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

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.