Livedo Reticularis and Cold Agglutinin Disease

Livedo reticularis is a symptom of cold agglutinin disease (CAD), an autoimmune disorder caused by autoantibodies called cold agglutinins when exposed to cold temperatures.

Cold agglutinins bind to red blood cells (RBCs) in cold conditions and cause them to clump, or agglutinate. This activates the complement pathway of the immune system and ultimately causes hemolytic anemia because of the excessive lysis, or disintegration, of the RBCs, leading to low hemoglobin levels. Hemoglobin is the protein in RBCs that carries oxygen.

What is livedo reticularis?

Livedo reticularis can be described as a striking reddish-brown to a bluish blotched and reticular (net-like) pattern of the peripheral blood vessels surrounding a pale central skin area.

Livedo reticularis is especially observed in the legs and occurs when exposed to cold temperatures in CAD patients because of disturbances in blood flow and reduced oxygen supply to the skin.

Diagnosis of livedo reticularis

The diagnosis of livedo reticularis is straightforward because it presents itself as reticulate architecture of the peripheral blood vessels on the skin.

Livedo reticularis can occur as a result of several diseases apart from CAD, including infections, cancer, neurological conditions, endocrine disorders, or as a side effect of certain medications.

Management of livedo reticularis

Livedo reticularis is generally benign in CAD and can be managed either by exposure to warmth or treatment of the underlying CAD. These treatments include plasmapheresis, blood transfusion, or treatment with rituximab alone or in combination with other medicines.

Last updated: Aug. 10, 2019


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