What immune deficiency is indicated by ice cube negative cold urticaria?

Ice cube negative cold urticaria is associated with PLAID syndrome (PLAutoimmune disease with Immunodeficiency). This syndrome is characterized by a defect in the ability to regulate immune responses, leading to both autoimmune manifestations and immunodeficiency. In this condition, exposure to cold can trigger urticaria (hives) without the presence of an ice cube (hence "ice cube negative"), indicating a specific hypersensitivity reaction to cold stimuli rather than a typical allergic response.

The connection between PLAID syndrome and cold urticaria stems from the underlying immune dysregulation seen in affected individuals. They may exhibit skin responses to cold exposure due to an abnormality in their immune system, which fails to properly manage these triggers, leading to more severe reactions.

Understanding this association is crucial for recognizing symptoms in patients with PLAID syndrome, as cold urticaria can serve as a clinical clue towards diagnosing the underlying condition. In contrast, other options like IgA deficiency, Common Variable Immunodeficiency, and Severe Combined Immunodeficiency do not typically present with cold-induced urticaria as a defining characteristic, making PLAID syndrome the more appropriate association in this scenario.