Haydarpasa Numune Education and Research Hospital, Sadik Eratik Eye Institute, Division of Ophthalmic Plastic and Reconstructive Surgery, University of Health Sciences, Istanbul, Turkey.
Cerrahpasa Faculty of Medicine, Department of Ophthalmology, Division of Ocular Oncology, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Ocul Immunol Inflamm. 2023 May;31(4):717-720. doi: 10.1080/09273948.2022.2054430. Epub 2022 Apr 11.
Even though subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and lupus erythematosus panniculitis (LEP) are two separate entities, recently they were claimed to represent two ends of a spectrum of T-cell-mediated orbital lymphoproliferative diseases.
A 78-year-old woman presented with a 1-month history of right-sided periorbital swelling and redness. There was a palpable mass in the medial right lower eyelid with restriction of upward and lateral gaze. MRI revealed a 14 × 7 mm hypointense lesion in the anteromedial orbit.
The systemic and ocular findings, orbital biopsy with histopathology and immunochemistry showed overlapping features of LEP and SPTCL. The patient was consulted with rheumatology and hematology, and the physicians arrived at a consensus that the patient existed in the above-mentioned disease spectrum. She was started on systemic immunosuppressive treatment and her clinical findings improved substantially.
This is the first report of a patient, who presented with orbital mass causing vision loss and gaze restriction that had overlapping clinical and histopathologic features of LEP and SPTCL consistent with this novel disease spectrum, in the literature.
尽管皮下脂膜炎样 T 细胞淋巴瘤（SPTCL）和红斑狼疮性脂膜炎（LEP）是两种独立的实体，但最近有人声称它们代表了 T 细胞介导的眼眶淋巴增生性疾病谱的两个极端。
一名 78 岁女性因右侧眶周肿胀和变红而出现 1 个月的病史。在右侧下眼睑内侧可触及肿块，伴有上和外侧凝视受限。MRI 显示前内侧眶内 14×7mm 的低信号病变。
全身和眼部表现、眼眶活检的组织病理学和免疫化学显示 LEP 和 SPTCL 的重叠特征。患者咨询了风湿病学和血液学，医生们达成共识，认为患者存在上述疾病谱。她开始接受全身免疫抑制治疗，临床症状显著改善。
这是首例报道的患者，该患者因眶内肿块导致视力丧失和凝视受限而就诊，其临床表现和组织病理学特征与 LEP 和 SPTCL 重叠，符合这一新型疾病谱。
