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

Ann Intern Med. 1977 Jun;86(6):742-4. doi: 10.7326/0003-4819-86-6-742.


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.

Publication types

  • Case Reports
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Blood Protein Disorders / complications
  • Humans
  • Male
  • Pancreatitis / complications
  • Panniculitis, Nodular Nonsuppurative / complications*
  • Phenotype
  • alpha 1-Antitrypsin Deficiency*