Clinical Features of AL Amyloidosis Presenting with Raccoon Eyes: A Case Report and Literature Review

Xie Yijie; Zhu Chao; Chen Zhou*

Xie Yijie, Zhu Chao and Chen Zhou*

Volume6-Issue9
Dates: Received: 2025-09-15 | Accepted: 2025-09-18 | Published: 2025-09-19
Pages: 1280-1289

Abstract

Objective: This study presents and examines the clinical profiles of 28 patients with immunoglobulin light chain (AL) Amyloidosis who exhibit the characteristic “raccoon eye” appearance. It seeks to delineate the typical clinical manifestations, diagnostic approaches, and therapeutic strategies associated with this condition, thereby aiming to augment the clinical awareness and treatment proficiency among physicians.

Methods: We present a case of “raccoon eye” in a 63-year-old male patient diagnosed with AL amyloidosis and detail his clinical presentation. Furthermore, we conducted a comprehensive literature review of related case reports in the PubMed database and emphasized the clinical features and treatment outcomes associated with AL amyloidosis featuring this rare form of periorbital purpura.

Results: We identified 27 cases of AL amyloidosis characterized by "raccoon eye" from the PubMed database, and including our reported case, a total of 28 patients were analyzed. Among them, 13 were male (46.4%) and 15 were female (53.6%), with a male-to-female ratio of 0.87:1. The average age for males was 66.31 ± 7.239 years, and for females, it was 66.00 ± 10.282 years. In these 28 patients, the majority exhibited renal and cardiac involvement. Renal involvement manifested as proteinuria or impaired renal function, while cardiac involvement was primarily characterized by left ventricular hypertrophy and early elevation of NT-proBNP levels. Additionally, some patients experienced involvement of the liver, lungs, nervous system, and macroglossia. AL amyloidosis can also present with nonspecific symptoms, mainly fatigue and unconscious weight loss. Six patients suffered from the disease within months after the appearance of the “raccoon eye” sign.

Conclusion: When the typical symptom of “raccoon eye” appears in patients, clinicians should be alert to the possibility of AL amyloidosis and promptly establish a diagnosis and initiate treatment.

FullText HTML FullText PDF DOI: 10.37871/jbres2183

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How to cite this article

Yijie X, Chao Z, Zhou C. Clinical Features of AL Amyloidosis Presenting with ‘Raccoon Eyes’: A Case Report and Literature Review. J Biomed Res Environ Sci. 2025 Sept 19; 6(9): 1280-1289. doi: 10.37871/jbres2183, Article ID: JBRES2183, Available at: https://www.jelsciences.com/articles/jbres2183.pdf

Subject area(s)

Pathology

Nephrology

References

Eder L, Bitterman H. Image in clinical medicine. Amyloid purpura. N Engl J Med. 2007 Jun 7;356(23):2406. doi: 10.1056/NEJMicm061510. PMID: 17554122.
Milutinovich J, Wu W, Savory J. Periorbital purpura after renal biopsy in primary amyloidosis. JAMA. 1979 Dec 7;242(23):2555. PMID: 490871.
Passos Rda H, Pereira A, Neto AC, Ribeiro AF, Kutner J, Hamerschlak N, Scheinberg M. Clinical image: bilateral black eyes (raccoon's eyes) in AL amyloidosis. Arthritis Rheum. 2006 Nov;54(11):3724. doi: 10.1002/art.22184. PMID: 17075892.
Lee HJ, Chang SE, Lee MW, Choi JH, Moon KC. Systemic amyloidosis associated with multiple myeloma presenting as periorbital purpura. J Dermatol. 2008 Jun;35(6):371-2. doi: 10.1111/j.1346-8138.2008.00487.x. PMID: 18578717.
Lestre S, Gonçalves A, João A, Ferreira A, Apetato M. Púrpura: manifestação de amiloidose sistémica primária [Purpura: primary systemic amyloidosis manifestation]. Acta Med Port. 2009 May-Jun;22(3):307-12. Portuguese. Epub 2009 Jul 15. PMID: 19686633.
Nicholson JA, Tappin J. Raccoon eyes in systemic AL amyloidosis. Br J Haematol. 2011 Jun;153(5):543. doi: 10.1111/j.1365-2141.2010.08489.x. Epub 2011 Apr 8. PMID: 21477158.
Eldho GC, Gailin BS, Mithun CM, Anoop TM, Sudha R. A case of dyspnea and periorbital rash. Proc (Bayl Univ Med Cent). 2012 Jul;25(3):248-9. doi: 10.1080/08998280.2012.11928842. PMID: 22754127; PMCID: PMC3377293.
Lavorato FG, Alves Mde F, Maceira JM, Unterstell N, Serpa LA, Azulay-Abulafia L. Primary systemic amyloidosis, acquired cutis laxa and cutaneous mucinosis in a patient with multiple myeloma. An Bras Dermatol. 2013 Nov-Dec;88(6 Suppl 1):32-5. doi: 10.1590/abd1806-4841.20132531. PMID: 24346874; PMCID: PMC3875965.
Colucci G, Alberio L, Demarmels Biasiutti F, Lämmle B. Bilateral periorbital ecchymoses. An often missed sign of amyloid purpura. Hamostaseologie. 2014;34(3):249-52. doi: 10.5482/HAMO-14-03-0018. Epub 2014 Jun 30. PMID: 24975676.
Hosoi T, Dhaliwal G, Tokuda Y. Amyloidosis in bilateral external auditory canals. BMJ Case Rep. 2014 May 13;2014:bcr2013200753. doi: 10.1136/bcr-2013-200753. PMID: 24825551; PMCID: PMC4024549.
Pereira VG, Jacinto M, Santos J, de Abreu TT. Periorbital purpura (raccoon's eyes). BMJ Case Rep. 2014 Aug 19;2014:bcr2013201407. doi: 10.1136/bcr-2013-201407. PMID: 25139913; PMCID: PMC4139576.
Inokuchi R, Tagami S, Maehara H. An elderly woman with bilateral raccoon eyes. Emerg Med J. 2016 Nov;33(11):781. doi: 10.1136/emermed-2015-205071. PMID: 28319930.
Kelsey A, Smith DH, Meng J, Murphy M, Rothe MJ. Amyloidosis: A story of how inframammary erosions eclipsed inconspicuous periorbital ecchymoses. Int J Womens Dermatol. 2016 Mar 2;2(1):18-22. doi: 10.1016/j.ijwd.2015.11.001. PMID: 28491996; PMCID: PMC5412114.
Forsyth CJ, Tiley CR. Raccoon eyes in systemic light chain amyloidosis. Med J Aust. 2017 May 15;206(9):384. doi: 10.5694/mja16.00848. PMID: 28490298.
Matsuura H, Anzai Y, Kuninaga N, Maeda T. Raccoon Eye Appearance: Amyloidosis. Am J Med. 2018 Jul;131(7):e305. doi: 10.1016/j.amjmed.2018.02.009. Epub 2018 Mar 14. PMID: 29550357.
Chandra A, Saha SK, Chakraborty U, Karmakar PS, Biswas G, Ray AK, Dutta S. Raccoon Eyes in Amyloidosis. Sultan Qaboos Univ Med J. 2020 Nov;20(4):e399-e400. doi: 10.18295/squmj.2020.20.04.021. Epub 2020 Dec 21. PMID: 33414950; PMCID: PMC7757934.
Mei S, Zhao Y, Li L, Mei C, Dai B. A 64-year-old woman with raccoon eyes following kidney biopsy: a case report. BMC Nephrol. 2020 Apr 17;21(1):140. doi: 10.1186/s12882-020-01770-4. PMID: 32303194; PMCID: PMC7165378.
Nguyen HT, Nguyen CTH. Cardiac amyloidosis mimicking acute coronary syndrome: a case report and literature review. Eur Heart J Case Rep. 2020 Oct 29;4(6):1-7. doi: 10.1093/ehjcr/ytaa325. PMID: 33442652; PMCID: PMC7793193.
Beatty PE, Killion L, Mc Hugh J, Tobin AM. Spontaneous bilateral peri-orbital purpura: an important clinical sign of primary systemic amyloidosis. BMJ Case Rep. 2021 Apr 1;14(4):e239478. doi: 10.1136/bcr-2020-239478. PMID: 33795269; PMCID: PMC8021580.
Chen-Xu M, Achilleos K. Periorbital purpura of AL amyloidosis. Rheumatology (Oxford). 2021 Mar 2;60(3):1165. doi: 10.1093/rheumatology/keaa545. PMID: 32944781.
Haász C, Kuroli E, Anker P, Márton DF, Szigeti Á, Nagy Z, Demeter J, Sárdy M, Medvecz M. Periorbitalis bőrtünetekkel járó könnyűlánc-amyloidosis [Case report of amyloid light-chain amyloidosis with periocular cutaneous involvement]. Orv Hetil. 2021 Aug 8;162(32):1303-1308. Hungarian. doi: 10.1556/650.2021.32137. PMID: 34370685.
LaTour D, Dao H, Limone B, Kivnick AR, Elsensohn A. Oral, periorbital, and inguinal purpura in a patient with paraproteinemia. JAAD Case Rep. 2022 Jul 2;27:79-81. doi: 10.1016/j.jdcr.2022.05.048. PMID: 35990230; PMCID: PMC9388871.
Mizuno S. Raccoon Eye Sign in Amyloid Light-chain Amyloidosis. Intern Med. 2022;61(15):2393. doi: 10.2169/internalmedicine.8927-21. Epub 2022 Aug 1. PMID: 35908961; PMCID: PMC9424071.
Sapkota S, Kuehl S, Pulluri B. Do Not Ignore Those Raccoon Eyes; They May Indicate Lethal AL Amyloidosis. Case Rep Oncol. 2022 Nov 11;15(3):1039-1048. doi: 10.1159/000527169. PMID: 36636680; PMCID: PMC9830280.
Jaber NF, Thelander U, Curman P. Extensive Amyloid Purpura: An Unusual Presentation of Myeloma-associated Light Chain Amyloidosis. Acta Derm Venereol. 2023 Jun 27;103:adv13367. doi: 10.2340/actadv.v103.13367. PMID: 37366561; PMCID: PMC10309058.
Mansilla-Polo M, Botella-Estrada R. Raccoon eyes as a diagnostic key for suspected amyloidosis. Rheumatol Adv Pract. 2023 Nov 28;8(1):rkad105. doi: 10.1093/rap/rkad105. PMID: 38076282; PMCID: PMC10710368.
Wang XF, Li T, Yang M, Huang Y. Periorbital purpura can be the only initial symptom of primary light chain amyloidosis: A case report. World J Clin Cases. 2024 Sep 16;12(26):5946-5951. doi: 10.12998/wjcc.v12.i26.5946. PMID: 39286381; PMCID: PMC11287502.
Senecal JB, Abou-Akl R, Allevato P, Mazzetti I, Hamm C, Parikh R, Woldie I. Amyloidosis: a case series and review of the literature. J Med Case Rep. 2023 Apr 21;17(1):184. doi: 10.1186/s13256-023-03886-1. PMID: 37081462; PMCID: PMC10120233.
Nader R, Angel-Korman A, Havasi A. Amyloidosis and the Kidney: An Update. Semin Nephrol. 2022 Nov;42(6):151343. doi: 10.1016/j.semnephrol.2023.151343. Epub 2023 May 4. PMID: 37148782.
Wangsa S, Novadian S, Zulkhair A. Renal amyloidosis: a narrative literature review. Bioscientia Medicina: J Biomed Transl Res. 2023;7(11):3753-3766.
Kumar N, Zhang NJ, Cherepanov D, Romanus D, Hughes M, Faller DV. Global epidemiology of amyloid light-chain amyloidosis. Orphanet J Rare Dis. 2022 Jul 19;17(1):278. doi: 10.1186/s13023-022-02414-6. PMID: 35854312; PMCID: PMC9295439.
Fotiou D, Dimopoulos MA, Kastritis E. Systemic AL Amyloidosis: Current Approaches to Diagnosis and Management. Hemasphere. 2020 Aug 10;4(4):e454. doi: 10.1097/HS9.0000000000000454. PMID: 32885146; PMCID: PMC7430233.
Aljama MA, Sidiqi MH, Dispenzieri A, Gertz MA, Lacy MQ, Buadi FK, Dingli D, Muchtar E, Fonder AL, Hayman SR, Hobbs MA, Gonsalves WI, Warsame RM, Kourelis T, Hwa YL, Kapoor P, Leung N, Go RS, Kyle RA, Rajkumar SV, Kumar SK. Comparison of different techniques to identify cardiac involvement in immunoglobulin light chain (AL) amyloidosis. Blood Adv. 2019 Apr 23;3(8):1226-1229. doi: 10.1182/bloodadvances.2019032458. PMID: 30975646; PMCID: PMC6482356.
Fotiou D, Theodorakakou F, Kastritis E. Biomarkers in AL Amyloidosis. Int J Mol Sci. 2021 Oct 9;22(20):10916. doi: 10.3390/ijms222010916. PMID: 34681575; PMCID: PMC8536050.
Aljama MA, Sidiqi MH, Dispenzieri A, Gertz MA, Lacy MQ, Buadi FK, Dingli D, Muchtar E, Fonder AL, Hayman SR, Hobbs MA, Gonsalves WI, Warsame RM, Kourelis T, Hwa YL, Kapoor P, Leung N, Go RS, Kyle RA, Rajkumar SV, Kumar SK. Comparison of different techniques to identify cardiac involvement in immunoglobulin light chain (AL) amyloidosis. Blood Adv. 2019 Apr 23;3(8):1226-1229. doi: 10.1182/bloodadvances.2019032458. PMID: 30975646; PMCID: PMC6482356.
Rajkumar SV, Dimopoulos MA, Palumbo A, Blade J, Merlini G, Mateos MV, Kumar S, Hillengass J, Kastritis E, Richardson P, Landgren O, Paiva B, Dispenzieri A, Weiss B, LeLeu X, Zweegman S, Lonial S, Rosinol L, Zamagni E, Jagannath S, Sezer O, Kristinsson SY, Caers J, Usmani SZ, Lahuerta JJ, Johnsen HE, Beksac M, Cavo M, Goldschmidt H, Terpos E, Kyle RA, Anderson KC, Durie BG, Miguel JF. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014 Nov;15(12):e538-48. doi: 10.1016/S1470-2045(14)70442-5. Epub 2014 Oct 26. PMID: 25439696.
Rajkumar SV. Multiple myeloma: 2016 update on diagnosis, risk-stratification, and management. Am J Hematol. 2016 Jul;91(7):719-34. doi: 10.1002/ajh.24402. PMID: 27291302; PMCID: PMC5291298.
Muchtar E, Gertz MA, Kumar SK, Lacy MQ, Dingli D, Buadi FK, Grogan M, Hayman SR, Kapoor P, Leung N, Fonder A, Hobbs M, Hwa YL, Gonsalves W, Warsame R, Kourelis TV, Russell S, Lust JA, Lin Y, Go RS, Zeldenrust S, Kyle RA, Rajkumar SV, Dispenzieri A. Improved outcomes for newly diagnosed AL amyloidosis between 2000 and 2014: cracking the glass ceiling of early death. Blood. 2017 Apr 13;129(15):2111-2119. doi: 10.1182/blood-2016-11-751628. Epub 2017 Jan 26. PMID: 28126928; PMCID: PMC5391625.
Wechalekar AD, Schonland SO, Kastritis E, Gillmore JD, Dimopoulos MA, Lane T, Foli A, Foard D, Milani P, Rannigan L, Hegenbart U, Hawkins PN, Merlini G, Palladini G. A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis. Blood. 2013 Apr 25;121(17):3420-7. doi: 10.1182/blood-2012-12-473066. Epub 2013 Mar 11. PMID: 23479568.