Abstract
Purpose
Conjunctival amyloidosis is a rare disorder. It is often clinically not suspected or diagnosed. This study intended to demonstrate the clinical and histopathologic features of this infrequent disease, including an immunohistochemical search for amyloidotic proteins.
Methods
Retrospective case series of the clinical and histopathologic characteristics of six patients with conjunctival amyloidosis. Immunohistochemical analysis with respect to possible amyloidotic components of the conjunctival deposits was performed.
Results
The diagnosis of amyloidosis was not suspected in all six cases presenting with an amelanotic conjunctival lesion. In three patients a conjunctival tumor of unknown origin, in one case each a papillomatous alteration of the conjunctiva, a conjunctival granulomatous inflammation, and a lymphoma were assumed respectively. The diagnosis of amyloidosis was made by histopathology. Immunohistochemical examination found lambda and kappa light chains as well as prealbumin within the amyloid deposits in one of the six specimens.
Conclusions
The diagnosis of amyloidosis has to be kept in mind in cases with an unclear conjunctival mass or inflammatory process. Only a tissue biopsy is able to prove the diagnosis. A possible underlying systemic disease has to be ruled out.
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References
Merlini G, Bellotti V (2003) Molecular mechanisms of amyloidosis. N Engl J Med 349:583–596
Fernandez-Flores A (2012) Cutaneous amyloidosis: a concept review. Am J Dermatopathol 34:1–17
Puchtler H, Sweat F, Levine M (1962) On the binding of congo red by amyloid. J Histochem Cytochem 10:355–364
Linke RP, Altland K, Ernst J, Gerhard L, Michels H, Saeger W, Willig F (1998) Praktische hinweise zur diagnose und therapie generalisierter amyloidosen. Deut Ärzteblatt 95:2626–2636
Demirci H, Shields CL, Eagle RC, Shields JA (2006) Conjunctival amyloidosis: report of six cases and review of the literature. Surv Ophthalmol 51:419–433
Madangopal AV (1962) Conjunctival amyloidosis. Br J Ophthalmol 46:749–752
Mathur SP, Mathur BP (1959) Conjunctival amyloidosis. Br J Ophthalmol 43:765–766
Jain NS, Gupta AN (1966) Amyloidosis of the conjunctiva. Br J Ophthalmol 50:102–104
Borodic GE, Beyer-Machule CK, Millin J, Conte J, Foster CS (1984) Immunoglobulin deposition in localized conjunctival amyloidosis. Am J Ophthalmol 98:617–622
Rosenblatt M, Spitz GF, Friedman AH, Kazam ES (1986) Localized conjunctival amyloidosis: case reports and review of the literature. Ophthalmologica 192:238–245
Marsh WM, Streeten BW, Hoepner JA, Zhang W, Davey FR (1987) Localized conjunctival amyloidosis associated with extranodal lymphoma. Ophthalmology 94:61–64
Moorman CM, McDonald B (1997) Primary (localized non-familial) conjunctival amyloidosis: three case reports. Eye 11:603–606
Shields JA, Eagle RC, Shields CL, Green MG, Singh AD (2000) Systemic amyloidosis presenting as a mass of the conjunctival semilunar fold. Am J Ophthalmol 130:523–525
Chaturvedi P, Lala M, Desai S, Sanghvi V (2000) A rare case of both eyelids swelling: isolated conjunctival amyloidosis. Indian J Ophthalmol 48:56–57
Pirouzmand F, Hurwitz J, Howarth D (2002) Primary localized bilateral conjunctival amyloidosis and epiphora. Orbit 21:139–144
Ray M, Tan AWT, Thamboo TP (2012) Atypical presentation of primary conjunctival amyloidosis. Can J Ophthalmol 47:e2–e4
Abdallah A-O, Westfall C, Brown H, Muzaffar J, Atrash S, Nair B (2012) Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis. Am J Case Rep 13:102–105
Lee H-M, Naor J, DeAngelis D, Rootman DS (2000) Primary localized conjunctival amyloidosis presenting with recurrence of subconjunctival hemorrhage. Am J Ophthalmol 129:245–247
Iijima S (1992) Primary systemic amyloidosis: a unique case complaining of diffuse eyelid swelling and conjunctival involvement. J Dermatol 19:113–118
Purcell JJ, Birkenkamp R, Tsai CC, Riner RN (1983) Conjunctival involvement in primary systemic nonfamilial amyloidosis. Am J Ophthalmol 95:845–847
Van der Hilst JCH, van der Meer JWM, Drenth JPH, Simon A (2007) AL amyloidosis enhances development of amyloid A amyloidosis. Br J Ophthalmol 156:748–749
Rodrigues MM, Cullen G, Shannon G (1976) Primary localized conjunctival amyloidosis following strabismus surgery. Can J Ophthalmol 11:177–179
Smith ME, Zimmerman LE (1966) Amyloidosis of the eyelid and conjunctiva. Arch Ophthalmol 75:42–51
Auw-Hädrich C, Witschel H (1997) Recurrent bilateral papillomatous conjunctival tumors. Isolated bilateral amyloidosis of the conjunctiva. Ophthalmologe 94:832–833
Topalkara A, Ben-Arie-Weintrob Y, Ferry JA, Foster CS (2007) Conjunctival marginal zone B-cell lymphoma (MALT lymphoma) with amyloid and relapse in the stomach. Ocul Immunol Inflamm 15:347–350
Setoguchi M, Hoshii Y, Takahashi M, Tanaka T, Nishida T, Ishihara T (1999) Conjunctival AL amyloidosis associated with a low-grade B-cell lymphoma. Amyloid 6:210–214
Khalifa YM, Bloomer MM, Margolis TP (2010) Secondary localized sectoral keratokonjunctival amyloidosis from ocular trauma. Cornea 29:1328–1329
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Suesskind, D., Ziemssen, F. & Rohrbach, J.M. Conjunctival amyloidosis — clinical and histopathologic features. Graefes Arch Clin Exp Ophthalmol 253, 1377–1383 (2015). https://doi.org/10.1007/s00417-015-2932-3
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DOI: https://doi.org/10.1007/s00417-015-2932-3