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A Growing Conjunctival Lesion in an Otherwise Healthy Patient

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 73-year-old man was referred to our service for assessment of a slow-growing conjunctival lesion in the left eye. He had first noticed left eye discomfort and a fullness of the left lower eyelid 3 months previously. He denied any vision or systemic symptoms. His ophthalmic history was noncontributory. The patient’s medical history was notable only for prostate carcinoma, which was treated with radiotherapy 12 years ago. On examination, his visual acuities were 20/25 OU. Slitlamp examination of the anterior segment revealed diffuse yellow-pink polypoidal papules affecting the inferior fornix and inferior palpebral conjunctiva of the left eye (Figure 1A). The upper palpebral conjunctiva in the left eye had a velvety yellow appearance (Figure 1B). The remainder of the examination of both eyes was unremarkable. Initial investigations, including a complete blood cell count, urinalysis, and measurement of inflammatory markers, kidney function, and prostate-specific antigen level, were within normal limits. Serum electrophoresis showed raised monoclonal IgM bands (7.25 g/L; reference range, 0.50-2.00 g/L) with a κ/λ light-chain ratio of 2.92 (reference range, 0.260-1.65).

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Conjunctival amyloidosis

D. Perform an incisional biopsy of the lesion

The appearance of this conjunctival lesion is suggestive of amyloidosis. Amyloid is an insoluble, fibrillar protein that can form tissue deposits, leading to functional impairment. The kidneys and heart are the most commonly affected organs in systemic amyloidosis. Primary localized conjunctival amyloidosis is a rare but well-described entity, most frequently affecting the palpebral conjunctiva and superior fornix.1 The diagnosis of amyloidosis is based on its characteristic histologic features that help differentiate it from other conditions, such as malignant neoplasms (lymphoma, squamous, or sebaceous cell carcinomas) and inflammatory disorders (pyogenic granuloma, sarcoidosis). Clinically, it is most easily distinguished from a more defined, pedunculated, and highly vascular pyogenic granuloma. Amyloid characteristically stains with Congo red and exhibits apple-green birefringence on polarization microscopy examination (Figure 2). Therefore, an incisional biopsy should be performed (choice D) for diagnostic purposes. This can also provide symptomatic relief by debulking the conjunctival mass with an estimated recurrence rate of less than 30%.1 A complete excisional biopsy (choice B) may be performed for solitary or more confined lesions, but it is less suitable for diffuse involvement of the conjunctiva as in this case.1,2 Unlike inflammatory conditions, localized conjunctival amyloidosis does not respond to corticosteroid therapy (choice A). Lubricants can provide temporary symptomatic relief; however, surgical debulking remains the mainstay of therapy. If orbital amyloidosis causes substantial ptosis, proptosis, or eye movement restrictions, further surgical intervention and targeted external beam radiotherapy may be considered.3,4

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Article Information

Corresponding Author: Sarah Schimansky, MB, BCh BAO, Department of Ophthalmology, The Royal United Hospitals Bath NHS Foundation Trust, Combe Park, Bath BA1 3NG, United Kingdom (sarah.schimansky@nhs.net).

Published Online: April 8, 2021. doi:10.1001/jamaophthalmol.2020.4628

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Demirci  H , Shields  CL , Eagle  RC  Jr , Shields  JA .  Conjunctival amyloidosis: report of six cases and review of the literature.   Surv Ophthalmol. 2006;51(4):419-433. doi:10.1016/j.survophthal.2006.04.007 PubMedGoogle ScholarCrossref
2.
Leibovitch  I , Selva  D , Goldberg  RA ,  et al.  Periocular and orbital amyloidosis: clinical characteristics, management, and outcome.   Ophthalmology. 2006;113(9):1657-1664. doi:10.1016/j.ophtha.2006.03.052 PubMedGoogle ScholarCrossref
3.
Copperman  TS , Truong  MT , Berk  JL , Sobel  RK .  External beam radiation for localized periocular amyloidosis: a case series.   Orbit. 2019;38(3):210-216. doi:10.1080/01676830.2018.1483407 PubMedGoogle ScholarCrossref
4.
Kang  S , Dehabadi  MH , Rose  GE , Verity  DH , Amin  S , Das-Bhaumik  R .  Ocular amyloid: adnexal and systemic involvement.   Orbit. 2020;39(1):13-17. doi:10.1080/01676830.2019.1594988 PubMedGoogle ScholarCrossref
5.
Rajkumar  SV , Gertz  MA , Kyle  RA .  Primary systemic amyloidosis with delayed progression to multiple myeloma.   Cancer. 1998;82(8):1501-1505. doi:10.1002/(SICI)1097-0142(19980415)82:8<1501::AID-CNCR11>3.0.CO;2-8 PubMedGoogle ScholarCrossref
6.
Madan  S , Dispenzieri  A , Lacy  MQ ,  et al.  Clinical features and treatment response of light chain (AL) amyloidosis diagnosed in patients with previous diagnosis of multiple myeloma.   Mayo Clin Proc. 2010;85(3):232-238. doi:10.4065/mcp.2009.0547 PubMedGoogle ScholarCrossref
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