Alpha1-antitrypsin deficiency-associated panniculitis: case report and review of treatment options

J Eur Acad Dermatol Venereol. 2005 Jul;19(4):487-90. doi: 10.1111/j.1468-3083.2005.01194.x.

Abstract

Alpha(1)-antitrypsin deficiency, a relatively frequent mutation in the population, is associated with the development of panlobular emphysema and liver cirrhosis. The deficiency is in rare cases associated with the development of panniculitis, and very differentiated clinical courses have been reported in the literature. We report a case of panniculitis in a patient with alpha(1)-antitrypsin deficiency and describe briefly the pathophysiology of the disease and current treatment possibilities.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Administration, Oral
  • Anti-Inflammatory Agents, Non-Steroidal / administration & dosage
  • Dapsone / administration & dosage
  • Diagnosis, Differential
  • Female
  • Humans
  • Infusions, Intravenous
  • Middle Aged
  • Panniculitis / etiology*
  • Panniculitis / pathology
  • Trypsin Inhibitors / administration & dosage
  • alpha 1-Antitrypsin / administration & dosage
  • alpha 1-Antitrypsin Deficiency / complications
  • alpha 1-Antitrypsin Deficiency / diagnosis*
  • alpha 1-Antitrypsin Deficiency / drug therapy

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Trypsin Inhibitors
  • alpha 1-Antitrypsin
  • Dapsone