Neutrophilic dermatosis: disease mechanism and treatment.
OBJECTIVE
The purpose of this review is to describe the physiopathological and therapeutic aspects of neutrophilic dermatosis, taking into account their most frequent associated conditions.


RESULTS
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.


CONCLUSIONS
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.
Department of Dermatology and Pathology, Université Paris Diderot, Sorbonne Paris Cité, Saint Louis Hospital, Paris, France.
Diane
D
Maalouf
Maxime
M
Battistella
Jean-David
JD
Bouaziz
D000818:Animals; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D019337:Hematologic Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D007375:Interleukin-1 / Q000378:metabolism; D009101:Multiple Myeloma / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology; D010265:Paraproteinemias / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology; D017511:Pyoderma Gangrenosum / Q000188:drug therapy / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D016463:Sweet Syndrome / Q000188:drug therapy / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors / Q000378:metabolism
D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein; D007375:Interleukin-1; D014409:Tumor Necrosis Factor-alpha