Department of Dermatology, University of Illinois at Chicago College of Medicine, Chicago, Illinois, USA.
Department of Pathology, University of Illinois at Chicago College of Medicine, Chicago, Illinois, USA.
Clin Dermatol. 2020 Mar-Apr;38(2):223-234. doi: 10.1016/j.clindermatol.2019.10.007. Epub 2019 Oct 24.
Langerhans cell histiocytosis (LCH) is an uncommon but serious inflammatory neoplasia that affects many organs, including the skin. Though uncommon, it should remain high on a clinician's differential diagnosis in treatment-resistant cases of conditions, such as seborrheic dermatitis, diaper dermatitis, arthropod bites, and many more. A thorough history nd physical examination for each patient can aid in the diagnosis; however, if clinically suspicious for LCH, a punch biopsy should be performed. Histologic evaluation of LCH is often enough to differentiate it from the many clinical mimickers. Characteristic findings include a histiocytic infiltrate with "coffee bean"-cleaved nuclei, rounded shape, and eosinophilic cytoplasm. Immunohistochemical stains, including CD1a, S100, and CD207 (langerin) are often needed for a definitive diagnosis. Electron microscopy also demonstrates the ultrastructural presence of Birbeck granules, but this is no longer needed due to immunohistochemical staining. Treatment is often necessary for LCH, if systemic involvement exists.
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见但严重的炎症性肿瘤，可影响许多器官，包括皮肤。尽管罕见，但在治疗抵抗的情况下，如脂溢性皮炎、尿布皮炎、节肢动物叮咬等，应将其作为临床医生鉴别诊断的重点。对每位患者进行详细的病史和体格检查有助于诊断；然而，如果临床上怀疑为 LCH，则应进行打孔活检。LCH 的组织学评估通常足以将其与许多临床表现相似的疾病区分开来。特征性发现包括具有“咖啡豆”状核分裂的组织细胞浸润，圆形形状和嗜酸性细胞质。通常需要免疫组织化学染色，包括 CD1a、S100 和 CD207（朗格汉斯细胞），以明确诊断。电子显微镜也显示出 Birbeck 颗粒的超微结构存在，但由于免疫组织化学染色，现在不再需要。如果存在全身受累，通常需要治疗 LCH。
