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Generalized pustular psoriasis: Divergence of innate and adaptive immunity

International Journal of Molecular Sciences, ISSN: 1422-0067, Vol: 22, Issue: 16
2021
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Secukinumab Surpasses Ustekinumab in Clearing Skin for Generalized Pustular Psoriasis

New research found secukinumab is superior to ustekinumab in rapidly clearing the skin for patients with generalized pustular psoriasis (GPP).1 “Our study demonstrated that secukinumab

Review Description

Generalized pustular psoriasis (GPP) is a severe, relapsing, immune-mediated disease characterized by the presence of multiple sterile pustules all over the body. The exact pathomecha-nisms behind GPP remain elusive, although increased interest in the genetic basis and immunological disturbances have provided some revealing insights into the underlying signaling pathways and their mutual interaction. The genetic background of GPP has been thoroughly investigated over the past few years. The conducted studies have identified genetic variants that predispose to pus-tular forms of psoriasis. The loss-of-function mutation of the interleukin 36 receptor antagonist gene, along with rare gain-of-function mutations in the gene that encodes the keratinocyte signaling molecule (CARD14), are examples of the uncovered abnormalities. Interleukin 36 (IL-36), along with neutrophils, is now considered a central cytokine in GPP pathogenesis, with IL-36 signaling providing a link between innate and adaptive immune responses. More recently, a new concept of inflam-mation, caused by a predominantly genetically determined abnormal activation of innate immune response and leading to inflammatory keratinization, has arisen. GPP is currently considered a rep-resentative of this novel group of skin conditions, called autoinflammatory keratinization diseases. As no therapeutic agents have been approved for GPP to date in the United States and Europe, the novel anti-IL-36R antibodies are particularly promising and may revolutionize management of the disease.

Bibliographic Details

Samotij, Dominik; Szczęch, Justyna; Reich, Adam

MDPI AG

Chemical Engineering; Biochemistry, Genetics and Molecular Biology; Chemistry; Computer Science

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