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

B. Gross, M. Grebe, M. Wencker, J. K. Stoller, L. M. Bjursten, Sabina Janciauskiene

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    55 Citations (SciVal)

    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
    Original languageEnglish
    Pages (from-to)370-375
    JournalDermatology
    Volume218
    Issue number4
    DOIs
    Publication statusPublished - 2009

    Subject classification (UKÄ)

    • Dermatology and Venereal Diseases

    Keywords

    • Augmentation therapy
    • Inflammation
    • Panniculitis
    • alpha(1)-Antitrypsin
    • Polymers

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