Changi General Hospital, Singapore city, Singapore.
Acta Paediatr. 2013 Nov;102(11):e514-8. doi: 10.1111/apa.12376. Epub 2013 Sep 2.
To describe an Asian population with isolated cutaneous Langerhans cell histiocytosis (LCH), presenting a review on previous studies on this subgroup of patients and comparing our cohort with other studies of cutaneous LCH with systemic involvement.
All patients diagnosed as LCH with cutaneous involvement presenting to a tertiary paediatric hospital (KK Women's & Children's Hospital) between January 2001 and December 2011 were reviewed. Information recorded included clinical presentation, investigation results, treatment and outcome.
We identified 10 patients with cutaneous manifestations of LCH. Six had isolated cutaneous LCH with no visceral or bony involvement, while four had cutaneous lesions with involvement of other organ systems. Of the patients with isolated skin involvement, three were treated with surgical excision, one with topical corticosteroids and two with multi-agent chemotherapy. The four patients with concomitant systemic involvement were treated with multi-agent chemotherapy. None of our patients with isolated cutaneous LCH progressed to multisystem disease during the follow-up period.
Cutaneous LCH, with or without other organ involvement at diagnosis has a good overall prognosis. However, long-term follow-up is recommended in view of possible recurrence.
描述一个孤立性皮肤朗格汉斯细胞组织细胞增生症（LCH）的亚洲人群，回顾该亚组患者的既往研究，并将我们的队列与其他伴有系统受累的皮肤 LCH 研究进行比较。
对 2001 年 1 月至 2011 年 12 月期间在一家三级儿科医院（KK 妇女儿童医院）就诊的所有诊断为皮肤受累的 LCH 患者进行回顾性分析。记录的信息包括临床表现、检查结果、治疗和结局。
我们共发现 10 例具有皮肤 LCH 表现的患者。6 例为孤立性皮肤 LCH，无内脏或骨骼受累，4 例为皮肤病变伴其他器官系统受累。在孤立性皮肤受累的患者中，3 例接受了手术切除，1 例接受了局部皮质类固醇治疗，2 例接受了多药化疗。4 例伴有系统受累的患者接受了多药化疗。在随访期间，我们的所有孤立性皮肤 LCH 患者均未进展为多系统疾病。
皮肤 LCH 无论在诊断时是否伴有其他器官受累，其总体预后良好。然而，鉴于可能会复发，建议进行长期随访。
