Severe panniculitis caused by homozygous ZZ alpha1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin).
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.
Chowdhury
M M U
MM
Department of Dermatology, University Hospital of Wales, Cardiff CF 14 4XW, Wales, UK.
Williams
E J
EJ
Morris
J S
JS
Ferguson
B J M
BJ
McGregor
A D
AD
Hedges
A R
AR
Stamatakis
J D
JD
Pope
F M
FM
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
D000515:alpha 1-Antitrypsin