α1-Antitrypsin deficiency, a relatively frequent mutation in the population, is associated with the development of panlobular emphysema and liver cirrhosis. The deficiency is in rare cases associated with the development of panniculitis , and very differentiated clinical courses have been reported in the literature. We report a case of panniculitis in a patient with a1-antitrypsin deficiency a nd describe briefly the pathophysiology of the disease and current treatment pos sibilities.
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