Cyanosis With Dysautonomia Mimics Raynaud Disease

Abstract

Patients with Ehlers-Danlos syndrome often present with discoloration of distal extremities, suggesting Raynaud disease. They are known to have an increased incidence of dysautonomia. A 16-year-old white female adolescent presented with purple feet and toes. The photos (Figs. 1, 2) show her purple feet and toes (cyanosis). Cyanosis, whichmay be due to deoxygenated blood from venous pooling, gives the appearance that may be confused with Raynaud disease. This prompted her rheumatology consultation for Raynaud disease. Her toes and feet felt cold to the touch; however, theywere notworsened by the cold. The patient s history and physical revealed no evidence to suggest autoimmune disease. Specifically, there was no history of triple phase color response, dysphagia, dyspnea, or muscle weakness. Clinical examination revealed no sclerodactyly, telangiectasia, periungual erythema, digital infarction, nodules, or calcinosis, and analysis of nailfold capillaries was normal. Her pertinent laboratory results included negative anti–nuclear antibodies, anti–double-stranded DNA antibody,