Cryoglobulinemia

Abstract

Cryoglobulins are proteins that precipitate in the cold. Cryoglobulinemia is usually asymptomatic, but in some instances it manifests as a disease affecting one or all organs. The prevalence of cryoglobulinemia is reported as approximately 1:100,000. Intestinal involvement in cryoglobulinemia occurs infrequently and can mimic inflammatory bowel disease. We present an atypical case of cryoglobulinemia with gastrointestinal manifestation. A 54 year old female with history of hepatitis C and schizophrenia presented to the emergency department with diarrhea, fever and rash for 1 week. She had completed a course of antibiotics two weeks prior for an upper respiratory infection. She reported 7–10 watery non bloody bowel movements and denied prior similar episodes. She denied nausea or vomiting, sick contacts, recent travel or unusual food intake. Her medications included risperdal, xanax and valium. On physical exam: T 102.2, BP of 100/67 mm of Hg, RR 22, and O2 sat 92% on room air. She was ill appearing. Her oropharynx was notable diffusely erythematous mucosa with ulcerations, she had no lymphadenopathy. Her lung exam revealed bibasilar crackles. Heart and Abdominal exams were unremarkable. Rectal exam revealed guaiac positive brown stool. Skin exam was consistent with non blanching malar rash on her face as well as scattered, weeping nodules. Significant labs included, WBC 3.2 th/l, hct 33%, MCV 87. Platelet count 192 th/cumm, AST 186 IU/l, ALT 103 IU/l, amylase 50 U/l, lipase 40U/l. Urine analysis showed moderate RBC and proteinuria. Blood cultures were negative. Clostridium difficile serology was negative. Her serum revealed the presence of cryoglobulins. Chest X ray showed bibasilar infiltrates and small pleural effusions and echo findings were consistent with pericardial effusion. Skin biopsy revealed vacuolar interface dermatitis with epidermal atrophy, prominent dermal leukocytoclasis and hemorrhage consistent with cryoglobulinemia type III. The patient was admitted and treated with several rounds of plasmapheresis following which her pericarditis and pleuritis subsided and her diarrhea resolved. We believe this puzzling case illustrates cryoglobulinemia with multiple organ involvement. Acute diarrhea can be a frequent presentation of many common conditions but after ruling out other potential causes we believe that this patients acute diarrhea was due to intestinal vasculitis and that may be a manifestation of cryoglobulinemia.