We present the case of a woman where the diagnosis of cold agglutinin disea
se could be made after we had noticed slight cutaneous manifestations durin
g a routine examination. Leading symptoms were livedo reticularis of the th
ighs and a history of acro-cyanosis and Raynaud's phenomenon upon cold expo
sure. The current knowledge about the etiology, clinical presentation and t
reatment of the disease is briefly discussed. Copyright (C) 2001 S.Karger A
G, Basel.