Pyoderma gangrenosum and Sweet syndrome: the prototypic neutrophilic dermatoses

Abstract

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.

Inflammasomes and dermatology

“…All monogenic autoinflammatory diseases present cutaneous symptoms, and these coincide with lesions stemming from neutrophilic dermatoses. 52 …”

The diagnosis you wish you had never operated on: Pyoderma gangrenosum misdiagnosed as necrotizing fasciitis—a case report

“…The ulcer often expands due to tissue necrosis and the wound can measure up to 30 cm or more. The lesions can be deep, exposing muscles and tendons (Wallach and Vignon-Pennamen 2015). The disease is commonly seen on the lower extremities but can occur at any location on the body surface.…”

“…The clinical course is commonly quiescent with an ulceration of prolonged duration. Fever and general malaise of variable intensity can occur (Wallach and Vignon-Pennamen 2015). …”

Oral Pyoderma Gangrenosum: Diagnosis, Treatment and Challenges: A Systematic Review

“…SS has a distinct clinical presentation that allows differentiating it from PG. Patients are febrile and present erythematous welldefined and asymmetrical plaques or papules on the skin [46]. Histopathology shows absence of vasculitis, a diffuse perivascular and nodular neutrophilic infiltrate, and various degrees of edema [46,47].…”