Severe panniculitis caused by homozygous ZZ alpha1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin).

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 · Br J Dermatol · 2002

case_report · Level V

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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.

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