Anogenital pruritus: lichen sclerosus in children.
Lichen sclerosus is a chronic mucocutaneous inflammatory condition affecting both adults and children in a bimodal age distribution. Children can present as young as 6 months of age but average around 5 years. Females present with vulvar itching, soreness, dysuria, or gastrointestinal complaints, while males tend to have difficulty retracting the foreskin leading to phimosis. On examination, white smooth atrophic plaques are found in the anogenital region with atrophy and possible distortion of anatomy. LS in children has been commonly misdiagnosed as sexual abuse, leading to delay in appropriate diagnosis and unnecessary turmoil for families. It is a chronic relapsing and remitting condition possibly due to autoimmunity. When evaluating patients, a complete history can help guide which patients may benefit from a systemic evaluation for autoimmune disease. Although some patients have spontaneous resolution during puberty, many do not. There is a significant risk of squamous cell carcinoma developing in genital LS in adults possibly from chronic inflammation, delay in diagnosis, and delay in appropriate treatment. The risk of squamous cell carcinoma in pediatric onset LS is undefined. It is also unclear if effective control of cutaneous inflammation can decrease the risk of malignant transformation. Treatment is aimed at decreasing symptoms and returning involved skin to its normal appearance. Relapses are common. Ultrapotent topical corticosteroids are first line for the treatment of LS and can be used intermittently for years for flares. Topical tacrolimus or pimecrolimus are also good treatment options at controlling inflammation. Patients need to be monitored every 6 to 12 months even when asymptomatic because of the potential for development of malignancy. Given the distressing nature of LS, support groups and a multidisciplinary approach are recommended.
Poindexter
Gabriele
G
Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA. GPoindex@unch.unc.edu
Morrell
Dean S
DS
D001004:Anus Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D001327:Autoimmune Diseases / Q000150:complications; D002294:Carcinoma, Squamous Cell / Q000209:etiology; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D005831:Genital Diseases, Female / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D005832:Genital Diseases, Male / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007223:Infant; D018459:Lichen Sclerosus et Atrophicus / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D008297:Male; D011379:Prognosis; D011537:Pruritus / Q000209:etiology*; D012307:Risk Factors
D007166:Immunosuppressive Agents