Amyloidosis is a rare group of disorders characterized by the deposition of insoluble
fibrillar proteins in a ?-pleated sheet configuration, known as amyloid, within the
extracellular and perivascular space, as the consequence of a wrong folding process
of normally soluble proteins, and may affect virtually any organ or tissue of the
body 1]–5].
Amyloidosis disorders are classified into three major forms: local, systemic, and
hereditary systemic amyloidosis 2]. The acquired forms may be primary (immunologic) and secondary (reactive) 2]. In primary amyloidosis (AL) deposits contain immunoglobulin light chains, usually
lambda and kappa monoclonal types, resulting from the abnormal production by plasmocytes
in the bone marrow 2], 6]. Secondary amyloidosis (AA) is formed from serum protein A, an acute phase reactant
protein that is synthesized in response to longstanding inflammation 7]. In hereditary amyloidosis protein deposits are constituted by a mutant form of the
transport protein transthyretin 8].
Ocular amyloidosis occurs most frequently as the local deposition of amyloid in the
same place where it originates or, rarely, may be part of a systemic disorder 3], 9]. Amyloid may accumulate in the eyelid, conjunctiva, cornea, vitreous, or anterior
orbit 3], 10].
Conjunctival amyloidosis is one of the most common forms of ocular involvement, mostly
occurring as a local deposition of amyloid and rarely in association with systemic
involvement 3], 10]–15]. Due to the rarity of conjunctival amyloidosis, its diagnosis can often be overlooked
or confused with other conditions that may affect the conjunctiva. It is more commonly
observed in middle-aged adult patients, presenting as an inflammatory or malignant
conjunctival entity 3], 11]. Besides, conjunctival malignant lesions can lead to amyloidosis and they should
be ruled out 3], 12].
The purpose of our study is to report a new case of systemic amyloidosis with bilateral
conjunctival involvement.