HERBERT M. RUBINSTEIN, M.D., F.A.C.P.; ADRIAN M. JAFFER, M.D.; JAMES C. KUDRNA, M.D.; Y. LERTRATANAKUL, M.D.; A. J. CHANDRASEKHAR, M.D.; D. SLATER, M.D.; FRANK R. SCHMID, M.D., F.A.C.P.
Two patients with profound decrease of alpha1-antitrypsin (PiZZ) presented with severe panniculitis (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.
RUBINSTEIN HM, JAFFER AM, KUDRNA JC, et al. Alpha1-Antitrypsin Deficiency with Severe Panniculitis: Report of Two Cases. Ann Intern Med. 1977;86:742–744. doi: https://doi.org/10.7326/0003-4819-86-6-742
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Published: Ann Intern Med. 1977;86(6):742-744.
Gastroenterology/Hepatology, Pancreatic Disease, Rheumatology.
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