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IL36RN gene
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Gene

IL36RN gene

The IL36RN gene provides instructions for a protein that regulates skin inflammation, and mutations in this gene are associated with severe pustular skin disorders.

Normal Function

The IL36RN gene provides instructions for making the interleukin 36 receptor antagonist (IL-36Ra) protein. Primarily found in the skin, this protein helps regulate the body's early immune response by controlling inflammation.

Inflammation is typically stimulated when proteins such as IL-36 alpha, IL-36 beta, or IL-36 gamma bind to a specific receptor, activating signaling pathways like NF-κB and MAPK. The IL-36Ra protein acts as a control mechanism by binding to the receptor, thereby blocking these inflammatory proteins from attaching and preventing excessive signaling.

Health Conditions Related to Genetic Changes

Generalized pustular psoriasis

Mutations in the IL36RN gene increase susceptibility to generalized pustular psoriasis (GPP), a serious skin disorder characterized by repeated episodes of red, inflamed skin and pus-filled blisters (pustules). These episodes may be accompanied by fever and systemic inflammation, often triggered by stress, infection, or medication.

Mutations associated with GPP reduce or eliminate the IL-36Ra protein. Without this regulatory protein, the inflammatory signaling pathways become overly active, leading to uncontrolled inflammation in the skin.

Other disorders

IL36RN gene mutations are also linked to other inflammatory skin conditions:

  • Acute generalized exanthematous pustulosis (AGEP): Characterized by a widespread rash and pustules, often triggered by medications.
  • Localized pustular psoriasis: Includes conditions like palmoplantar pustulosis (affecting hands and feet) and acrodermatitis continua of Hallopeau (affecting fingertips and toes).
  • Geographic tongue: A condition where the tongue develops irregular, map-like patches.

Other Names for This Gene

  • FIL1
  • FIL1(DELTA)
  • FIL1D
  • IL-1 related protein 3
  • IL-1F5
  • IL-36Ra
  • IL1F5
  • IL1HY1
  • IL1L1
  • IL1RP3
  • IL36RA
  • interleukin 1 family, member 5 (delta)
  • interleukin-1 HY1
  • interleukin-1 receptor antagonist homolog 1
  • interleukin-1-like protein 1
  • interleukin-36 receptor antagonist protein
  • MGC29840
  • PSORP
  • PSORS14

Frequently Asked Questions

GPP flares are often triggered by specific factors, including stress, infections (especially upper respiratory infections), and certain medications like corticosteroids (especially when rapidly withdrawn), lithium, or some anti-malarial drugs. Avoiding these known triggers is crucial for managing GPP. This includes stress reduction techniques, prompt treatment of infections, and careful consultation with a doctor before starting or stopping any medications.
Mutations in the IL36RN gene are primarily associated with GPP, but they can also increase susceptibility to other inflammatory skin conditions. These include Acute Generalized Exanthematous Pustulosis (AGEP), which is often drug-induced and characterized by widespread pustules, and localized forms of pustular psoriasis like palmoplantar pustulosis (affecting hands and feet) and acrodermatitis continua of Hallopeau (affecting fingertips and toes). The gene's role in regulating inflammation means its dysfunction can manifest in various skin-related inflammatory responses.
Treatment for GPP flares typically involves systemic medications to control inflammation and prevent complications. This may include retinoids (like acitretin), immunosuppressants (like cyclosporine or methotrexate), or biologics that target specific inflammatory pathways (such as IL-36 receptor inhibitors). During acute flares, hospitalization may be necessary for supportive care, fluid management, and intensive skin treatment. Long-term management focuses on preventing flares with ongoing medication and trigger avoidance.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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