Neutrophilic Dermatitis and Associated Conditions

Neutrophilic dermatitis encompasses a spectrum of inflammatory skin disorders characterised by dense dermal infiltrates of mature neutrophils in the absence of primary vasculitis. Prominent among these is acute febrile neutrophilic dermatosis (Sweet syndrome), which presents with tender erythematous papules, plaques or nodules often accompanied by systemic features such as fever and neutrophilia. Other entities include pyoderma gangrenosum, subcutaneous neutrophilic panniculitis and rare necrotising variants that can mimic infectious processes. These conditions may arise idiopathically, be paraneoplastic (particularly in haematological malignancies), drug‐induced or associated with systemic inflammatory diseases. Current understanding emphasises dysregulated innate immunity, aberrant cytokine signalling (notably interleukin‐1β and G‐CSF), inflammasome activation and, in some cases, somatic mutations altering neutrophil function. Diagnosis relies on clinical assessment supported by histopathology to exclude vasculitis or infection. First‐line therapy remains systemic corticosteroids, with increasing use of steroid‐sparing agents and targeted biologics based on molecular stratification. Advances in genomics and immunology are refining disease subtypes and informing personalised management strategies, with implications for global patient populations dealing with auto‐inflammatory and paraneoplastic syndromes.

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Neutrophilic Dermatitis and Associated Conditions publication trend

The graph below shows the total number of articles in neutrophilic dermatitis and associated conditions across all publications each year (not limited to Nature Index journals).

Technical terms

Neutrophilic dermatoses: A group of skin disorders marked by sterile dermal neutrophil infiltrates without primary vasculitis.

Sweet syndrome (acute febrile neutrophilic dermatosis): An inflammatory disorder with sudden onset of tender skin lesions, fever and neutrophilia.

Pyoderma gangrenosum: A neutrophil‐driven ulcerative skin condition often linked to systemic disease.

Inflammasome: A multi‐protein complex that activates inflammatory cytokines such as interleukin-1β.

PI3K/AKT signalling: A key intracellular pathway regulating cell survival and immune cell activation.

Interleukin-1β (IL-1β): A pro‐inflammatory cytokine central to neutrophil recruitment and activation.

References

1. Identification of a neutrophil-specific PIK3R1 mutation facilitates targeted treatment in a patient with Sweet syndrome. Journal of Clinical Investigation (2023).
2. Atypical Sweet syndrome: skin sinus tracts in an acutely febrile patient after lymphoma treatment: a case report. Frontiers in Immunology (2023).
3. Insights Into the Pathogenesis of Sweet's Syndrome. Frontiers in Immunology (2019).
4. Necrotizing neutrophilic dermatosis: A diagnostic challenge with a need for multi-disciplinary recognition, a case report. Annals of Medicine and Surgery (2020).