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.