Neurovascular Instability Syndrome: A Unifying Term to Describe the Coexistence of Temperature-Related Vascular Disorders in Affected Patients

Abstract

Temperature-related vascular disorders including pernio, erythromelalgia, and Raynaud phenomenon present a diagnostic and therapeutic challenge. We describe the unusual cooccurrence of perniosis and erythromelalgia. Although these conditions are aggravated by disparate environmental conditions, their coexistence may help to elucidate their pathophysiologic characteristics. A 37-year-old woman was referred to our institution 2 years after the onset of erythema, pain, and dusky papules on the feet for which extensive diagnostic workup and therapeutic trials had been unsuccessful. Initially, she had developed painful red papules on the toes triggered by cold temperature. Evaluation of a skin biopsy specimen showed findings consistent with pernio.