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Abstract
<p class="first" id="d2480928e87">Pyoderma gangrenosum, a dramatic ulcerative skin
disease, and Sweet syndrome, a papular
dermatosis, were described independently. It was subsequently shown that they share
many characteristics, including clinical overlap and the frequent association with
multisystemic disorders. The group of the neutrophilic dermatoses encompasses these
two dermatoses, as well as other conditions having in common an aseptic neutrophilic
infiltrate predominating in the epidermis and/or the dermis and/or the subcutis. Some
patients also experience neutrophilic infiltrates in other organs, defining the neutrophilic
disease. Recent research suggests that the neutrophilic dermatoses could be considered
as the cutaneous expression of the autoinflammation, an aberrant hyperproduction of
interleukin-1. Autoinflammation is responsible for monogenic diseases, and is also
involved in the mechanism of many polygenic conditions, including the neutrophilic
dermatoses.
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