Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)
Identifieur interne : 002205 ( Main/Merge ); précédent : 002204; suivant : 002206Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)
Auteurs : M. M. U. Chowdhury [Royaume-Uni] ; E. J. Williams ; J. S. Morris ; B. J. M. Ferguson ; A. D. Mcgregor [Royaume-Uni] ; A. R. Hedges ; J. D. Stamatakis ; F. M. Pope [Royaume-Uni]Source :
- British Journal of Dermatology [ 0007-0963 ] ; 2002-12.
English descriptors
- Teeft :
- A1at, Antitrypsin, Antitrypsin deficiency, Bayer, Bayer corporation, Bridgend, British association, British journal, Case report, Dermatologist, Dermatology, Functional activity, Human enzyme, Liver disease, Liver transplantation, Maintenance therapy, Panniculitis, Phenotype, Prolastin, Rapid progression, Septicaemia, Severe panniculitis, Skin necrosis, Trunk distribution, Upper thighs.
Abstract
Summary Severe panniculitis caused by α1‐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.
Url:
DOI: 10.1046/j.1365-2133.2002.05095.x
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<front><div type="abstract" xml:lang="en">Summary Severe panniculitis caused by α1‐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.</div>
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