Fronek Lisa F, Grubbs Hailey, Dorton David W, Miller Richard
Dermatology, Hospital Corporation of America / University of South Florida Morsani College of Medicine: Largo Medical Center Program, Largo, USA.
Dermatology, Broward Health Medical Center, Fort Lauderdale, USA.
Cureus. 2020 Aug 19;12(8):e9861. doi: 10.7759/cureus.9861.
Langerhans cell histiocytosis (LCH) is an infrequent clonal proliferative disorder of myeloid dendritic cells. It has a wide variety of cutaneous manifestations and retains the possibility of systemic implications. Because LCH is predominantly a disease of childhood, there are well-established clinical definitions, as well as guidelines regarding workup and treatment, in the context of pediatric disease. Here we present a case of isolated cutaneous LCH in an adult male, followed by a discussion of our diagnostic plan and treatment course. The patient exhibited a small, excoriated, yellow papule on his inferior forehead during a skin examination. The specimen underwent tangential shave biopsy; histopathologic evaluation with appropriate immunohistochemical staining confirmed a diagnosis of cutaneous LCH. After thorough investigation via serologic and imaging diagnostics, we confirmed isolated cutaneous disease. The patient underwent wide local excision (WLE) with no evidence of recurrence. It is crucial to appropriately screen all patients diagnosed with cutaneous LCH for internal organ involvement. The authors aim to highlight the need for further investigations to ultimately dictate standardized management and treatment for isolated cutaneous LCH in the adult population.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的髓样树突状细胞克隆增殖性疾病。它有多种皮肤表现，并存在全身受累的可能性。由于LCH主要是一种儿童疾病，在儿科疾病的背景下，有完善的临床定义以及关于检查和治疗的指南。在此，我们报告一例成年男性孤立性皮肤LCH病例，随后讨论我们的诊断计划和治疗过程。该患者在皮肤检查时，在前额下部出现一个小的、有擦伤的黄色丘疹。标本进行了切向剃除活检；经适当免疫组化染色的组织病理学评估确诊为皮肤LCH。通过血清学和影像学诊断进行全面检查后，我们确认是孤立性皮肤疾病。患者接受了广泛局部切除（WLE），无复发迹象。对所有诊断为皮肤LCH的患者进行适当筛查以确定是否存在内脏器官受累至关重要。作者旨在强调进一步研究的必要性，以最终确定成人孤立性皮肤LCH的标准化管理和治疗方法。
