Premium
Severe panniculitis caused by homozygous ZZ α 1 ‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin ® )
Author(s) -
Chowdhury M.M.U.,
Williams E.J.,
Morris J.S.,
Ferguson B.J.M.,
McGregor A.D.,
Hedges A.R.,
Stamatakis J.D.,
Pope F.M.
Publication year - 2002
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.2002.05095.x
Subject(s) - panniculitis , medicine , alpha 1 antitrypsin deficiency , enzyme , population , phenotype , pathology , immunology , biology , biochemistry , gene , environmental health
Summary Severe panniculitis caused by α 1 ‐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.
Accelerating Research
Robert Robinson Avenue,
Oxford Science Park, Oxford
OX4 4GP, United Kingdom
Address
John Eccles HouseRobert Robinson Avenue,
Oxford Science Park, Oxford
OX4 4GP, United Kingdom