Neutrophilic Dermatoses: a Clinical Update

Curr Dermatol Rep. 2022;11(2):89-102. doi: 10.1007/s13671-022-00355-8. Epub 2022 Mar 16.

Abstract

Purpose of review: Neutrophilic dermatoses are defined by the presence of a sterile neutrophilic infiltrate on histopathology. This review focuses on the pathogenesis, epidemiology, clinicopathological features, diagnosis, and management of four disorders: Sweet syndrome, pyoderma gangrenosum, Behçet syndrome, and neutrophilic eccrine hidradenitis.

Recent findings: Recent studies have provided insight into the complex pathogenesis of neutrophilic dermatoses. Evidence supports an intricate interplay of abnormal neutrophil function and inflammasome activation, malignant transformation into dermal infiltrating neutrophils, and genetic predisposition.

Summary: Neutrophilic dermatoses have diverse cutaneous and extracutaneous manifestations and may be associated with significant morbidity and mortality. Common underlying associations include infectious, inflammatory, and neoplastic disorders, as well as drug reactions. Emerging diagnostic and therapeutic frameworks identify an expanding role for biologic and targeted anti-inflammatory therapies.

Keywords: Acute febrile neutrophilic dermatosis; Behçet disease; Neutrophilic dermatosis; Neutrophilic eccrine hidradenitis; Pyoderma gangrenosum; Sweet syndrome.

Publication types

  • Review