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

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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

Detaljer

Författare
Forskningsområden

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Dermatologi och venereologi

Nyckelord

Originalspråkengelska
Sidor (från-till)370-375
TidskriftDermatology
Volym218
Utgivningsnummer4
StatusPublished - 2009
PublikationskategoriForskning
Peer review utfördJa