Purpose of review: The purpose of this review is to describe the physiopathological and therapeutic aspects of neutrophilic dermatosis, taking into account their most frequent associated conditions.
Recent findings: In autoinflammatory syndromes featuring neutrophilic dermatosis, the role of interleukin-1 and tumor necrosis factor (TNF)-α cytokines in the immunopathogenesis of neutrophilic dermatosis has supported their classification as autoinflammatory diseases. In malignancy-associated neutrophilic dermatosis, the role of the malignant clone in myeloid neoplasms and the role of the monoclonal gammopathy and/or of the malignant plasmocyte clone in myeloma have been underlined.
Summary: Recent insights into neutrophilic dermatosis' pathophysiology have encouraged the use of targeted biological therapies for their treatment. Although systemic glucocorticoids remain the mainstay of treatment for Sweet's syndrome and pyoderma gangrenosum, anti-TNF-α is becoming the preferred treatment when pyoderma gangrenosum is accompanied by inflammatory bowel disease or rheumatoid arthritis. Interleukin-1 receptor inhibitor anakinra is a promising therapeutic alternative for refractory Sweet's syndrome.