α1-Antitrypsin deficiency panniculitis (phenotype PiZZ) precipitated postpartum and successfully treated with dapsone

Severe panniculitis caused by homozygous

Severe panniculitis caused by homozygous ZZ α1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)

✍ M.M.U. Chowdhury; E.J. Williams; J.S. Morris; B.J.M. Ferguson; A.D. McGregor; A. 📂 Article 📅 2002 🏛 John Wiley and Sons 🌐 English ⚖ 93 KB

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 complication