一名8岁女孩的外阴病变：多系统朗格汉斯细胞组织细胞增多症的皮肤表现
Vulvar Lesions in an 8-Year-Old Girl: Cutaneous Manifestations of Multisystem Langerhans Cell Histiocytosis.
作者信息
Jorgensen Elisa M, Chen Peter P, Rutter Sarah, Cron Julia A
机构信息
Department of Obstetrics, Gynecology, and Reproductive Sciences, Yale University School of Medicine, New Haven, Connecticut.
Department of Pathology, Yale University School of Medicine, New Haven, Connecticut.
出版信息
J Pediatr Adolesc Gynecol. 2018 Apr;31(2):153-155. doi: 10.1016/j.jpag.2017.09.010. Epub 2017 Oct 6.
BACKGROUND
Langerhans cell histiocytosis (LCH) is a rare localized or systemic disease characterized by proliferation of myeloid-derived dendritic cells. Vulvar lesions might be the herald symptom of LCH and might mimic other cutaneous lesions. Prognosis varies widely on the basis of the extent and spread of disease.
CASE
An 8-year-old girl with a 4-month history of vulvar lesions resistant to topical steroids was referred by her pediatrician. Vulvar biopsy was diagnostic for LCH. Imaging studies revealed a left hip lesion consistent with LCH. The patient was subsequently treated for multisystem LCH with vinblastine and prednisone.
SUMMARY AND CONCLUSION
Although rare, LCH might be diagnosed by gynecologic providers and should be included in the differential diagnosis of genital lesions. We recommend having a low threshold for performing biopsy of vulvar lesions.
背景
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的局限性或全身性疾病，其特征为髓源性树突状细胞增殖。外阴病变可能是LCH的先兆症状，且可能类似其他皮肤病变。预后因疾病的范围和扩散程度而异。
病例
一名8岁女童，有4个月的外阴病变史，对局部类固醇治疗无效，由其儿科医生转诊。外阴活检诊断为LCH。影像学检查显示左髋部病变与LCH相符。该患者随后接受了长春碱和泼尼松治疗多系统LCH。
总结与结论
尽管罕见，但LCH可能由妇科医生诊断，且应列入生殖器病变的鉴别诊断中。我们建议对外阴病变进行活检的阈值要低。
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