Essential mixed cryoglobulinemia manifesting as recurrent perioral edema: report of a case.

Abstract

Cryoglobulins are immunoglobulins that precipitate in vitro at temperatures <37°C but that redissolve if heated; they are broadly divided into 3 classes. Cryoglobulinemia has a plethora of clinical manifestations, but it typically presents a vasculitic syndrome, most notably a triad of purpura, arthralgia, and weakness secondary to cryoglobulin deposition in small or medium-sized blood vessels. The highly variable clinical presentation and inconsistent laboratory isolation of cryoglobulins mean that the diagnosis may be one of exclusion or of retrospective review. We describe a case of a 67-year-old woman whose principal symptom was recurrent perioral edema, which was characteristically induced by cold. Raised cryoglobulins combined with a positive autoantibody screen suggested mixed cryoglobulinemia secondary to Sjögren syndrome. However, in the subsequent 6 years, she has remained generally well, having developed no autoimmune, neoplastic, or other disease, suggesting the diagnosis is one of "essential" mixed cryoglobulinemia. No treatment other than symptomatic analgesia has been required.

• Authors: Stergios Doumas, Alexandros Kolokotronis, Matina Zormpa, A W Barrett
• Journal: Oral surgery, oral medicine, oral pathology and oral radiology (Oral Surg Oral Med Oral Pathol Oral Radiol) Vol. 117 Issue 1 Pg. e57-60 (Jan 2014) ISSN: 2212-4411 [Electronic] United States
• PMID: 24332336 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2014 Elsevier Inc. All rights reserved.

Chemical References

• Autoantibodies

Topics

• Aged
• Autoantibodies (analysis)
• Cold Temperature
• Cryoglobulinemia (complications, diagnosis)
• Diagnosis, Differential
• Edema (etiology)
• Female
• Humans
• Mouth (pathology)
• Mouth Diseases (etiology)
• Sjogren's Syndrome (diagnosis)