Abstract：
OBJECTIVE: Ophthalmic histiocytic lesions comprise a heterogeneous rare group of disorders that are characterized by an abnormal proliferation of histiocytes and may affect all age groups of both sexes. The aim of this study was to highlight the basic demographic, clinical, and histopathological characteristics of this rare group of diseases in ophthalmic practice, which has not been previously studied in this area. Only individual cases have been previously reported.
METHODS: This was a retrospective study of all biopsied ocular and periocular histiocytic lesions from two centers, King Khaled Eye Specialist Hospital (KKESH) and King Abdulaziz University Hospital (KAUH) in Riyadh, Saudi Arabia, from January 1993 to December 2018. The histopathological diagnosis was confirmed, and the cases were re-classified by reviewing all histopathological slides. The corresponding demographic and clinical data were analyzed. A relevant literature review was also carried out for comparison of our collected analyzed data to published data and to draw our own conclusions.
RESULTS: A total of 34 ocular/periocular histiocytic lesions in 28 patients who were mostly Saudis (92.9%) were included. The male-to-female ratio was 4:3. The median age at presentation was 6.4 years (range: 2.8-35 years). Twenty-two patients had unilateral involvement, and six patients had bilateral lesions. In patients with Langerhans cell histiocytosis (LCH; L group), the most common presenting findings were eyelid swelling (75%), periocular tenderness (37.5%), proptosis/globe displacement (37.5%) eyelid erythema (25%), and orbital pain (12.5%). In patients with Rosai Dorfman disease (RDD; R group), proptosis/globe displacement occurred in all patients and 80% had decreased vision. Patients in the C group (Cutaneous non-LCH histiocytoses) had variable clinical features because of the different locations of the histiocytic lesions, with the majority involving the eyelids (66.7%). Diagnosis was accurately reached clinically in 38.8%, 33.7%, and 46.7% of patients in the L, C, and R groups, respectively. Overall, the clinical diagnosis was in concordance with the histopathologic diagnosis in 14 out of 34 lesions (41.2%).
CONCLUSIONS: Histiocytic disease is more likely to be overlooked clinically owing to its rarity. In the C group, juvenile xanthogranuloma (JXG) was the most commonly encountered histiocytic lesion and had a tendency to present at a later age with extremely rare intraocular involvement in contrast to previously published reports. The median age at presentation was higher in group R. All patients in group L had strictly unilateral disease, while RDD (group R) was most commonly bilateral. Future research on genetic aspects, management, and prognosis is necessary.
METHODS: This was a retrospective study of all biopsied ocular and periocular histiocytic lesions from two centers, King Khaled Eye Specialist Hospital (KKESH) and King Abdulaziz University Hospital (KAUH) in Riyadh, Saudi Arabia, from January 1993 to December 2018. The histopathological diagnosis was confirmed, and the cases were re-classified by reviewing all histopathological slides. The corresponding demographic and clinical data were analyzed. A relevant literature review was also carried out for comparison of our collected analyzed data to published data and to draw our own conclusions.
RESULTS: A total of 34 ocular/periocular histiocytic lesions in 28 patients who were mostly Saudis (92.9%) were included. The male-to-female ratio was 4:3. The median age at presentation was 6.4 years (range: 2.8-35 years). Twenty-two patients had unilateral involvement, and six patients had bilateral lesions. In patients with Langerhans cell histiocytosis (LCH; L group), the most common presenting findings were eyelid swelling (75%), periocular tenderness (37.5%), proptosis/globe displacement (37.5%) eyelid erythema (25%), and orbital pain (12.5%). In patients with Rosai Dorfman disease (RDD; R group), proptosis/globe displacement occurred in all patients and 80% had decreased vision. Patients in the C group (Cutaneous non-LCH histiocytoses) had variable clinical features because of the different locations of the histiocytic lesions, with the majority involving the eyelids (66.7%). Diagnosis was accurately reached clinically in 38.8%, 33.7%, and 46.7% of patients in the L, C, and R groups, respectively. Overall, the clinical diagnosis was in concordance with the histopathologic diagnosis in 14 out of 34 lesions (41.2%).
CONCLUSIONS: Histiocytic disease is more likely to be overlooked clinically owing to its rarity. In the C group, juvenile xanthogranuloma (JXG) was the most commonly encountered histiocytic lesion and had a tendency to present at a later age with extremely rare intraocular involvement in contrast to previously published reports. The median age at presentation was higher in group R. All patients in group L had strictly unilateral disease, while RDD (group R) was most commonly bilateral. Future research on genetic aspects, management, and prognosis is necessary.
摘要：
目的：眼科组织细胞病变包括一组异质性罕见的疾病，其特征是组织细胞异常增殖，并可能影响男女所有年龄组。这项研究的目的是强调基本的人口统计学，临床,以及眼科实践中这种罕见疾病的组织病理学特征，这是以前在这方面没有研究过的。以前只报告过个别病例。
方法：这是一项回顾性研究，对来自两个中心的所有活检眼和眼周组织细胞病变进行了回顾性研究，利雅得的哈立德国王眼科专科医院（KKESH）和阿卜杜勒阿齐兹国王大学医院（KAUH），沙特阿拉伯,从1993年1月到2018年12月。组织病理学诊断得到证实，通过回顾所有组织病理学切片对病例进行重新分类.分析相应的人口统计学和临床数据。还进行了相关的文献综述，以将我们收集的分析数据与已发表的数据进行比较，并得出我们自己的结论。
结果：共包括28例主要为沙特（92.9％）的患者中的34个眼部/眼周组织细胞病变。男女比例为4:3。演示时的中位年龄为6.4岁（范围：2.8-35岁）。22例患者有单侧受累,6例患者有双侧病变。在朗格汉斯细胞组织细胞增生症患者（LCH；L组）中，最常见的表现是眼睑肿胀（75％），眼周压痛（37.5%），眼球突出/眼球移位（37.5％）眼睑红斑（25％），和眼眶疼痛（12.5％）。在RosaiDorfman病患者（RDD；R组）中，所有患者均出现眼球突出/眼球移位,80%的患者视力下降.C组患者（皮肤非LCH组织细胞病）由于组织细胞病变的不同位置而具有不同的临床特征，大多数涉及眼睑（66.7％）。临床诊断准确达38.8%,33.7%,和46.7%的病人在L，C,和R组，分别。总的来说,在34个病灶中,有14个病灶的临床诊断与组织病理学诊断一致(41.2%).
结论：组织细胞疾病由于其罕见而在临床上更容易被忽视。在C组中,幼年黄色肉芽肿(JXG)是最常见的组织细胞性病变,与以前发表的报道相比,其倾向于在更晚的年龄出现,并伴有极其罕见的眼内受累.R组的中位年龄更高。L组的所有患者都患有严格的单侧疾病，而RDD（R组）最常见的是双侧。遗传方面的未来研究，管理,预后是必要的。
方法：这是一项回顾性研究，对来自两个中心的所有活检眼和眼周组织细胞病变进行了回顾性研究，利雅得的哈立德国王眼科专科医院（KKESH）和阿卜杜勒阿齐兹国王大学医院（KAUH），沙特阿拉伯,从1993年1月到2018年12月。组织病理学诊断得到证实，通过回顾所有组织病理学切片对病例进行重新分类.分析相应的人口统计学和临床数据。还进行了相关的文献综述，以将我们收集的分析数据与已发表的数据进行比较，并得出我们自己的结论。
结果：共包括28例主要为沙特（92.9％）的患者中的34个眼部/眼周组织细胞病变。男女比例为4:3。演示时的中位年龄为6.4岁（范围：2.8-35岁）。22例患者有单侧受累,6例患者有双侧病变。在朗格汉斯细胞组织细胞增生症患者（LCH；L组）中，最常见的表现是眼睑肿胀（75％），眼周压痛（37.5%），眼球突出/眼球移位（37.5％）眼睑红斑（25％），和眼眶疼痛（12.5％）。在RosaiDorfman病患者（RDD；R组）中，所有患者均出现眼球突出/眼球移位,80%的患者视力下降.C组患者（皮肤非LCH组织细胞病）由于组织细胞病变的不同位置而具有不同的临床特征，大多数涉及眼睑（66.7％）。临床诊断准确达38.8%,33.7%,和46.7%的病人在L，C,和R组，分别。总的来说,在34个病灶中,有14个病灶的临床诊断与组织病理学诊断一致(41.2%).
结论：组织细胞疾病由于其罕见而在临床上更容易被忽视。在C组中,幼年黄色肉芽肿(JXG)是最常见的组织细胞性病变,与以前发表的报道相比,其倾向于在更晚的年龄出现,并伴有极其罕见的眼内受累.R组的中位年龄更高。L组的所有患者都患有严格的单侧疾病，而RDD（R组）最常见的是双侧。遗传方面的未来研究，管理,预后是必要的。
