[Neutrophilic and pustular dermatoses : New autoinflammatory diseases and syndromes]

This article reviews noninfectious inflammatory dermatoses with mainly neutrophilic infiltrates and the formation of pustules. The infiltrate containing neutrophils may either be mild as in urticaria or very dense, even with leukocytoclasia, as in Sweet syndrome or pyoderma gangrenosum. Neutrophilic infiltrates and pustular eruptions are caused by different noninfectious immunomechanisms. For some of them, mutations have been found (e.g. NLRC4 mutation in cryopyrin-associated periodic syndromes (CAPS) leading to activation of the inflammasome; IL36RN mutation in pustular psoriasis resulting in uncontrolled IL36 signaling). Neutrophilic dermatoses are of high interest, as they may be the cause of underlying benign or malignant conditions. In recent years, efficient targeted therapies have been developed.