Clinical Characteristics, Etiologic Associations, Laboratory Findings, Treatment, and Proposal of Diagnostic Criteria of Pernio (Chilblains) in a Series of 104 Patients at Mayo Clinic, 2000 to 2011

Abstract Objective To further characterize the clinical features, etiologic associations, laboratory findings, and treatment of pernio. Patients and Methods This is a retrospective review of patients with pernio seen at our institution between January 1, 2000, and December 31, 2011. Results Of 104 patients with pernio (mean age at diagnosis, 38.3 years), 82 (79%) were women. Pernio affected the toes in 85 patients (82%) and the fingers in 31 (30%). Thirty-eight patients (37%) had at least 1 abnormal laboratory test result, and test results were positive for cold agglutinins in 11 (55%) of 20 tested patients. Results were negative for cryoglobulins in all tested patients (n=53). Four patients (4%) had connective tissue disease (nonlupus) associated with pernio; 3 patients (3%) had an associated hematologic malignant disease. Conservative treatments (eg, warming, drying, and smoking cessation) provided complete response in 23 (82%) of 28 patients with follow-up data. Conclusion To our knowledge, this study represents one of the largest single-center case series of pernio to date. Most of the patients did not have an underlying systemic association with pernio, although a few patients had pernio in association with connective tissue disease or hematologic malignant disease.