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Abstract Purpose Posterior uveitis is a common chorioretinal pathology affecting all ages worldwide and is a frequent reason for referral to the retina clinic. The spectrum of etiologies for uveitis is very broad and includes infectious and auto-immune diseases. Inflammation can be confined to the eye or may be a part of systemic disease. A useful outline is therefore proposed to aid in the correct diagnosis of these challenging entities. The situation is further complicated by the fact that many neoplastic conditions resemble features of posterior uveitis; they are known as “masqueraders of uveitis”. Here, we summarize different posterior uveitides that present with rare findings, along with masqueraders that can be difficult to distinguish. These conditions pose a diagnostic dilemma resulting in delay in treatment because of diagnostic uncertainty. Methods An extensive literature search was performed on the MEDLINE/PUBMED, EBSCO and Cochrane CENTRAL databases from January 1985 to January 2022 for original studies and reviews of predetermined diagnoses that include posterior uveitic entities, panuveitis and masquerade syndromes. Results We described conditions that can present as mimickers of posterior uveitis (i.e., immune check-points inhibitors and Vogt-Koyanagi-Harada-like uveitis; leukemia and lymphoma associated posterior uveitis), inflammatory conditions that present as mimickers of retinal diseases (i.e., Purtscher-like retinopathy as a presentation of systemic lupus erythematosus; central serous chorioretinopathy masquerading inflammatory exudative retinal detachment), and uveitic conditions with rare and diagnostically challenging etiologies (i.e., paradoxical inflammatory effects of anti-TNF-α; post vaccination uveitis; ocular inflammation after intravitreal injection of antiangiogenic drugs). Conclusion This review of unique posterior uveitis cases highlights the overlapping features of posterior uveitis (paradoxical inflammatory effects of anti -TNF α and uveitis; Purtscher-like retinopathy as a presentation of systemic lupus erythematosus, …) and the nature of retinal conditions (ischemic ocular syndrome, or central retinal vein occlusion, amyloidosis, inherited conditions like retinitis pigmentosa, autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV), etc.…) that may mimic them is represented. Careful review of past uveitis history, current medications and recent vaccinations, detailed examination of signs of past or present inflammation, eventually genetic testing and/ or multimodal retinal imaging (like fluorescein angiography, EDI-OCT, OCT-angiography for lupus Purtscher-like retinopathy evaluation, or ICG for central serous retinopathy, or retinal amyloid angiopathy) may aid in correct diagnosis.

Purpose: To describe a case of bilateral, sequential placoid choroidopathy following uncomplicated Descemet's membrane endothelial keratoplasty (DMEK). Observations: A 49-year old woman presented with flashing lights and central visual field scotomas after undergoing uncomplicated DMEK combined with cataract surgery for Fuch's endothelial dystrophy in the right eye. She was found to have placoid choroidopathy responsive to systemic steroids and for which a comprehensive work-up was unrevealing. Three and a half months later, she underwent DMEK surgery in the fellow eye and again developed placoid choroidopathy in the operated eye. Work-up was again unrevealing and the lesion followed a similar course to the first eye on systemic steroids. Over the course of seven (right eye) and three and a half months (left eye) of follow-up, the uncorrected visual acuity was 20/20 bilaterally and the retinal lesions had modestly improved. Conclusions and Importance: We report a case of placoid choroidopathy following uncomplicated DMEK combined with cataract surgery in both eyes of a single patient. This case expands upon the reported complications following DMEK surgery and suggests a need to remain aware of posterior segment complications following endothelial keratoplasty. Keywords: DMEK, Endothelial keratoplasty, Placoid choroidopathy, Chorioretinitis