A 32-year-old woman with hypocomplementemic urticarial vasculitis (HUV) presented for evaluation of ongoing hives, inflammatory arthritis, shortness of breath, and substernal chest pain.History of present illness. At the age of 15 years old, she developed urticarial lesions provoked by the cold and inflammatory arthritis. She was evaluated by rheumatology for pain and swelling of her joints at the age of 29 years old. By this time, the hives were no longer only triggered by the cold. The hives were pruritic but also associated with a burning sensation, lasted for more than 1 day, and resolved with hyperpigmentation. They were steroid responsive. She denied any fevers, angioedema, ocular, renal, gastrointestinal, or pulmonary symptoms at that time, and the only other symptom was pain and swelling of the joints. A skin biopsy of an urticarial lesion was performed and showed moderately dense infiltrate of neutrophils and eosinophils tightly cuffed around vessels of the upper and mid-dermis with leukocytoclasis and perivascular fibrin, consistent with urticarial vasculitis. Immunofluorescence was not performed on the skin biopsy. Serologic evaluation included a negative antinuclear antibody, negative extractable nuclear antigen panel, and negative testing for antiphospholipid antibody syndrome (Table 1). C3 was low at 73 mg/dl (normal 89-193 mg/dl), C4 was 11 mg/dl (normal 15-57 mg/dl), and C1q complement component was undetectable (normal 109-242 μg/ml). Potential etiologies of urticaria were considered, including familial cold autoinflammatory syndrome, but given the absence of fevers, family history, and age at presentation, this was thought to be unlikely. Systemic lupus erythematosus was ruled out based on serologic work-up. Based on the urticaria, skin biopsy, low C3, low C4, low C1q, and inflammatory arthritis, she was diagnosed with HUV. 1