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Woman Develops Purple Lace-Like Rash Following New York Cold Spell


A cold snap in upstate New York that dropped local temperatures to -9°C (15°F) sent one woman to the hospital with a rare, purple lace-like rash that covered her entire body. Konika Sharma/NEJM

A cold snap in upstate New York that dropped local temperatures to -9°C (15°F) sent one woman to the hospital with a rare, purple lace-like rash that covered her entire body.

For a week, the 70-year-old woman had felt dizzy and displayed the symptoms of a rash. Two weeks prior to that, she had experienced a viral respiratory tract infection. Doctors examining her found that she had a “generalized, macular, nonblanching rash” – a rash often associated with meningitis that doesn’t fade when pressed with a glass. It had a “reticular pattern with purplish discoloration consistent with livedo reticularis,” a condition that is caused by spasms of blood vessels or abnormal blood circulation near the surface of the skin. The rash makes the skin look “mottled and purplish” in what the Mayo Clinic describes as a “net-like pattern”. This underlying condition can be triggered both by a viral infection and cold temperatures.


“When affected people's blood is exposed to cold temperatures (32 to 50ºF), certain proteins that normally attack bacteria (IgM antibodies) attach themselves to red blood cells and bind them together into clumps (agglutination),” according to the Genetic and Rare Diseases Information Center. “This eventually causes red blood cells to be prematurely destroyed (hemolysis) leading to anemia and other associated signs and symptoms.”

The woman's "severe exacerbation based on profound anemia" was treated with "multiple transfusions of warm blood." Konika Sharma/NEJM

The woman also had a condition known as autoimmune hemolytic anemia, a rare blood disorder resulting from the immune system attacking red blood cells within the body. Symptoms generally include vomiting or diarrhea, dizziness, headaches, cold hands and feet, jaundice, chest pain, among others. The medical world is not sure what causes it, but the condition has been linked to autoimmune diseases, cancer, and infections from bacteria, viruses, and parasites.

"It is called 'cold' because when patients are exposed to cold environments, symptoms get triggered that cause red cell agglutination (the clumping of red cells that hampers their flee-flowing abilities)," said treating doctor Konika Sharma in an interview with IFLScience. "As this disease affects only 15 percent of all patients with autoimmune hemolytic anemia, which is 1 case per million people per year, it is considered a rare disease."

Non-pharmacological measures are the preferred mode of therapy. This includes keeping the patient warm, minimizing cold exposure, wearing warm clothes, or moving to warmer areas, said Sharma. She warns that clinical trials have not shown evidence to prove the efficacy of these measures. The woman was experiencing "a severe exacerbation based on profound anemia" and was treated with "multiple transfusions of warm blood."


“The patient was warmed and treated with blood transfusions and rituximab for 1 week,” wrote the doctors in the New England Journal of Medicine. When she was released from the hospital, she was no longer as dizzy but her rash was still present.

If left untreated, anemia can lead to a lack of oxygen to the tissues, which can trigger cell death that may prove fatal if it affects the organs. 


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