Extensive cutaneous necrosis in a 12-year-old girl: an unusual complication of secondary cold agglutinin syndrome

Abstract

Cold agglutinin syndrome (CAS) secondary to infection is rare, usually presenting with anaemia and minor skin changes. A 12-year-old girl with secondary CAS associated with extensive cutaneous necrosis is reported. She presented with fever and multiple necrotic lesions over both cheeks, the tip of nose, ear margins, hands and buttocks, along with pallor, hepatospenomegaly, acrocyanosis and gangrene of the fifth digit of the right hand. She had anaemia, unconjugated hyperbilirubinaemia and a positive direct antiglobulin test owing to cold agglutinins of the IgM type with anti-i specificity and titres of 1:512 at 4°C. Results of bone marrow examination were normal and cryoglobulins were negative. Cold antibodies released even during a brief, self-limited febrile illness can cause widespread cutaneous gangrene. We believe this is the first report in the paediatric age group.