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 develop...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.展开更多
文摘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.