Pustular Forms of Psoriasis Related to Autoinflammation

  • Satveer K. Mahil
  • Jonathan N. Barker
  • Francesca CaponEmail author


Psoriasis is a common inflammatory skin disorder that is classified into multiple disease subtypes. The pustular variants, which are characterized by neutrophilic infiltrates, present with acute, potentially-life threatening episodes of pustulation and systemic upset (generalised pustular psoriasis, GPP) or with chronic and disabling pustulation of the hands and feet (palmar plantar pustulosis, PPP and acrodermatitis continua of Hallopeau, ACH). These are very severe conditions that are extremely difficult to treat.

In the last few years, genetic studies have identified three disease genes (IL36RN, AP1S3 and CARD14) that are mutated in one or more forms of pustular psoriasis. This has demonstrated a shared genetic basis for these conditions and highlighted an autoinflammatory pathogenesis, driven by abnormal IL-36 signalling. In this context, the ongoing development of IL-36 antagonists holds the promise of delivering efficient therapeutics for a group of diseases that have a profound impact on quality of life.


Pustular psoriasis Interleukin-36 IL-36 IL36RN AP1S3 CARD14 Skin autoinflammation DITRA 



Acrodermatitis continua of Hallopeau


CARD14 mediated psoriasis


Deficiency of the interleukin 36 receptor antagonist


Generalised pustular psoriasis


Interleukin-36 receptor antagonist


Palmar plantar pustulosis.


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Satveer K. Mahil
    • 1
    • 2
    • 3
  • Jonathan N. Barker
    • 1
    • 2
    • 3
  • Francesca Capon
    • 1
    Email author
  1. 1.Division of Genetics and Molecular MedicineKing’s College LondonLondonUK
  2. 2.St John’s Institute of Dermatology, Guy’s and St Thomas’ HospitalLondonUK
  3. 3.Guy’s HospitalLondonUK

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