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 Table of Contents  
Year : 2020  |  Volume : 58  |  Issue : 4  |  Page : 296-297

Primary conjunctival amyloidosis causing ptosis, ectropion, and chalazion-like eyelid lumps

Department of Ophthalmic Plastics, Orbital Disease and Ocular Oncology, Narayana Nethralaya, Bengaluru, Karnataka, India

Date of Submission21-Jul-2020
Date of Acceptance18-Sep-2020
Date of Web Publication16-Dec-2020

Correspondence Address:
Dr. Rwituja Thomas
Department of Ophthalmic Plastics, Orbital Disease and Ocular Oncology Service, Narayana Nethralaya, 121/C Chord Road, Rajajinagar 1st R Block, Bengaluru - 560 010, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_95_20

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Primary conjunctival amyloidosis is a rare entity. We present a patient with bilateral conjunctival amyloid, presenting with ptosis, ectropion, and chalazion-like lumps. Histopathology showed an amorphous eosinophilic lesion, and staining with Congo red showed apple-green birefringence.

Keywords: Amyloid, chalazion, conjunctival, ectropion, eyelid, ptosis

How to cite this article:
Thomas R, Hari P, Kiran A, Gupta R. Primary conjunctival amyloidosis causing ptosis, ectropion, and chalazion-like eyelid lumps. TNOA J Ophthalmic Sci Res 2020;58:296-7

How to cite this URL:
Thomas R, Hari P, Kiran A, Gupta R. Primary conjunctival amyloidosis causing ptosis, ectropion, and chalazion-like eyelid lumps. TNOA J Ophthalmic Sci Res [serial online] 2020 [cited 2023 Jan 28];58:296-7. Available from: https://www.tnoajosr.com/text.asp?2020/58/4/296/303675

  Introduction Top

Primary amyloidosis of the conjunctiva is a rare entity, presumed to occur due to an immunological alteration confined to the conjunctiva.[1] Amyloidosis may also occur in ocular tissue as a secondary or reactive form following systemic involvement.[2] It has been reported to manifest in varied forms such as conjunctival injection, epiphora, subconjunctival hemorrhage, lid edema, and as a calcified plaque.[3],[4],[5] Although commonly unilateral, bilateral involvement has been noted in chronic and progressive cases.[4] Other ocular tissues known to be affected as a part of amyloidosis are the eyelid, cornea, recti muscles, lacrimal gland, and vitreous.[2],[6] We report the clinicopathological findings of chronic bilateral conjunctival amyloidosis in an elderly patient, presenting as multiple chalazia and resulting in ectropion and ptosis.

  Case Report Top

A 66-year-old female presented with complaints of multiple nodular swellings of upper and lower lids of both eyes. The swellings had been present for 10 years, for which the patient had undergone multiple courses of topical and systemic medications, including oral doxycycline, without any improvement. She had also undergone incision and curettage for one of the lesions. There was no history of any infections in the past or any family history of a similar condition.

Best-corrected visual acuity was 20/20 in both eyes. Examination revealed thickening of upper and lower lids bilaterally along with distorted margins and several nodular swellings. The upper lids were ptotic, and the lower lids showed ectropion due to the weight of the lesions [Figure 1]a.
Figure 1: (a) Clinical photograph of patient shows bilateral ptosis, chalazion-like lumps temporally in both upper lids and bilateral lower lid ectropion (black arrows). (b) On eversion of the lids: irregular, yellow-pink mass seen on conjunctiva. (c) Congo red stain (×10) of lesion shows amorphous internal appearance. (d) Same lesion shows apple.green birefringence (white arrow) under polarized light

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The nodules were firm in consistency, nontender, and some appeared superficially on the surface of the tarsal conjunctiva [Figure 1]b. With the provisional differential diagnoses of conjunctival amyloidosis and conjunctival lymphoproliferative lesion, biopsy and histopathologic examination was performed.

In the peroperative eye, the lesions were seen to be pink-yellowish in color and appeared superficial. Both upper and lower conjunctival lesions were removed surgically, partly for the purpose of debulking.

Histopathology showed pale, eosinophilic acellular deposits [Figure 1]c. The deposits displayed a classical apple-green birefringence under polarized light with Congo red stain, confirming the diagnosis of conjunctival amyloid deposition [Figure 1]d.

On systemic evaluation, the blood counts and renal function tests were normal, and the patient had no contributory systemic factors for ocular amyloidosis. She was offered the option of further debulking of the eyelids, with ptosis and ectropion correction, but did not follow-up further.

  Discussion Top

Amyloid is an eosinophilic, amorphous protein that can get deposited in several human organs and tissues. There have been a few case series and sporadic case reports of primary conjunctival amyloidosis involving the bulbar, palpebral, and semilunar folds of conjunctiva.[1],[4],[6] We report a case of bilateral conjunctival amyloidosis involving the eyelids that presented as multiple chalazia. The lesion may recur within 3 months, or remain quiescent for 10 years.[7],[8] Cryotherapy with liquid nitrogen has been a popular treatment modality.[9] We restricted our treatment to surgical debulking.

Primary conjunctival amyloidosis is an entity that has possibly been under-diagnosed due to the histopathology being reported as inflammation at times or due to a lack of complete knowledge of the condition.[8] Hence, recurrent eyelid or conjunctival masses presenting in the manners discussed above should be evaluated to rule out amyloidosis.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Demirci H, Shields CL, Eagle RC Jr., Shields JA. Conjunctival amyloidosis: Report of six cases and review of the literature. Surv Ophthalmol 2006;51:419-33.  Back to cited text no. 1
Correa LJ, Maccio JP, Esposito E, Monti R, Gonzalez-Castellanos ME, Paradelo M, et al. Systemic amyloidosis with bilateral conjunctival involvement: A case report. BMC Ophthalmol 2015;15:77.  Back to cited text no. 2
Moorman CM, McDonald B. Primary (localised non-familial) conjunctival amyloidosis: three case reports. Eye (Lond) 1997;11 (Pt 5):603-6.  Back to cited text no. 3
Pirouzmand F, Hurwitz J, Howarth D. Primary localized bilateral conjunctival amyloidosis and epiphora. Orbit 2002;21:139-44.  Back to cited text no. 4
Kaliki S, Morawala A, Gowrishankar S. Primary eyelid amyloidosis presenting as a calcified plaque: A rare presentation. Ophthalmic Plast Reconstr Surg 2018;34:e212.  Back to cited text no. 5
Ray M, Tan AW, Thamboo TP. Atypical presentation of primary conjunctival amyloidosis. Can J Ophthalmol 2012;47:e2-4.  Back to cited text no. 6
Mesa-Gutiérrez JC, Huguet TM, Garcia NB, Ginebreda JA. Primary localized conjunctival amyloidosis: A case report with a ten-year follow-up period. Clin Ophthalmol 2008;2:685-7.  Back to cited text no. 7
Chakraborti C, Chaudhury KP, Biswas RR. Primary conjunctival amyloidosis. Oman J Ophthalmol 2014;7:16-8.  Back to cited text no. 8
[PUBMED]  [Full text]  
Fraunfelder FW, Fraunfelder FT. Liquid nitrogen cryotherapy of a conjunctival vascular tumor. Cornea 2005;24:116-7.  Back to cited text no. 9


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