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Journal of Clinical Immunology

, Volume 39, Issue 8, pp 832–835 | Cite as

Successful Treatment of PAPA Syndrome with Dual Adalimumab and Tacrolimus Therapy

  • Amika K. SoodEmail author
  • Diana B. McShane
  • Paul B. Googe
  • Eveline Y. Wu
Letter to Editor
  • 144 Downloads

Introduction

Pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome is a rare autosomal dominant disorder with variable expressivity [1] and results from mutations in the PSTPIP1 gene. The PSTPIP1 protein is a cytoskeletal protein within hematopoietic cells that serves as a scaffold for the binding of other cellular proteins, such as pyrin, protein tyrosine phosphatases, c-Abl, CD2, and WASP. Through these interactions, PSTPIP1 regulates several cellular functions including IL-lβ release, cytoskeleton organization, cell migration, and T cell activation [2, 3]. Depending on the mutation location within the PSTPIP1 gene and consequent alterations in protein-protein interactions, a spectrum of autoinflammatory disorders may result and are collectively referred to as PSTPIP1-associated inflammatory diseases (PAID). While the underlying pathogenesis is not completely understood, the PAID spectrum is characterized by dysregulated IL-lβ release and neutrophil responses [4], and...

Keywords

Pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome PSTPIP1-associated inflammatory diseases (PAID) autoinflammatory diseases adalimumab tacrolimus 

Notes

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Pediatric Allergy, Immunology, and RheumatologyUniversity of North Carolina at Chapel HillChapel HillUSA
  2. 2.Department of DermatologyUniversity of North Carolina at Chapel HillChapel HillUSA

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