Carneiro Inês, Friande António, Araújo Maria
Serviço de Oftalmologia. Centro Hospitalar Universitário do Porto. Porto. Portugal.
Acta Med Port. 2019 Sep 2;32(9):617-620. doi: 10.20344/amp.10179.
Langerhans cell histiocytosis remains an enigmatic disease with a very heterogeneous presentation. We describe a rare case of orbital Langerhans cell histiocytosis in a 39-year-old female patient who presented right orbital pain and edema of the upper right eyelid. Surgery showed a friable lesion and underlying bone irregularity. Morphological aspects and immunohistochemical profile favored the diagnosis of Langerhans cell histiocytosis, which was confirmed with evidence of Langerin expression. The staging tests did not reveal any organ involvement, so we decided to follow the algorithm proposed by Euro Histio Net: in case of unifocal disease and in a single organ, clinical surveillance was preferred. This case aims to raise awareness of a manifestation of Langerhans cell histiocytosis, which should always be considered as a differential diagnosis in adults with osteolytic orbital lesions.
朗格汉斯细胞组织细胞增多症仍然是一种表现非常异质性的神秘疾病。我们描述了一例罕见的眼眶朗格汉斯细胞组织细胞增多症，患者为一名39岁女性，表现为右侧眼眶疼痛和右上眼睑水肿。手术显示病变易碎且下方骨质不规则。形态学特征和免疫组化结果支持朗格汉斯细胞组织细胞增多症的诊断，朗格素表达的证据进一步证实了这一诊断。分期检查未发现任何器官受累，因此我们决定遵循欧洲组织细胞增多症网络提出的方案：对于单灶性疾病且仅累及单一器官的情况，首选临床监测。本病例旨在提高对朗格汉斯细胞组织细胞增多症一种表现形式的认识，在患有溶骨性眼眶病变的成人中，应始终将其视为鉴别诊断的考虑因素。
