Cutaneous Necrosis Associated with Cold Agglutinins

This article is an answer to the Case – Necrotic Skin Lesions on Nose, Ears, and Legs after Exposure to Cold

Histologic examination revealed thrombosis of all dermal and subcutaneous vessels but almost no inflammatory infiltration and no signs of vasculitis.

A test for cold agglutinins was positive at a 1:64 dilution, and there were signs of a mild hemolytic anemia. Screening for hypercoagulability and paraproteins was negative, and there were no signs of cancer.

Cold agglutinins (immunoglobulins, predominantly IgM) typically lead to agglutination of erythrocytes below certain temperatures, causing impaired microcirculation and hemolysis.

The patient was treated with heparin, iloprost, plasmapheresis, and surgical débridement of necrotic skin and was given strict instructions to protect herself against cold. After initial improvement, she was lost to follow-up.