Are neutrophilic dermatoses autoinflammatory disorders?

Neutrophils constitute essential players in inflammatory responses and are the first line of defence against harmful stimuli. However, dysregulation of neutrophil homeostasis can result in excessive inflammation and subsequent tissue damage. Neutrophilic dermatoses are a spectrum of inflammatory disorders characterized by skin lesions resulting from a neutrophil-rich inflammatory infiltrate in the absence of infection. The exact molecular pathophysiology of neutrophilic dermatoses has long been poorly understood. Interestingly, neutrophil-rich cutaneous inflammation is also a cardinal feature of several autoinflammatory diseases with skin involvement, the latter being caused by aberrant innate immune responses. Overactivation of the innate immune system leading to increased production of interleukin-1 family members and sterile' neutrophil-rich cutaneous inflammation are features of both inherited autoinflammatory syndromes with skin involvement and an increasing number of neutrophilic dermatoses. Therefore, we propose that autoinflammation may be a cause of neutrophilic dermatoses. What's already known about this topic? Many inherited autoinflammatory diseases have characteristic skin manifestations composed of a neutrophil-rich cutaneous inflammatory infiltrate. Neutrophilic dermatoses have similar clinical skin manifestations to inherited autoinflammatory diseases caused by overproduction of certain cytokines of the interleukin-1 family. What does this study add? Certain unifying features of these disorders suggest that they are a cutaneous consequence of autoinflammation and may be considered as autoinflammatory diseases. Some of these neutrophilic dermatoses may lack IL-1 as a triggering cytokine, but can still benefit from IL-1-targeted therapy.