Selective IgM deficiency associated with adult-onset Still disease

Selective immunoglobulin M (IgM) deficiency is a rare immunodeficiency disorder characterized by decreased serum levels of IgM. Those with IgM deficiency may present with recurrent infections and have an increased predisposition to allergic and autoimmune diseases.1 Adult-onset Still disease (AOSD) is a rare autoinflammatory disease characterized by high-spiking fevers, evanescent salmon-colored rash, and multiorgan involvement. It is a diagnosis based on clinical criteria and age of onset.2 A case of selective IgM deficiency associated with AOSD has not been reported previously.

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