Skip to main content

Interleukin-1-related cytokines as potential biomarkers in autoinflammatory skin diseases

  • 469 Accesses


Urticarial rash is a hallmark symptom of autoinflammatory diseases such as Cryopyrin-associated periodic syndrome (CAPS) and Schnitzler's syndrome (SchS). Clinically, the urticarial rash may not be distinguished from the skin symptoms in chronic urticaria patients. As interleukin-1ß (IL-1ß) has been shown to play a pivotal role in the pathogenesis of CAPS and SchS we here aim at investigating IL-1ß and related cytokines for their potential as diagnostic skin biomarkers in patients with urticarial autoinflammatory syndromes.

Materials and methods

Immunohistochemical stainings (neutrophil marker myeloperoxidase (MPO), IL-1ß, IL-6, IL-18) from lesional skin of patients with CAPS (n=3), SchS (n=9) and chronic spontaneous urticaria (csU) (n=10) as well as healthy control skin samples (n=10) were analyzed by quantitative histomorphometry and compared with cytokine protein concentrations assessed by ELISA.


Quantitative histomorphometry revealed a higher percentage of neutrophil-dominated dermal cell infiltrate in autoinflammatory diseases that was significant for SchS skin samples as compared with csU samples and healthy controls (p ≤ 0.05). Analysis of IL-1ß, IL-6 and IL-18 positive cells in CAPS and SchS skin showed higher cell numbers which were much less pronounced in csU and healthy control samples. In addition, protein concentrations of all three cytokines were significantly higher in autoinflammatory diseases as compared with csU patients and healthy controls (p ≤ 0.05).


Our study confirms the predominance of neutrophil-dominated cell infiltrates and demonstrates an upregulation of IL-1-related cytokines in the skin of urticarial autoinflammatory diseases. We suggest to further explore these cytokines as diagnostic biomarkers in larger patient samples.

Author information

Correspondence to H Bonnekoh.

Rights and permissions

This article is published under an open access license. Please check the 'Copyright Information' section for details of this license and what re-use is permitted. If your intended use exceeds what is permitted by the license or if you are unable to locate the licence and re-use information, please contact the Rights and Permissions team.

About this article

Verify currency and authenticity via CrossMark

Cite this article

Bonnekoh, H., Maurer, M. & Krause, K. Interleukin-1-related cytokines as potential biomarkers in autoinflammatory skin diseases. Pediatr Rheumatol 13, P197 (2015).

Download citation


  • Urticaria
  • Chronic Urticaria
  • Autoinflammatory Disease
  • Chronic Spontaneous Urticaria
  • Autoinflammatory Syndrome