Chilblains and Raynaud Phenomenon are Usually Not a Sign of Hereditary Protein C and S Deficiencies.

Abstract

Hereditary protein C and S deficiencies are risk factors for thrombosis. They are associated with purpura fulminans and coumarin-induced skin necrosis. Recently;necrotic livedo of the extremities;severe chilblains and severe frostbite have been observed in protein C or S deficient patients. Our study was designed to evaluate the prevalence of cold-induced acral manifestations in patients with protein C or S deficiency. One-hundred-and-six patients with protein C or S deficiency and controls matched for sex and age were studied by questionnaire. Data included any history of acral manifestation possibly related to cold exposure;i.e. chilblains;Raynaud phenomenon;acrocyanosis and possible associated factors. Assessment of the diagnosis by a dermatologist was recorded. No difference was found in the prevalence of acral manifestations between patients and controls. This study suggests that protein C and S deficiencies are not risk factors for cold-induced acral manifestations.