Systemic amyloidosis with bilateral conjunctival involvement: a case report

Abstract

BackgroundConjunctival amyloidosis is a very rare condition, generally unilateral, and presents mostly as an isolated condition without systemic compromise. Our purpose is to present a new case of systemic amyloidosis with a bilateral conjunctival involvement.Case PresentationA 66-years-old caucasian female complaining of conjunctival hemorrhage and chemosis in both eyes for the last five years had been discontinuously treated with topical antibiotics and corticosteroids without any evident improvement. She presented with a pink-yellow infiltration in the inferior conjunctiva of both eyes. Conjunctival biopsy under optical microscopy revealed amyloid deposit, confirmed by Congo red staining. Mucosal biopsy from esophagus and rectus confirmed amyloidosis by Congo red stain. Immunohistochemistry of bone marrow biopsy showed an increased number of plasma cells and an over-expression of light chain kappa subunit. She was treated with corticosteroids and lubrication with an improvement of symptoms. Ocular lesions remained stable after a follow-up of 3 years.ConclusionsConjunctival amyloidosis is a rare entity that may be overlooked, and should be differentiated from chronic conjunctivitis and conjunctival malignancies. Although it presents most frequently as a local process, a systemic involvement should always be ruled out.

A Rare Case of Amyloidosis of the Eyelid and Conjunctiva

“…It can be both systemic and localised [ 1 , 7 ]. Amyloidosis of the conjunctiva and eyelid is a rare entity that is typically benign [ 5 , 6 , 9 ]. Preceding causes for amyloidosis can include trauma, infection, and inflammation [ 5 ].…”

“…Systemic examination and investigations are required to rule out both systemic amyloid and also neoplastic plasma cell disease [ 4 , 9 ].…”

Orofacial amyloidosis—unusual ­presentation of a rare condition: A case report

“…This accumulation of excessive amyloid may affect virtually any organ or tissue of the body, which can be clinically deleterious impairing both structure and function. Amyloid is an insoluble fibrillar protein, which results due to an abnormal folding process of normally soluble proteins in a β‐pleated sheet configuration . As a result, the body finds it difficult to remove these nonrecyclable and nonbiodegradable proteins, which get deposited as insoluble fibrils in different tissues and organs .…”

“…This deposition of amyloid can be either localized, where it is restricted in one small area of the body or systemic, affecting many organs of the body. This disease may be acquired or hereditary; the acquired forms may be primary (immunologic) and secondary (reactive) . Amyloidosis affecting the head and neck may involve any site; however, the most common sites are the larynx and the tongue.…”

Long-Term Follow-Up of 4 Patients with Conjunctival Amyloidosis

“…There have been few reports of localized AL amyloidosis of the conjunctiva associated with no or trace systemic involvement [15, 17]. However, conjunctival amyloidosis may be the initial sign of systemic disease [17-20], and systemic workup is recommended. This report describes the long-term follow-up of 4 cases with histologically proven conjunctival amyloidosis treated at a single institution.…”