An Unusual Neutrophilic Dermatosis

Abstract

The neutrophilic dermatoses are a heterogeneous group of cutaneous disorders unified by common histologic findings of a noninfectious, extensive dermal neutrophilic inflammatory infiltrate. Systemic manifestations are common, often with overlapping clinical features among this group of inflammatory dermatoses. This diverse group of diseases has historically been subclassified based upon the presence or absence of vasculitis. This has been a controversial point as entities originally characterized as lacking vascular damage have been shown to occasionally display evidence of vascular injury. Whether the injury to vessels is the primary pathogenetic mechanism, as in immune complex-mediated injury, or a secondary phenomenon, is often difficult to determine on histopathologic grounds alone. Entities to be discussed are inflammatory dermatoses characterized by a dense dermal neutrophilic infiltrate, including Sweet syndrome, pyoderma gangrenosum, rheumatoid neutrophilic dermatitis, bowel associated dermatitis-arthritis syndrome, Behçet disease, erythema elevatum diutinum (EED) and granuloma faciale (GF).