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“Autoinflammatory psoriasis”—genetics and biology of pustular psoriasis

Cellular & Molecular Immunology volume 18, pages 307–317 (2021)Cite this article

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Abstract

Psoriasis is a chronic inflammatory skin condition that has a fairly wide range of clinical presentations. Plaque psoriasis, which is the most common manifestation of psoriasis, is located on one end of the spectrum, dominated by adaptive immune responses, whereas the rarer pustular psoriasis lies on the opposite end, dominated by innate and autoinflammatory immune responses. In recent years, genetic studies have identified six genetic variants that predispose to pustular psoriasis, and these have highlighted the role of IL-36 cytokines as central to pustular psoriasis pathogenesis. In this review, we discuss the presentation and clinical subtypes of pustular psoriasis, contribution of genetic predisposing variants, critical role of the IL-36 family of cytokines in disease pathophysiology, and treatment perspectives for pustular psoriasis. We further outline the application of appropriate mouse models for the study of pustular psoriasis and address the outstanding questions and issues related to our understanding of the mechanisms involved in pustular psoriasis.

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Acknowledgements

This work was supported by the Babcock Endowment Fund (L.C.T. and J.E.G.), the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health under award numbers R01-AR060802 (J.E.G.), P30-AR075043 (J.E.G.), and K01-AR072129 (L.C.T.), and the National Institute of Allergy and Infectious Diseases under award number R01-AR069071 (J.E.G.), the A. Alfred Taubman Medical Research Institute (J.E.G. and J.M.K.), the National Psoriasis Foundation (J.E.G, N.L.W., J.M.K., E.M., and L.C.T.), and the Parfait Emerging Scholar Award (J.M.K.). L.C.T. is supported by the Dermatology Foundation, the Arthritis National Research Foundation, and the National Psoriasis Foundation.

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Authors and Affiliations

  1. Graduate Program in Immunology, University of Michigan, Ann Arbor, MI, USA

    Ranjitha Uppala

  2. Department of Dermatology, University of Michigan, Ann Arbor, MI, USA

    Ranjitha Uppala, Lam C. Tsoi, Paul W. Harms, Bo Wang, Allison C. Billi & Johann E. Gudjonsson

  3. Department of Pathology, University of Michigan, Ann Arbor, MI, USA

    Paul W. Harms

  4. Department of Dermatology, University of California, Davis, Sacramento, CA, USA

    Emanual Maverakis

  5. Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA

    J. Michelle Kahlenberg

  6. A. Alfred Taubman Medical Research Institute, Ann Arbor, MI, USA

    J. Michelle Kahlenberg & Johann E. Gudjonsson

  7. Departments of Nutrition and Dermatology, Case Western Reserve University, Cleveland, OH, USA

    Nicole L. Ward

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  1. Ranjitha Uppala
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Uppala, R., Tsoi, L.C., Harms, P.W. et al. “Autoinflammatory psoriasis”—genetics and biology of pustular psoriasis. Cell Mol Immunol 18, 307–317 (2021). https://doi.org/10.1038/s41423-020-0519-3

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