Primary Localized Conjunctival Amyloidosis Presenting Corneal Whorl-like Opacity Patterns

Purpose To report an unusual case of corneal whorl-like opacity patterns in a patient of primary localized conjunctival amyloidosis. Methods Case report and review of literature. Results A 35-year old man with a case of primary localized conjunctival amyloidosis presented with corneal whorl-like opacity patterns and underwent conjunctival and limbal biopsies. Histopathological evaluation showed dense deposition of amorphous, hyaline, eosinophilic material with week periodic acid-Schiff positivity and congophilia on congo red stain. There was birefringence under a polarized microscope. These findings were suggestive of amyloid deposition. tumor-associated calcium signal transducer-2 mutation was negative ruling out gelatinous drop-like corneal dystrophy. Systemic evaluation was negative for primary systemic amyloidosis. A diagnosis of primary localized conjunctival amyloidosis with corneal whorl-like opacity patterns was confirmed. Conclusions This is an unusual case report of primary localized conjunctival amyloidosis, presenting as corneal whorl-like opacity patterns. Primary localized conjunctival amyloidosis usually involves the fornix and then spreads to lids causing lid thickening, blepharoptosis, subconjunctival, or orbital mass. In our case, it probably started around limbus, involving palisades of Vogt and caused corneal whorl-like opacity patterns which were the presenting feature.