PDF(Pubmed)
Abstract：
Langerhans cells, often referred to as the \"macrophages of the skin\", are dendritic cells that normally reside in the epidermis and papillary dermis. Just like macrophages, they function as antigenpresenting cells that activate naive T cells. Certain mutations such as those involving the BRAF gene can cause unopposed production of Langerhans cells, which is known as Langerhans cell histiocytosis (LCH). LCH triggers an inflammatory immune response that causes systemic manifestations such as fever and fatigue, as well as other manifestations depending on the affected organs. The pathogenesis behind LCH remains poorly understood. It is still unknown whether it is a neoplastic process or a reactive cancer-mimicking illness. Diagnosis of LCH is confirmed by biopsy, and treatment is largely dependent on the extent and severity of the disease. Common treatments include corticosteroids, excision, radiation, and chemotherapy. We present a case of a 1-year-old Saudi male with LCH.
摘要：
朗格汉斯细胞,通常被称为“皮肤的巨噬细胞”，是通常存在于表皮和乳头状真皮中的树突状细胞。就像巨噬细胞一样，它们作为抗原呈递细胞激活幼稚T细胞。某些突变，例如涉及BRAF基因的突变，可以导致朗格汉斯细胞的无对抗产生，这被称为朗格汉斯细胞组织细胞增生症（LCH）。LCH引发炎症免疫反应，引起全身表现，如发烧和疲劳，以及其他取决于受影响器官的表现。LCH背后的发病机制仍然知之甚少。目前尚不清楚这是一个肿瘤过程还是一个反应性癌症模仿疾病。LCH的诊断通过活检证实，治疗在很大程度上取决于疾病的程度和严重程度。常见的治疗包括皮质类固醇，切除,辐射,和化疗。我们介绍了一名1岁的沙特男性患有LCH的病例。
