By Gary M. White, MD

Cold agglutinin disease is a rare form of autoimmune hemolytic anemia in which exposure to cold triggers thrombi formation.


The ears and other acral sites become red and inflamed upon exposure to the cold.


Cold avoidance is key. Systemic steroids have been used as have cytotoxic agents, plasmapheresis and rituximab.


Red, inflamed ears after a day exposed to the cold on a boat. JAMA Derm 2015;151;785.


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