Remittent idiopathic necrotizing acrocyanosis - A rare entity

Abstract

Remittent idiopathic necrotizing acrocyanosis is a very rare condition characterized by persistent systemic cyanotic or erythrocyanotic discoloration of hands and feet. It is associated with pain, tenderness of fingers and toes and may present as ulceration or gangrene of extremities. It is aggravated with cold exposure but persists even in summer. Acrocyanosis is not due to any systemic disease; peripheral arteriolar constriction with secondary vasodilatation due to disordered vascular tone of unknown etiology has been postulated. It responds to peripheral vasodilator drug but usually needs continuous long term therapy along with avoidance of cold exposure. We report the case of a 53-year-old male farmer with remittent necrotizing acrocyanosis.