Cold agglutinin disease--the importance of cutaneous signs

We present the case of a woman where the diagnosis of cold agglutinin disease could be made after we had noticed slight cutaneous manifestations during a routine examination. Leading symptoms were livedo reticularis of the thighs and a history of acrocyanosis and Raynaud's phenomenon upon cold exposure. The current knowledge about the etiology, clinical presentation and treatment of the disease is briefly discussed.