title: Severe panniculitis caused by homozygous ZZ alpha1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin).
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.
authors.affiliation: Department of Dermatology, University Hospital of Wales, Cardiff CF 14 4XW, Wales, UK.
authors.forename: M M U
authors.initials: MM
authors.lastname: Chowdhury
authors.forename: E J
authors.initials: EJ
authors.lastname: Williams
authors.forename: J S
authors.initials: JS
authors.lastname: Morris
authors.forename: B J M
authors.initials: BJ
authors.lastname: Ferguson
authors.forename: A D
authors.initials: AD
authors.lastname: McGregor
authors.forename: A R
authors.initials: AR
authors.lastname: Hedges
authors.forename: J D
authors.initials: JD
authors.lastname: Stamatakis
authors.forename: F M
authors.initials: FM
authors.lastname: Pope
mesh_terms: D000328:Adult; D005260:Female; D006720:Homozygote; D006801:Humans; D015434:Panniculitis / Q000188:drug therapy / Q000209:etiology*; D010375:Pedigree; D000515:alpha 1-Antitrypsin / Q000627:therapeutic use*; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications* / Q000188:drug therapy / Q000235:genetics
chemical_list: D000515:alpha 1-Antitrypsin