Alpha1-Antitrypsin Deficiency with Severe Panniculitis

Abstract

Two patients with profound decrease of .alpha.1-antitrypsin (PiZZ) presented with severe panniculitis (Weber-Christian disease); 1 had systemic panniculitis, including pancreatitis. Another possible case is quoted from the literature. Although milder forms of panniculitis can have normal Pi phenotypes and alpha1-antitrypsin levels, the marked reduction of antiproteolytic activity found in PiZZ homozygotes may predispose to or aggravate the lesions of Weber-Christian disease.