Neutrophilic dermatoses as systemic diseases.
Neutrophilic dermatoses (ND) are inflammatory skin conditions characterized by a sterile infiltrate of normal polymorphonuclear leukocytes. The main clinical forms of ND include Sweet syndrome, pyoderma gangrenosum, erythema elevatum diutinum, subcorneal pustular dermatosis, and their atypical or transitional forms. ND are often idiopathic, but they may be associated with myeloid hematologic malignancies (Sweet syndrome), inflammatory bowel disease or rheumatoid arthritis (pyoderma gangrenosum), and monoclonal gammopathies (erythema elevatum diutinum, subcorneal pustular dermatosis). The possible infiltration of internal organs with neutrophils during the setting of ND underlies the concept of a neutrophilic systemic disease. ND may be seen as a polygenic autoinflammatory syndrome due to their frequent association with other autoinflammatory disorders (monogenic or polygenic) and the recent published efficacy of interleukin-1 blocking therapies in their management.
Université Paris Diderot, Sorbonne Paris Cité; AP-HP, Paris, France; Service de Dermatologie et Service d'Anatomo-Pathologie, Hôpital Saint Louis, 1 Avenue Claude Vellefaux, 75010 Paris, France.
Lola
L
Prat
Université Paris Diderot, Sorbonne Paris Cité; AP-HP, Paris, France; Service de Dermatologie et Service d'Anatomo-Pathologie, Hôpital Saint Louis, 1 Avenue Claude Vellefaux, 75010 Paris, France. Electronic address: jean-david.bouaziz@sls.aphp.fr.
Jean-David
JD
Bouaziz
Université Paris Diderot, Sorbonne Paris Cité; AP-HP, Paris, France; Service de Dermatologie et Service d'Anatomo-Pathologie, Hôpital Saint Louis, 1 Avenue Claude Vellefaux, 75010 Paris, France.
Daniel
D
Wallach
Université Paris Diderot, Sorbonne Paris Cité; AP-HP, Paris, France; Service de Dermatologie et Service d'Anatomo-Pathologie, Hôpital Saint Louis, 1 Avenue Claude Vellefaux, 75010 Paris, France.
Marie-Dominique
MD
Vignon-Pennamen
Université Paris Diderot, Sorbonne Paris Cité; AP-HP, Paris, France; Service de Dermatologie et Service d'Anatomo-Pathologie, Hôpital Saint Louis, 1 Avenue Claude Vellefaux, 75010 Paris, France.
Martine
M
Bagot
D006402:Hematologic Diseases / Q000150:complications; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000150:complications; D008171:Lung Diseases / Q000150:complications; D009140:Musculoskeletal Diseases / Q000150:complications*; D009504:Neutrophils; D017511:Pyoderma Gangrenosum / Q000150:complications* / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000473:pathology; D016463:Sweet Syndrome / Q000150:complications* / Q000473:pathology; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications / Q000473:pathology