Literature DB >> 12452881

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

M M U Chowdhury1, E J Williams, J S Morris, B J M Ferguson, A D McGregor, A R Hedges, J D Stamatakis, F M Pope.   

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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Year:  2002        PMID: 12452881     DOI: 10.1046/j.1365-2133.2002.05095.x

Source DB:  PubMed          Journal:  Br J Dermatol        ISSN: 0007-0963            Impact factor:   9.302


  5 in total

1.  Severe postoperative wound healing disturbance in a patient with alpha-1-antitrypsin deficiency: the impact of augmentation therapy.

Authors:  Marionna Cathomas; Alexandra Schüller; Daniel Candinas; Roman Inglin
Journal:  Int Wound J       Date:  2015-03-26       Impact factor: 3.315

Review 2.  Efficacy of alpha1-antitrypsin augmentation therapy in conditions other than pulmonary emphysema.

Authors:  Ignacio Blanco; Beatriz Lara; Frederick de Serres
Journal:  Orphanet J Rare Dis       Date:  2011-04-12       Impact factor: 4.123

3.  New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy.

Authors:  B Gross; M Grebe; M Wencker; J K Stoller; L M Bjursten; S Janciauskiene
Journal:  Dermatology       Date:  2009-02-16       Impact factor: 5.366

Review 4.  Alpha 1-antitrypsin deficiency. 3: Clinical manifestations and natural history.

Authors:  M Needham; R A Stockley
Journal:  Thorax       Date:  2004-05       Impact factor: 9.139

5.  Alpha1-antitrypsin deficiency: a clinical-genetic overview.

Authors:  Raja T Abboud; Tanya N Nelson; Benjamin Jung; Andre Mattman
Journal:  Appl Clin Genet       Date:  2011-03-31
  5 in total

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