Primary amyloidosis of the conjunctiva and eyelid is a rare and often misdiagnosed condition. It is characterized by the deposition of insoluble amyloid fibrils, which are misfolded proteins, in the body. Amyloidosis can be systemic or localized with different types of amyloid fibril proteins identified using mass spectrometry. Ocular involvement in amyloidosis can lead to corneal dystrophies, glaucoma, vitreous opacities, and other symptoms. Diagnosis involves clinical examination and histopathological assessment. Treatment options depend on the extent of involvement and may include surgical excision, glaucoma management, vitrectomy, or liver transplantation in rare cases. We present a rare case of localized conjunctival amyloidosis initially misdiagnosed as pyogenic granuloma, with clinical symptoms of ptosis, periorbital swelling, and conjunctival lesions. The patient underwent excision of the lesions, and subsequent evaluation did not reveal systemic amyloidosis. Ocular amyloidosis requires careful diagnosis and consideration of systemic involvement for appropriate management.

UNASSIGNED: Conjunctival lymphoma, conjunctival amyloidosis and benign reactive lymphoid hyperplasia (BRLH) are conditions that often have a similar appearance on the ocular surface. The use of high resolution anterior segment optical coherence tomography (HR-OCT) enables clinicians to evaluate distinctive differences in tissue morphology and cellular patterns in various ocular surface conditions. In this study, we characterize the morphological differences seen in conjunctival lymphoma, conjunctival amyloidosis and BRLH on HR-OCT imaging.
UNASSIGNED: A retrospective chart review was performed of patients with biopsy proven conjunctival lymphoma, conjunctival amyloidosis and BRLH between 2012 and 2019 at the Bascom Palmer Eye Institute. Patients were excluded if HR-OCT imaging was not performed on initial presentation.
UNASSIGNED: Thirty-four total eyes of 27 patients were identified. Twenty eyes had conjunctival lymphoma (16 patients), 8 eyes had conjunctival amyloidosis (6 patients) and 6 eyes had BRLH (5 patients). All conditions appeared clinically as pink, red or yellow subepithelial lesions but had different features on HR-OCT. In lymphoma, HR-OCT images typically showed homogenous, dark subepithelial lesions with smooth borders, containing monomorphic dot-like infiltrates. HR-OCT images of amyloidosis typically showed heterogeneous, dark lesions with irregular borders, often containing hyperreflective linear infiltrates. HR-OCT images of BRLH showed variable infiltration of the subepithelial tissue, at times with homogenous lesions containing dot-like infiltrates like lymphoma and other times with more hyperreflective, subepithelial tissue. Flow cytometry and gene rearrangement was needed for final differentiation between BRLH and lymphoma lesions.
UNASSIGNED: Distinctive features on HR-OCT of conjunctival lymphoma, conjunctival amyloidosis and BRLH can help characterize these lesions beyond what is apparent with the clinical examination. Future studies can further validate this technology\'s use with more subtle and challenging lesions.

Conjunctival amyloidosis is a rare cause of ocular inflammation, mass, and hemorrhage that can be difficult to diagnose and treat. In this case series, we describe 4 patients with a histopathological diagnosis of conjunctival amyloidosis treated at a single institution. All patients underwent surgical excision and biopsy. On histopathological examination, 3 patients had local deposition of either kappa or lambda monoclonal immunoglobulin light chains, favoring localized amyloid light-chain amyloidosis. Systemic workup to exclude rheumatologic disorders (e.g., anti-neutrophil cytoplasmic antibody and rheumatoid factors) and hematological disorders (e.g., imaging, biopsies, and serum protein electrophoresis/urine protein electrophoresis) was negative except for a positive abdominal fat biopsy in 1 patient. Patients were followed for an average of 6.1 years (range 4 months to 15 years) with stable ocular disease.

Amyloidosis is a disorder characterized by the deposition of insoluble abnormal proteins in the extracellular space. It may occur as a localized lesion or as a systemic disease involving multiple organs and systems. Localized conjunctival amyloidosis is rare and is less frequently associated with systemic involvement. Although amyloidosis itself is a benign lesion involvement of multiple organs and systems is associated with poor prognosis. Diagnosis of amyloidosis is made on biopsy specimens with Congo red staining for the appearance of apple-green birefringence under polarized light microscopy. Liquid chromatography tandem-mass spectrometry (LC-MS/MS) is much more sensitive in diagnosing amyloidosis and can determine the type of amyloid deposit. Here we reported a case of conjunctival amyloidosis in a 52 year-old male patient who was presented with left lower eyelid swelling to our medical center. He has a complicated past medical history of anti-phospholipid antibody syndrome, Buerger\'s disease (thromboangitis obliterans), and small cell lymphoma (SLL) of the right orbit/eyelid. The patient received radiation to the right orbit to treat SLL with therapy completed one and a half years prior to presentation. Physical examination revealed a firm, raised yellowish colored lesion in the left lower conjunctiva. The conjunctival lesion was biopsied, and tissue sections were examined with Congo red stains and LC-MS/MS analysis. The biopsy showed amyloid deposits without evidence of malignancy, and the type of proteins in the deposit was immunoglobulin light chain (AL) of kappa type. A complete work up was taken for possible systemic involvement of amyloidosis and results were all negative. To our knowledge, this is the first case of localized conjunctival amyloidosis with a history of contralateral orbit/eyelid SLL.