Ocular Pathology

Anatomy and pathology of the human eye. Use it to review eye pathology for Ophthalmology Board Review or OKAP.

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Tuesday, December 26, 2006

What is conjunctival amyloidosis?

Primary Amyloidosis of the Conjunctiva
Definition: Amyloidosis of the conjunctiva may be primary or secondary. Primary localized deposition in the substantia propria occurs without systemic involvement. Secondary amyloidosis of the conjunctiva may occur in association with corneal involvement, or secondary to inflammation, or as part of a systemic condition such as multiple myeloma.
Incidence/Prevalence: This is a rare condition. Only small groups of patients have been reported. Some authors report an incidence of about .002% in surgical pathology material (1).
Etiology: The material deposited in primary amyloidosis of the conjunctiva is generally immunoglobulin-related proteins, amyloid light chains. IgD, IgA lamda have been described.
Clinical Findings: Primary amyloidosis of the conjunctiva occurs in young to middle aged adults as unilateral or bilateral (~20%) solitary or waxy, firm painless nodules or fusiform swellings. Ptosis may be present (~60%) with tarsal or levator involvement (number 1).
Pain is present in about 20% of cases. There may be associated vascular dilation and hyperemia (number 2).
Histopathology: In hematoxylin and eosin stained sections, amyloid is viewed as nodular collections of amorphous homogenous eosinophilic deposits in the substantia propria (arrow 3)

and sometimes in the walls of blood vessels (arrow 4). There may be associated inflammation and even a foreign body giant cell response to the amyloid. When stained with the fluorescent Congo red dye and viewed under polarized light with a second polarizing analyzer, the amyloid deposits exhibit red-green dichroism (arrow 5), a result of several factors including retardation of birefringence, linear and circular dichroism, the Cotton effect and other influences (see more in depth discussion). Note in the photograph that some of the amyloid deposits appear green and other appear yellow, orange and even bright red. The multiple influences in some case are additive.
Treatment: Once the diagnosis of a primary localized process has been made by excluding systemic conditions that would warrant other therapy, debulking of the tumor is often performed with good success.

Prognosis: Only about 15% of the tumors progress; the others remain stable or regress.

Demrici et al: Survey Ophthalmology 2006;51:419-33.

2. Leibovitch et al. Ophthalmology 2006;113:1657-64.

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