Primary (localised non-familial) conjunctival amyloidosis: Three case reports

Abstract

Conjunctival amyloidosis is an uncommon condition which usually arises secondary to chronic infection or trauma to the eye. It is rare to find significant deposits of amyloid involving the conjunctiva alone in the absence of a known antecedent infective, traumatic or hereditofamilial disorder. We report three patients with primary localised conjunctival amyloidosis. Each patient presented with a painless swelling in the lower fornix; in two this gradually extended to involve the bulbar and palpebral conjunctiva of both eyes with friable yellow deposits. Detailed clinical photographs illustrate the specific features of the disease. None of the patients had previous ocular pathology and bacterial, viral and fungal cultures of the conjunctiva were negative. There were no signs of inflammation. Conjunctival biopsies in each case showed large amorphous nodular deposits of amyloid within the substantia propria. Extensive systemic investigations including serum and urinary protein immunoelectrophoresis were normal. Follow-up ranged from 2 to 15 years after presentation. Treatment was symptomatic. As the typical features of amyloidosis present late in the course of conjunctival disease a diagnostic biopsy is often delayed. Once there is histopathological verification plasma cell disease must be excluded, although if the amyloid is limited to the conjunctiva systemic involvement is unlikely.