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Case Reports
. 1977 Jun;86(6):742-4.
doi: 10.7326/0003-4819-86-6-742.

Alpha1-antitrypsin deficiency with severe panniculitis. Report of two cases

H M Rubinstein, A M Jaffer, J C Kudrna, Y Lertratanakul, A J Chandrasekhar, D Slater, F R Schmid
Case Reports

Alpha1-antitrypsin deficiency with severe panniculitis. Report of two cases

H M Rubinstein et al. Ann Intern Med. 1977 Jun.

Abstract

Two patients with profound decrease of alpha1-antitrypsin (PiZZ) presented with severe pannicultis (Weber-Christian disease); one 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.

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