Severe panniculitis caused by homozygous ZZ alpha1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin)

Br J Dermatol. 2002 Dec;147(6):1258-61. doi: 10.1046/j.1365-2133.2002.05095.x.

Abstract

Severe panniculitis caused by alpha1-antitrypsin deficiency is very rare even though the ZZ phenotype occurs in 1 : 3500 of the population of northern Europe. We describe a 33-year-old woman with rapidly progressing panniculitis and extensive skin necrosis with multiple life-threatening complications. Initial treatment followed by maintenance therapy with human purified enzyme (Prolastin, Bayer, Bridgend, U.K.) has been life-saving.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Homozygote*
  • Humans
  • Panniculitis / drug therapy
  • Panniculitis / etiology*
  • Pedigree
  • alpha 1-Antitrypsin / therapeutic use*
  • alpha 1-Antitrypsin Deficiency / complications*
  • alpha 1-Antitrypsin Deficiency / drug therapy
  • alpha 1-Antitrypsin Deficiency / genetics

Substances

  • alpha 1-Antitrypsin