Academic Journal
New Findings in PiZZ α 1 -Antitrypsin Deficiency-Related Panniculitis
| العنوان: | New Findings in PiZZ α 1 -Antitrypsin Deficiency-Related Panniculitis |
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| المؤلفون: | Gross, B., Grebe, M., Wencker, M., Stoller, J.K., Bjursten, L.M., Janciauskiene, S. |
| المصدر: | Dermatology ; volume 218, issue 4, page 370-375 ; ISSN 1018-8665 1421-9832 |
| بيانات النشر: | S. Karger AG |
| سنة النشر: | 2009 |
| الوصف: | Panniculitis is a recognized but unusual complication of a severe deficiency of α 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. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| DOI: | 10.1159/000202982 |
| الاتاحة: | http://dx.doi.org/10.1159/000202982 https://www.karger.com/Article/Pdf/202982 |
| Rights: | https://www.karger.com/Services/SiteLicenses ; https://www.karger.com/Services/SiteLicenses |
| رقم الانضمام: | edsbas.AFC8C046 |
| قاعدة البيانات: | BASE |
| DOI: | 10.1159/000202982 |
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