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New Findings in PiZZ alpha(1)-Antitrypsin Deficiency-Related Panniculitis Demonstration of Skin Polymers and High Dosing Requirements of Intravenous Augmentation Therapy

Gross, B.; Grebe, M.; Wencker, M.; Stoller, J. K.; Bjursten, L. M. and Janciauskiene, Sabina LU (2009) In Dermatology 218(4). p.370-375
Abstract
Panniculitis is a recognized but unusual complication of a severe deficiency of alpha(1)-antitrypsin (AAT), with fewer than 100 cases described to date. Like the pathogenesis of emphysema in severe PiZZ deficiency of AAT, panniculitis has been hypothesized to be an inflammatory process, possibly related to Z AAT polymer formation and to an unopposed anti-inflammatory screen in the context of deficient serum levels of AAT. The current report presents a 31-year-old woman with PiZZ AAT deficiency-associated panniculitis. Our case extends current knowledge of AAT-associated panniculitis in 2 ways: (1) we demonstrate Z-type AAT polymers in the skin, which supports the inflammatory pathogenesis of panniculitis and the potential pro-inflammatory... (More)
Panniculitis is a recognized but unusual complication of a severe deficiency of alpha(1)-antitrypsin (AAT), with fewer than 100 cases described to date. Like the pathogenesis of emphysema in severe PiZZ deficiency of AAT, panniculitis has been hypothesized to be an inflammatory process, possibly related to Z AAT polymer formation and to an unopposed anti-inflammatory screen in the context of deficient serum levels of AAT. The current report presents a 31-year-old woman with PiZZ AAT deficiency-associated panniculitis. Our case extends current knowledge of AAT-associated panniculitis in 2 ways: (1) we demonstrate Z-type AAT polymers in the skin, which supports the inflammatory pathogenesis of panniculitis and the potential pro-inflammatory role of polymers; (2) we show that a high dose and long- term use of intravenous augmentation therapy (90 mg/kg body weight once weekly during 3 years) can ameliorate the frequency and severity of panniculitis associated with AAT deficiency. Copyright (C) 2009 S. Karger AG, Basel (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Augmentation therapy, Inflammation, Panniculitis, alpha(1)-Antitrypsin, Polymers
in
Dermatology
volume
218
issue
4
pages
370 - 375
publisher
Karger
external identifiers
  • wos:000265799000014
  • scopus:67651091349
ISSN
1421-9832
DOI
10.1159/000202982
language
English
LU publication?
yes
id
3bf574cc-1382-4b41-8159-a19a3ffcbe31 (old id 1426382)
date added to LUP
2009-06-29 16:09:15
date last changed
2017-11-12 03:27:01
@article{3bf574cc-1382-4b41-8159-a19a3ffcbe31,
  abstract     = {Panniculitis is a recognized but unusual complication of a severe deficiency of alpha(1)-antitrypsin (AAT), with fewer than 100 cases described to date. Like the pathogenesis of emphysema in severe PiZZ deficiency of AAT, panniculitis has been hypothesized to be an inflammatory process, possibly related to Z AAT polymer formation and to an unopposed anti-inflammatory screen in the context of deficient serum levels of AAT. The current report presents a 31-year-old woman with PiZZ AAT deficiency-associated panniculitis. Our case extends current knowledge of AAT-associated panniculitis in 2 ways: (1) we demonstrate Z-type AAT polymers in the skin, which supports the inflammatory pathogenesis of panniculitis and the potential pro-inflammatory role of polymers; (2) we show that a high dose and long- term use of intravenous augmentation therapy (90 mg/kg body weight once weekly during 3 years) can ameliorate the frequency and severity of panniculitis associated with AAT deficiency. Copyright (C) 2009 S. Karger AG, Basel},
  author       = {Gross, B. and Grebe, M. and Wencker, M. and Stoller, J. K. and Bjursten, L. M. and Janciauskiene, Sabina},
  issn         = {1421-9832},
  keyword      = {Augmentation therapy,Inflammation,Panniculitis,alpha(1)-Antitrypsin,Polymers},
  language     = {eng},
  number       = {4},
  pages        = {370--375},
  publisher    = {Karger},
  series       = {Dermatology},
  title        = {New Findings in PiZZ alpha(1)-Antitrypsin Deficiency-Related Panniculitis Demonstration of Skin Polymers and High Dosing Requirements of Intravenous Augmentation Therapy},
  url          = {http://dx.doi.org/10.1159/000202982},
  volume       = {218},
  year         = {2009},
}