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Panniculitis can arise from many underlying causes. Potential causes include connective tissue disease such as systemic lupus erythematosus and scleroderma, lymphoproliferative disorders, pancreatic disease, gout, chronic kidney disease, alpha 1-antitrypsin (alpha1 AT) deficiency and adverse reactions to medications. Alpha 1 AT deficiency is one of the most common hereditary disorders of Caucasians and Europeans, and commonly associated with pulmonary and hepatic injury. Panniculitis is an uncommon first manifestation of this deficiency, with fewer than 100 cases reported in the English-language scientific literature.

PReS-FINAL-2256: Panniculitis revealing quantitative alpha 1 antitrypsine deficiency