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alpha 1-antitrypsin deficiency-associated panniculitis: resolution with intravenous alpha 1-antitrypsin administration and liver transplantation.

Abstract
Panniculitis is a rare complication of alpha 1-antitrypsin (A1AT) deficiency that is characterized by acute inflammatory infiltrate and fat necrosis. Different treatment strategies are used to provide symptomatic relief. Here we describe two patients with homozygous A1AT deficiency who developed panniculitis and were successfully treated with A1AT replacement. The patient who received a liver transplant experienced complete resolution of the skin lesions. The patient who received A1AT intravenously showed complete response, but the skin lesions recurred when the levels of A1AT fell below 50 mg/100 ml. Panniculitis secondary to A1AT deficiency can be successfully treated with liver transplantation or intravenous infusion of A1AT.
AuthorsK O'Riordan, A Blei, M S Rao, M Abecassis
JournalTransplantation (Transplantation) Vol. 63 Issue 3 Pg. 480-2 (Feb 15 1997) ISSN: 0041-1337 [Print] United States
PMID9039946 (Publication Type: Case Reports, Journal Article)
Chemical References
  • alpha 1-Antitrypsin
Topics
  • Female
  • Humans
  • Infusions, Intravenous
  • Liver Transplantation
  • Male
  • Middle Aged
  • Panniculitis (drug therapy, etiology, surgery)
  • alpha 1-Antitrypsin (administration & dosage, therapeutic use)
  • alpha 1-Antitrypsin Deficiency

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