Poindexter Gabriele, Morrell Dean S
Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
Pediatr Ann. 2007 Dec;36(12):785-91. doi: 10.3928/0090-4481-20071201-07.
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.
硬化性苔藓是一种慢性黏膜皮肤炎症性疾病,在成人和儿童中呈双峰年龄分布。儿童最早可在6个月时发病,但平均发病年龄约为5岁。女性患者表现为外阴瘙痒、疼痛、排尿困难或胃肠道不适,而男性患者往往存在包皮回缩困难,导致包茎。检查时,在肛门生殖器区域可发现白色光滑的萎缩性斑块,伴有萎缩和可能的解剖结构变形。儿童期的硬化性苔藓常被误诊为性虐待,导致诊断延误,给家庭带来不必要的困扰。它是一种慢性复发和缓解性疾病,可能与自身免疫有关。在评估患者时,完整的病史有助于指导哪些患者可能从自身免疫性疾病的系统评估中获益。虽然一些患者在青春期可自发缓解,但许多患者并非如此。成人生殖器硬化性苔藓有发生鳞状细胞癌的显著风险,可能是由于慢性炎症、诊断延误和治疗不当所致。儿童期发病的硬化性苔藓发生鳞状细胞癌的风险尚不明确。目前也不清楚有效控制皮肤炎症是否能降低恶变风险。治疗旨在减轻症状,使受累皮肤恢复正常外观。复发很常见。超强效外用糖皮质激素是治疗硬化性苔藓的一线药物,可间歇性使用数年以控制病情发作。外用他克莫司或吡美莫司在控制炎症方面也是不错的治疗选择。由于存在恶变的可能性,即使患者无症状,也需要每6至12个月进行监测。鉴于硬化性苔藓令人痛苦的特性,建议成立支持小组并采用多学科方法进行治疗。