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Requests for reprints should be addressed to Hiroki Matsuura, MD, Department of General Internal Medicine, Kurashiki Central Hospital, 1-1-1, Miwa, Kurashiki-city, Okayama 710-8602, Japan.
An 82-year-old woman presented to our outpatient clinic with a 6-month history of
recurrent internal bleeding near the eyes. The patient had a history of type 2 diabetes
mellitus, but had no significant medical history including traumatic injuries. Physical
examination revealed bilateral periorbital ecchymosis and large tongue (Figure). Serum protein electrophoresis revealed an M-peak in the gamma fraction, and urine
immunofixation electrophoresis was positive for Bence-Jones protein. In addition,
amyloid fibrils were detected in biopsy of abdominal subcutaneous fat tissue. On the
basis of the clinical and immunohistological findings, we made a diagnosis of amyloid
light-chain amyloidosis. Amyloid light-chain amyloidosis is a rare, multisystem disease
characterized by the deposition of amyloid in various organs and extracellular tissue,
including the kidney, heart, liver, digestive tract, spleen, and autonomic nervous
system.
However, symptoms in the early stage vary widely and include weakness, epigastric
discomfort, weight loss, and poor appetite. These symptoms are often vague and nonspecific,
and can be confused with other disorders. The gradual amyloid deposition results in
damage and affected function in the organs and tissues, and the other symptoms in
the most commonly affected organs and tissues include nephrotic-range proteinuria,
congestive cardiomyopathy, macroglossia, hematemesis, hematochezia, and autonomic
or sensory neuropathy. Untreated amyloidosis has progressive, irreversible consequences
and can ultimately result in death. Thus, an early and precise diagnosis of amyloid
light-chain amyloidosis is essential in order to prevent further organ damage. Therefore,
when the raccoon eye appearance is present, clinicians should consider amyloidosis
as a possible differential diagnosis.
FigurePhotograph of the patient's face shows bilateral periorbital ecchymosis (raccoon eye
appearance).
Funding: The authors do not have any funding sources in the writing of the manuscript or the decision to submit for publication.
Conflicts of Interest: The authors do not have any conflicts of interest.
Authorship: HM contributed to patient care, wrote the manuscript, and contributed to discussion. YA, NK, and TA also contributed to patient care and discussion.
