1.
Br J Dermatol
; 147(6): 1258-61, 2002 Dec.
Artigo
em Inglês
| MEDLINE
| ID: mdl-12452881
RESUMO
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.