Departments of Surgery, Texas Tech University Health Sciences Center, El Paso, TX, USA.
Pathology at Texas Tech University Health Sciences Center, El Paso, TX, USA.
J Med Case Rep. 2021 Jul 22;15(1):357. doi: 10.1186/s13256-021-02924-0.
Langerhans cells belong to the histiocytic system and give rise to two tumors: Langerhans cell histiocytosis and Langerhans cell sarcoma. Clinical aggressiveness and degree of atypia distinguish the two neoplasms. Langerhans cell histiocytosis can infiltrate a single or multiple organ systems and particularly affects bone, skin, and lymph nodes. Perianal cutaneous Langerhans cell histiocytosis is a rare condition in adults, with 15 cases reported in the literature.
We present the case of a 50-year-old hispanic man who presented with a 9-month history of pruritus ani and a personal history of diabetes insipidus. Punch biopsy confirmed a lesion of Langerhans cells origin but could not exclude Langerhans cell sarcoma because of limited sample size. An additional biopsy was planned as well as a positron emission tomography scan to determine the extent of disease spread. While the patient failed to follow up for repeat biopsy, the positron emission tomography scan was performed and was negative for metastatic disease. A stable perianal lesion of Langerhans cell histiocytosis with benign clinical features in a 50-year-old male despite lack of treatment is extremely rare and has not been described in the literature so far. Here, we review the presentation and workup of patients with Langerhans cell histiocytosis, review the relevant literature, and discuss treatment planning.
Perianal Langerhans cell histiocytosis is rare, and there should be a high index of suspicion with chronic or new perianal lesions, especially in a patient with a history of diabetes insipidus. It is also important to consider the patient's full clinical course when it is not possible to reach a definitive pathological diagnosis before management.
朗格汉斯细胞属于组织细胞系统，可引发两种肿瘤：朗格汉斯细胞组织细胞增生症和朗格汉斯细胞肉瘤。两种肿瘤通过临床侵袭性和异型程度进行区分。朗格汉斯细胞组织细胞增生症可浸润单个或多个器官系统，尤其影响骨骼、皮肤和淋巴结。肛周皮肤朗格汉斯细胞组织细胞增生症在成人中较为罕见，文献中报道了 15 例病例。
我们报告了一例 50 岁西班牙裔男性患者，其病史为 9 个月肛门瘙痒和尿崩症。皮肤活检证实为朗格汉斯细胞起源的病变，但由于样本量有限，不能排除朗格汉斯细胞肉瘤。计划进行额外的活检和正电子发射断层扫描，以确定疾病扩散的范围。尽管该患者未能进行重复活检，但进行了正电子发射断层扫描，结果未显示转移性疾病。在没有治疗的情况下，一名 50 岁男性肛周存在稳定的朗格汉斯细胞组织细胞增生症病变，且具有良性临床特征极为罕见，目前尚未在文献中描述。在此，我们回顾了朗格汉斯细胞组织细胞增生症患者的表现和检查结果，回顾了相关文献，并讨论了治疗计划。
肛周朗格汉斯细胞组织细胞增生症罕见，对于慢性或新发肛周病变，尤其是有尿崩症病史的患者，应高度怀疑该病。在无法明确病理诊断的情况下，考虑患者的完整临床过程对于管理也很重要。
