Neutrophil-mediated skin diseases: immunology and genetics


Neutrophil-mediated skin diseases encompass a wide spectrum of diseases characterized by polymorphic cutaneous manifestations. They are histopathologically hallmarked by sterile neutrophilic infiltrates involving the skin and, more rarely, the internal organs. Neutrophil-mediated skin diseases share clinicopathological features with those of autoinflammatory diseases, which present in patients with recurrent episodes of aseptic inflammation in the affected organs in absence of allergy or frank autoimmunity. In classic autoinflammatory diseases, mutations in genes regulating innate immune responses leads to altered activation of the inflammasome. The inflammasome is a molecular platform responsible for triggering autoinflammatory processes via the uncontrolled release of interleukin (IL)-1β, as well as other proinflammatory cytokines and chemokines, leading to neutrophil recruitment into the affected organs.

Increasing evidence indicates that pro-inflammatory cytokines such as IL-1β, in synergy with IL-17 and tumour necrosis factor (TNF)-alpha, play a pivotal role in the pathophysiology of neutrophil-mediated skin diseases. Moreover, mutations in autoinflammatory genes, like Proline-Serine-Threonine Phosphatase Interacting Protein 1 (PSTPIP1), have been demonstrated in neutrophil-mediated skin diseases such as pyoderma gangrenosum and its syndromic presentation known as PASH (pyoderma gangrenosum, acne and suppurative hidradenitis), giving rise to a spectrum of polygenic autoinflammatory conditions.

The aim of this Research Topic is to gather a collection of basic Review articles and Original Research articles on neutrophil-mediated skin diseases in order to (i) provide an update on their genetic profile; (ii) promote improved understanding of the pathophysiological basis of these diseases; (iii) define new biomarkers predicting clinical outcome and response to treatment, and (iv) pave the way to a pathogenesis-driven therapeutic approach. In this Research Topic, we welcome the submission of Reviews, Mini-Reviews and Original Research articles that cover the following topics on neutrophil-mediated skin diseases:

1. Genetics.

2. Role of immune cells and cytokines.

3. Autoinflammation and neutrophil-mediated skin diseases.

4. New biomarkers.

5. Pyoderma gangrenosum and its syndromic forms.

6. Sweet’s syndrome.

7. Hidradenitis suppurativa.

8. Amicrobial pustulosis of the folds (APF).

9. Neutrophilic urticarial dermatosis.

10. Psoriasis vulgaris and pustular psoriasis.

11. Behçet syndrome.

12. New therapeutic approaches.

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Media Contact:

Kathy Andrews
Journal Manager
Journal of Clinical & Experimental Dermatology Research