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Published in: BMC Ophthalmology 1/2015

Open Access 01-12-2015 | Case report

Systemic amyloidosis with bilateral conjunctival involvement: a case report

Authors: Leandro J. Correa, J. Pablo Maccio, Evangelina Esposito, Rodolfo Monti, M. Eugenia Gonzalez-Castellanos, Martin Paradelo, Horacio M. Serra, Julio A. Urrets-Zavalia

Published in: BMC Ophthalmology | Issue 1/2015

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Abstract

Background

Conjunctival 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 Presentation

A 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.

Conclusions

Conjunctival 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.
Literature
2.
go back to reference Westermark P, Benson MD, Buxbaum JN, Cohen AS, Frangione B, Ikeda S, et al. Amyloid: Toward terminology clarification. Report from the Nomenclature Committee of the International Society of Amyloidosis. Amyloid. 2005;12:1–4.CrossRefPubMed Westermark P, Benson MD, Buxbaum JN, Cohen AS, Frangione B, Ikeda S, et al. Amyloid: Toward terminology clarification. Report from the Nomenclature Committee of the International Society of Amyloidosis. Amyloid. 2005;12:1–4.CrossRefPubMed
3.
go back to reference Leibovitch I, Selva D, Goldberg RA, Sullivan TJ, Saeed P, Davis G, et al. Periocular and orbital amyloidosis: clinical characteristics, management, and outcome. Ophthalmology. 2006;113:1657–64.CrossRefPubMed Leibovitch I, Selva D, Goldberg RA, Sullivan TJ, Saeed P, Davis G, et al. Periocular and orbital amyloidosis: clinical characteristics, management, and outcome. Ophthalmology. 2006;113:1657–64.CrossRefPubMed
4.
go back to reference Demirci H, Shields CL, Eagle Jr RC, Shields JA. Conjuctival amyloidosis: report of six cases and review of the literature. Surv Ophthalmol. 2006;51:419–33.CrossRefPubMed Demirci H, Shields CL, Eagle Jr RC, Shields JA. Conjuctival amyloidosis: report of six cases and review of the literature. Surv Ophthalmol. 2006;51:419–33.CrossRefPubMed
5.
go back to reference Westermark GT, Fändrich M, Westermark P. AA amyloidosis: pathogenesis and targeted therapy. Annu Rev Pathol. 2015;10:321–44.CrossRefPubMed Westermark GT, Fändrich M, Westermark P. AA amyloidosis: pathogenesis and targeted therapy. Annu Rev Pathol. 2015;10:321–44.CrossRefPubMed
6.
go back to reference Glenner GG, Terry W, Harada M, Isersky C, Page D. Amyloid fibril proteins: proof of homology with immunoglobulin light chains by sequence analyses. Science. 1971;172:1150–1.CrossRefPubMed Glenner GG, Terry W, Harada M, Isersky C, Page D. Amyloid fibril proteins: proof of homology with immunoglobulin light chains by sequence analyses. Science. 1971;172:1150–1.CrossRefPubMed
7.
go back to reference Lachmann HJ, Goodman HJ, Gilbertson JA, Gallimore JR, Sabin CA, Gillmore JD, et al. Natural history and outcome in systemic AA amyloidosis. N Engl J Med. 2007;356:2361–71.CrossRefPubMed Lachmann HJ, Goodman HJ, Gilbertson JA, Gallimore JR, Sabin CA, Gillmore JD, et al. Natural history and outcome in systemic AA amyloidosis. N Engl J Med. 2007;356:2361–71.CrossRefPubMed
8.
go back to reference Rowczenio DM, Noor I, Gillmore JD, Lachmann HJ, Whelan C, Hawkins PN, et al. Online registry for mutations in hereditary amyloidosis including nomenclature recommendations. Hum Mutat. 2014;35:E2403–12.CrossRefPubMed Rowczenio DM, Noor I, Gillmore JD, Lachmann HJ, Whelan C, Hawkins PN, et al. Online registry for mutations in hereditary amyloidosis including nomenclature recommendations. Hum Mutat. 2014;35:E2403–12.CrossRefPubMed
9.
go back to reference Al-Nuaimi D, Bhatt PR, Steeples L, Irion L, Bonshek R, Leatherbarrow B. Amyloidosis of the orbit and adnexae. Orbit. 2012;31:287–98.CrossRefPubMed Al-Nuaimi D, Bhatt PR, Steeples L, Irion L, Bonshek R, Leatherbarrow B. Amyloidosis of the orbit and adnexae. Orbit. 2012;31:287–98.CrossRefPubMed
10.
go back to reference Aryasit O, Preechawai P, Kayasut K. Clinical presentation, treatment, and prognosis of periocular and orbital amyloidosis in a university-based referral center. Clin Ophthalmol. 2013;7:801–5.CrossRefPubMedPubMedCentral Aryasit O, Preechawai P, Kayasut K. Clinical presentation, treatment, and prognosis of periocular and orbital amyloidosis in a university-based referral center. Clin Ophthalmol. 2013;7:801–5.CrossRefPubMedPubMedCentral
11.
go back to reference Ray M, Tan AW, Thamboo TP. Atypical presentation of primary conjuctival amiloidosis. Can J Ophthalmol. 2012;47:e2–4.CrossRefPubMed Ray M, Tan AW, Thamboo TP. Atypical presentation of primary conjuctival amiloidosis. Can J Ophthalmol. 2012;47:e2–4.CrossRefPubMed
12.
go back to reference Abdallah AO, Westfall C, Brown H, Muzaffar J, Atrash S, Nair B. Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis. Am J Case Rep. 2012;13:102–5.CrossRefPubMedPubMedCentral Abdallah AO, Westfall C, Brown H, Muzaffar J, Atrash S, Nair B. Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis. Am J Case Rep. 2012;13:102–5.CrossRefPubMedPubMedCentral
13.
go back to reference Purcell Jr JJ, Birkenkamp R, Tsai CC, Riner RN. Conjunctival involvement in primary systemic nonfamilial amyloidosis. Am J Ophthalmol. 1983;95:845–7.CrossRefPubMed Purcell Jr JJ, Birkenkamp R, Tsai CC, Riner RN. Conjunctival involvement in primary systemic nonfamilial amyloidosis. Am J Ophthalmol. 1983;95:845–7.CrossRefPubMed
14.
go back to reference Iijima S. Primary systemic amyloidosis: a unique case complaining of diffuse eyelid swelling and conjunctival involvement. J Dermatol. 1992;19:113–8.CrossRefPubMed Iijima S. Primary systemic amyloidosis: a unique case complaining of diffuse eyelid swelling and conjunctival involvement. J Dermatol. 1992;19:113–8.CrossRefPubMed
15.
go back to reference Shields JA, Eagle RC, Shields CL, Green M, Singh AD. Systemic amyloidosis presenting as a mass of the conjunctival semilunar fold. Am J Ophthalmol. 2000;130:523–5.CrossRefPubMed Shields JA, Eagle RC, Shields CL, Green M, Singh AD. Systemic amyloidosis presenting as a mass of the conjunctival semilunar fold. Am J Ophthalmol. 2000;130:523–5.CrossRefPubMed
16.
go back to reference Rodrigues G, Sanghvi V, Lala M. A rare cause of unilateral upper and lower eyelid swelling: isolated conjuctival amyloidosis. Korean J Ophthalmol. 2001;15:38–40.CrossRefPubMed Rodrigues G, Sanghvi V, Lala M. A rare cause of unilateral upper and lower eyelid swelling: isolated conjuctival amyloidosis. Korean J Ophthalmol. 2001;15:38–40.CrossRefPubMed
17.
go back to reference Patrinely JR, Koch DD. Surgical management of advanced ocular adnexal amyloidosis. Arch Ophthalmol. 1992;110:882–5.CrossRefPubMed Patrinely JR, Koch DD. Surgical management of advanced ocular adnexal amyloidosis. Arch Ophthalmol. 1992;110:882–5.CrossRefPubMed
18.
go back to reference Fraunfelder FW. Liquid nitrogen cryotherapy for conjunctival amyloidosis. Arch Ophthalmol. 2009;127:645–8.CrossRefPubMed Fraunfelder FW. Liquid nitrogen cryotherapy for conjunctival amyloidosis. Arch Ophthalmol. 2009;127:645–8.CrossRefPubMed
19.
go back to reference Khaira M, Mutamba A, Meligonis G, Rose GE, Plowman PN, O´Donnell H. The use of radiotherapy for the treatment of localized orbital amyloidosis. Orbit. 2008;27:432–7.CrossRefPubMed Khaira M, Mutamba A, Meligonis G, Rose GE, Plowman PN, O´Donnell H. The use of radiotherapy for the treatment of localized orbital amyloidosis. Orbit. 2008;27:432–7.CrossRefPubMed
20.
go back to reference Murdoch IE, Sullivan TJ, Moseley I, Hawkins PN, Pepys MB, Tan SY, et al. Primary localized amyloidosis of the orbit. Br J Ophthalmol. 1996;80:1083–6.CrossRefPubMedPubMedCentral Murdoch IE, Sullivan TJ, Moseley I, Hawkins PN, Pepys MB, Tan SY, et al. Primary localized amyloidosis of the orbit. Br J Ophthalmol. 1996;80:1083–6.CrossRefPubMedPubMedCentral
21.
go back to reference Seider N, Beiran I, Kaltreider SA. Advanced localized primary conjunctival amyloidosis: surgical rehabilitation using mucous membrane graft implantation. Isr Med Assoc J. 2006;8:439–40.PubMed Seider N, Beiran I, Kaltreider SA. Advanced localized primary conjunctival amyloidosis: surgical rehabilitation using mucous membrane graft implantation. Isr Med Assoc J. 2006;8:439–40.PubMed
Metadata
Title
Systemic amyloidosis with bilateral conjunctival involvement: a case report
Authors
Leandro J. Correa
J. Pablo Maccio
Evangelina Esposito
Rodolfo Monti
M. Eugenia Gonzalez-Castellanos
Martin Paradelo
Horacio M. Serra
Julio A. Urrets-Zavalia
Publication date
01-12-2015
Publisher
BioMed Central
Published in
BMC Ophthalmology / Issue 1/2015
Electronic ISSN: 1471-2415
DOI
https://doi.org/10.1186/s12886-015-0075-2

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