excoriated, and haemorrhagic. Lichen sclerosus occurs in both sexes but is generally Dysuria and pain on defaecation can be troublesome held to be much less common in males. Although and persistent. In its most severe form lichen the major clinical problem with lichen sclerosus is in sclerosus may lead to obliterative, atrophic scarring females, histological evidence of lichen sclerosus (in of the genitalia. the male often termed balanitis xerotica obliterans) can frequently be found after circumcision for Diagnosis scarring phimosis.10 Lichen sclerosus should there- fore always be considered in any boy presenting with There is usually little difficulty establishing the this condition. Circumcision is generally curative. diagnosis of childhood lichen sclerosus on the basis Lichen sclerosus has a strong predilection for the of the clinical features described above. In our anogenital region but may affect any area of the experience vulval biopsy is of no value and causes skin. unnecessary discomfort to the patient. This is In girls the most commonly seen pattern is because the histology of lichen sclerosus, although involvement of both the vulva and perianal region, characteristic, is often rather subtle, so that the 1204 Arch Dis Child: first published as 10.1136/adc.64.8.1204 on 1 August 1989. Downloaded from Lichen sclerosus 1205 Concern regarding sexual abuse As severe lichen sclerosus may cause excoriation, erosion, haemorrhage, blistering, or purpura of the vulva, it is perhaps not surprising that cases have been mistaken for sexual abuse. Several such cases have been reported,1 and others have undoubtedly occurred. Wallace described such a case in 1970, well before the current increased awareness of sexual abuse.4 It is well recognised that lichen sclerosus may develop at sites of trauma (that is, exhibit the isomorphic (Kobner) phenomenon).4 14 It has also been claimed that a child with lichen sclerosus is 'more likely to sustain injuries after abuse'.3 Not- withstanding these observations, it must be stated that there is absolutely no evidence to support any link between lichen sclerosus and sexual abuse. Indeed, the chances of these two problems ocurring together would seem very remote indeed. Regrettably, we have found that concern regarding misdiagnosis of lichen sclerosus as sexual abuse is now perceived not only by doctors but also by parents of affected girls. In our experience this has often resulted in hesitation before seeking medical advice. copyright. Treatment There is no established specific treatment for lichen sclerosus. When the disease is mild or asymptomatic no treatment is required. Cases with mild pruritis Figure Typical 'figure ofeight'pattern ofanogenitallichen and soreness benefit from the application of bland sclerosus. emollient creams such as E45 (Crookes Products), http://adc.bmj.com/ which are soothing. Mild topical steroids in com- bination with antibacterial and antifungal compounds diagnosis may easily be missed unless already (for example, Daktacort, Janssen; Terra-Cortril, suspected clinically. The most common histological Pfizer) also appear to be of value in speeding finding is a featureless band ofoedematous hyalinised resolution of exacerbations and preventing and collagen in the upper dermis.'3 There is often a treating bacterial or candidal superinfection. Lactu- lymphohistiocytic infiltrate in the deeper dermis lose is often useful in cases associated with pain on particularly around skin appendages such as hair defaecation, and more potent laxatives such as on September 25, 2021 by guest. Protected follicles. Rete pegs are short or absent. The epidermis senna may be required during exacerbations. is usually atrophic, and hyperkeratosis is sometimes In severe cases, the use of potent topical steroid present. These changes are often masked during creams, for example 0-05% fluocinonide cream exacerbations by acute inflammatory changes due to (Metosyn, Stuart), or 0*05% clobetasol propionate excoriation and superinfection. cream (Dermovate, Glaxo), is justified, applied There are few conditions that can be confused twice daily for short periods of up to three weeks. clinically with lichen sclerosus in childhood. Lichen The use of topical steroids in lichen sclerosus may be planus, although unusual, may occur at this age, but a source ofconcern, especially to non-dermatologists, mucosal lesions are usually delicate, lace like, white in view of the risk of adding steroid induced atrophy striae. Lichen simplex chronicus (circumscribed to the atrophic scarring of the disease. In our neurodermatitis) is more pruritic in nature, and the experience, however, short courses ofpotent steroids lesions are poorly circumscribed areas of hyperkera- produce considerable improvement. Furthermore, a tosis and excoriation lacking the specific features of recent study of seven adult cases treated with lichen sclerosus. Leucoplakia is not seen in childhood. clobetasol propionate cream twice daily for 12 weeks Arch Dis Child: first published as 10.1136/adc.64.8.1204 on 1 August 1989. Downloaded from 1206 Berth-Jones, Graham-Brown, and Burns showed histological improvement in all subjects sus et atrophicus in children misdiagnosed as sexual abuse. (KL Dalziel, PR Millard, FT Wojnarowska, personal Br Med J 1987;294:1404-5. communication). 2 Priestley BL, Bleehan SS. Lichen sclerosus et atrophicus in children misdiagnosed as sexual abuse. (Letter.) Br Med J 1987;295:21 1. Prognosis 3 Davidson DC, Clarke MDB, Kean HB. Lichen sclerosus et atrophicus in children misdiagnosed as sexual abuse. (Letter.) The prognosis is somewhat uncertain, but appears Br Med J 1987;295:211. 4 Wallace HJ. Lichen sclerosus et atrophicus. Transactions of considerably better than in adults, as resolution may the St John's Hospital Dermatological Society 1971;57:9-30. occur at or before puberty. Ditkowski et al recorded Harrington CI, Dunsmore IR. An investigation into the incidence complete remission in six of eight reported cases by of auto-immune disorders in patients with lichen sclerosus et the age of 14.15 Clark and Muller observed resolution, atrophicus. Br J Dermatol 1981;104:563-6. 6 Meyrick Thomas RH, Ridley CM, McGibbon DH, Black MM. which was usually complete, in 12 of 24 cases of Lichen sclerosus et atrophicus and autoimmunity-a study of anogenital disease, but found no consistent temporal 350 women. Br J Dermatol 1988;118:41-6. relation to the menarche.1' Wallace reported 7 Shirer JA, Ray MC. Familial occurrence of lichen sclerosus et complete resolution
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