A Rare Case Report of Periorbital Pseudolymphoma

Pseudolymphoma (PSL) of the orbit is a benign lymphoid hyperplasia (LH). It is a rare disease with an extensive range of known causative agents. LH is further classified into "reactive" (RLH) and "atypical" (ALH) types. It clinically presents as a single or a few plaques and/or nodular lesions, particularly on the head, neck, and upper trunk. It must be differentiated from orbital malignant lymphoma. In this report, we present a case of a 58-year-old Pakistani female with an asymptomatic recurrent right periorbital swelling for three years. It was clinically diagnosed as an angiotensin-converting enzyme (ACE) inhibitor-induced angioedema as it responded to stopping the ACE inhibitor; however, after four months, the patient again started to develop right periorbital swelling. An incisional biopsy revealed perivascular and periadnexal infiltration of lymphocytes, plasma cells, and a few neutrophils along with pigmentary incontinence. The formation of multiple lymphoid follicles and infiltration by monomorphic lymphoid cells in deeper skeletal muscle fibers were also observed. Immunohistochemistry (IHC) showed polyclonality and low Ki-67 labeling (20%), corresponding to periorbital RLH. Our objective in this study is to highlight the importance of considering PSL as a differential diagnosis in periorbital swelling. We also suggest that recurrent angioedema may lead to PSL.