An update on generalized pustular psoriasis

Expert Rev Clin Immunol. 2019 Sep;15(9):907-919. doi: 10.1080/1744666X.2019.1648209. Epub 2019 Sep 5.


Introduction: Generalized pustular psoriasis (GPP) is a rare, severe relapsing/remitting, multisystem disease that can be difficult to treat. Recent clinical, histological, and genetic evidence suggests that GPP is a distinct clinical entity from plaque psoriasis and requires a separate diagnosis. The interleukin-36 pathway appears to be central to GPP pathogenesis. As no therapeutic agents have been approved for GPP to date in the United States or Europe, the introduction of anti-IL-36 therapies may change disease management. Areas covered: Using PubMed and Google Scholar, we reviewed the literature for articles related to GPP, psoriasis, and the genetics, pathogenesis, and treatment thereof. Expert opinion: New therapeutic options and updated guidelines for GPP treatment are needed. Ideal agents would have rapid onset of action and rapid time to achieve disease clearance, have the ability to prevent acute flares and avert recurrence, and possess a favorable safety profile. Such therapies should be readily accessible via approval or listing on formularies. Scoring systems to establish GPP disease burden and objective outcome measures could also help with further evaluation of therapies and treatment access issues. IL-36 remains a promising target, as supported by early phase data suggesting efficacy and safety for a novel anti-IL-36 therapy.

Keywords: Biologics; GPP; IL-36; IL-36 receptor antagonist; generalized pustular psoriasis; von Zumbusch.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Dermatologic Agents / therapeutic use*
  • Humans
  • Interleukin-1 / antagonists & inhibitors*
  • Interleukin-1 / metabolism
  • Molecular Targeted Therapy
  • Mutation
  • Psoriasis / diagnosis
  • Psoriasis / drug therapy*
  • Psoriasis / metabolism
  • Receptors, Interleukin / antagonists & inhibitors*
  • Receptors, Interleukin / genetics
  • Receptors, Interleukin / metabolism
  • Signal Transduction / drug effects*
  • Skin / drug effects*
  • Skin / metabolism
  • Skin / pathology


  • Dermatologic Agents
  • Interleukin-1
  • Receptors, Interleukin
  • interleukin 36, human
  • interleukin-36 receptor, human