New Findings in PiZZ alpha(1)-Antitrypsin Deficiency-Related Panniculitis Demonstration of Skin Polymers and High Dosing Requirements of Intravenous Augmentation Therapy
(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)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/1426382
- author
- Gross, B. ; Grebe, M. ; Wencker, M. ; Stoller, J. K. ; Bjursten, L. M. and Janciauskiene, Sabina LU
- organization
- publishing date
- 2009
- 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
- 2016-04-01 12:11:57
- date last changed
- 2022-03-28 21:34:57
@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}}, keywords = {{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}}, doi = {{10.1159/000202982}}, volume = {{218}}, year = {{2009}}, }