Department of Medical Imaging, Jiangxi Provincial People's Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang, Jiangxi, China.
Department of Integrated Oncology, Center for Integrated Oncology (CIO), University Hospital Bonn, Germany.
Medicine (Baltimore). 2023 Sep 1;102(35):e34881. doi: 10.1097/MD.0000000000034881.
Langerhans cell histiocytosis (LCH) is a kind of rare disease in which dendritic cells proliferate abnormally. It often occurs in children and can involve any tissue and organ. The affected sites usually include bone, skin, pituitary gland, and lungs, while the thyroid gland and external auditory canal are rarely observed. The perineal and labial involvement of this disease has not been reported yet.
A 47-year-old female patient experienced a swelling of the anterior neck area without an obvious inducement. She noticed a quail egg-like mass on the left side, and the mass increased progressively within 3 months. The anterior neck area was found to be swollen, and some flaky red rashes were seen on the scalp and bilateral external auditory canals.
Imaging examination showed enlarged thyroid and cervical lymph nodes, multiple low-density nodules in the liver, and reduced signal in the posterior pituitary gland. The biopsy pathological result of the increased left cervical lymph node indicated that LCH was detected.
VP regimen (vincristine, dexamethasone per os) and related supportive treatments were given as inducing chemotherapy for 6 weeks.
After the second chemotherapy, the rash on the scalp and external auditory canal improved, and the neck mass was significantly reduced. After the third chemotherapy, the rash was mostly disappeared, while the neck lumps increased during chemotherapy. Thus, clatribine chemotherapy was recommended as the follow-up.
Imaging examinations played an important role in the diagnosis and follow-up of the disease, especially 18F-FDG PET/CT, which could show multiple involving organs at the same time. When a patient suffering from diabetes insipidus, skin rash, or fever, has a high FDG uptake PET/CT result in multiple tissues and organs throughout the body, it is necessary to consider the possibility of LCH.
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见的疾病，其中树突状细胞异常增殖。它常发生于儿童，可累及任何组织和器官。受累部位通常包括骨、皮肤、垂体和肺，而甲状腺和外耳道则很少观察到。目前尚未报道该病的会阴部和唇部受累。
一名 47 岁女性患者因颈前区肿胀就诊，无明显诱因。患者发现左侧有鹌鹑蛋样包块，且在 3 个月内逐渐增大。颈前区肿胀，头皮及双侧外耳道可见片状红色皮疹。
影像学检查显示甲状腺及颈部淋巴结肿大，肝脏内多发低密度结节，垂体后叶信号减低。左侧颈部增大淋巴结活检病理结果提示朗格汉斯细胞组织细胞增生症。
给予 VP 方案（长春新碱、口服地塞米松）诱导化疗 6 周。
第 2 次化疗后，头皮及外耳道皮疹好转，颈包块明显缩小；第 3 次化疗后皮疹基本消退，化疗期间颈包块增大，建议后续行氯法拉滨化疗。
影像学检查在疾病的诊断和随访中起重要作用，特别是 18F-FDG PET/CT，可同时显示多个受累器官。当糖尿病患者出现皮疹、发热或不明原因的多器官 FDG 摄取增高时，需考虑 LCH 的可能。
