摘要
目的总结并分析皮肤ALK阳性组织细胞增生症(ALK-positive histiocytosis)的临床及组织病理特点。方法回顾性分析2023年1月—2024年12月中国医学科学院皮肤病研究所确诊的3例原发于皮肤的ALK阳性组织细胞增生症患者的临床及组织病理资料,并进行文献复习。结果3例患者年龄分别为8岁、29岁和40岁,临床表现为头面部或躯干单发无痛性丘疹或结节,临床误诊为皮肤纤维瘤或黄色肉芽肿。皮损组织病理检查示真皮内梭形或上皮样组织细胞增生,伴Touton多核巨细胞及淋巴细胞浸润。免疫组化示间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)、CD68及CD163均(+);S-100蛋白(-);荧光原位杂交检测证实存在ALK基因断裂。3例患者均行手术切除,随访9~23个月无复发。结论皮肤ALK阳性组织细胞增生症是一种罕见的组织细胞源性肿瘤,预后通常良好,需与幼年黄色肉芽肿、朗格汉斯细胞组织细胞增生症等疾病鉴别。
Objective To analyze the clinical and pathological characteristics of cutaneous ALKpositive histiocytosis.Methods We retrospectively studied the clinicopathological data of three patients diagnosed with primary cutaneous anaplastic lymphoma kinase(ALK)-positive histiocytosis at Hospital for Skin Diseases(Institute of Dermatology),Chinese Academy of Medical Sciences and Peking Union Medical College from January 2023 to December 2024.A review of related literature was conducted.Results The patients,aged 8 to 40 years,presented with solitary,painless papules or nodules on the head and face region or on the trunk,which were clinically misdiagnosed as dermatofibroma or xanthogranulomas.Histopathological examination revealed a dermal infiltrate of spindle-to-epithelioid histiocytes accompanied by Touton-type multinucleated giant cells and lymphocytes.Immunohistochemistry demonstrated the dermal infiltrate was positive for ALK(diffuse cytoplasmic positivity),CD68,CD163,and was negative for S-100.Fluorescence in situ hybridization(FISH)confirmed ALK gene rearrangements.All three patients underwent surgical excision,with no recurrence during a follow-up period of 9-23 months.Conclusion Cutaneous ALK-positive histiocytosisis is a rare histiocytic-derived neoplasm with a favorable prognosis and requires molecular confirmation to differentiate it from juvenile xanthogranuloma,Langerhans cell histiocytosis,and other histiocytic proliferations.
作者
丁倩
惠云
宋昊
陈浩
DING Qian;HUI Yun;SONG Hao;CHEN Hao(Department of Dermatology,The Second Affiliated Hospital of Chongqing Medical University,Yuzhong District,Chongqing 400o10,China;Department of Dermatology,Jinling Hospital,Affiliated Hospital of Medical School,Nanjing University,Nanjing 210042,China;Department of Pathology,Hospital for Skin Diseases(Institute of Dermatology),Chinese Academy of Medical Sciences and Peking Union Medical College,Nanjing 210042,China)
出处
《临床皮肤科杂志》
2026年第2期88-91,共4页
Journal of Clinical Dermatology
