2nd August 2013, Volume 126 Number 1379

Michael Prystajecky, Habib U Rehman

An 85-year-old woman was seen in the emergency department with abdominal pain and constipation. Her medical history included dialysis-dependent chronic renal impairment, AL amyloidosis, and monoclonal IgG lambda gammopathy.

Bilateral periorbital (“raccoon eyes”) and nasolabial fold ecchymosis was noticed (see Figures 1 and 2). Raccoon eyes are due to deposition of amyloid in the capillary walls, leading to fragility of capillaries, which can burst after minor trauma like sneezing, cough, or rubbing the eyes. Raccoon eyes can be a sign of bleeding diathesis in AL amyloidosis, particularly if the patient has acquired factor X deficiency.

Figure 1
Figure 2

pry1

pry2

Focal or generalised haemorrhage is a commonly encountered clinical problem in patients with amyloidosis and can prove fatal at times. The bleeding manifestations frequently observed in patients with immunoglobulin light chain amyloidosis (AL) have been attributed to different pathogenic factors including amyloid deposits in capillary walls, deficiency of some clotting factors, and the presence of plasma components interfering with fibrin formation. However, in one study involving 100 patients, bleeding occurred frequently in the absence of abnormalities of clotting tests, suggesting that haemorrhage in amyloidosis is most often due to amyloid infiltration of blood vessels.1

Raccoon eye sign is not pathognomonic of amyloidosis, and bilateral periorbital ecchymosis has also been described in thoracic and fractures of the skull base.2,3 The occurrence of periorbital ecchymosis, either spontaneously or after minimal trauma or Valsalva manoeuver, should raise the possibility of vascular fragility secondary to amyloidosis.

Author Information

Michael Prystajecky, Internal Medicine Resident, College of Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada; Habib U Rehman, Clinical Associate Professor; Department of Medicine, Qu’Appelle Health Region, Regina General Hospital, Regina, Saskatchewan, Canada

Correspondence

Dr HU Rehman, Clinical Associate Professor, Department of Medicine, Regina Qu’Appelle Health Region, Regina General Hospital, 1440 – 14th Avenue, Regina, SK, S4P 0W5, Canada.

Correspondence Email

habib31@sasktel.net

References

  1. Yood RA, Skinner M, Rubinow A, et al. Bleeding manifestations in 100 patients with amyloidosis. JAMA 1983; 249:1322–4.
  2. Deakin CD. Bilateral periorbital hematoma (raccoon eyes) following thoracic crush injuries: case reports. J Trauma 1995;38:816–17.
  3. Herbella FA, Mudo M, Delmonti C, et al. ‘Raccoon eyes’ (periorbital haematoma) as a sign of skull base fracture. Injury 2001;32:745–7.