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.