Subcorneal Pustular Dermatosis Occuring in Association with Pyoderma Gangrenosum and Rheumatoid Arthritis: A Triple Whammy!

Neutrophilic dermatoses are a wide group of disorders encompassing indolent to severely disabling conditions. A co-existence of two such conditions, pyoderma gangrenosum (PG) and subcorneal pustular dermatosis, necessitates a thorough investigation for IgA dysglobulinemia. We report a middle-aged woman who developed PG following 18 years of (undiagnosed) subcorneal pustular dermatosis, along with rheumatoid arthritis, a known association of PG.

Novel Therapeutic Approaches and Targets for the Treatment of Neutrophilic Dermatoses, Management of Patients with Neutrophilic Dermatoses and Future Directions in the Era of Biologic Treatment.

Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders characterized by the presence of a sterile, predominantly neutrophilic infiltrate on histopathology. Universally accepted and validated guidelines for the management of neutrophilic dermatoses do not exist, also given the paucity of randomized controlled study and high-quality data. However, the literature on the effective use of biologic therapies is rapidly expanding. This article reviews the epidemiology, clinical characteristics, histopathologic features, and management of pyoderma gangrenosum as well as Sweet's syndrome, sub-corneal pustular dermatoses and bowel-associated dermatosis arthritis syndrome. The use of biologic agents, including tumor necrosis factor α-inhibitors, anti-IL1, anti-IL-17, and IL-23 are discussed in detail.

Drug management of neutrophilic dermatoses.

INTRODUCTION: Neutrophilic dermatoses are a heterogenous group of chronic, cutaneous inflammatory conditions characterized by the accumulation of neutrophils in the skin and by systemic inflammation. Neutrophilic dermatoses can be idiopathic or associated with other inflammatory or systemic diseases, including the group of the hereditary, autoinflammatory syndromes. Clinical management is challenging, due to limited clinical evidence and lack of clinical practice guidelines. Areas covered: This review provides an overview of current therapeutic management of the three prototypical neutrophilic dermatoses, aseptic pustulosis of the folds, Sweet syndrome and pyoderma gangrenosum. In addition, we describe innovative, pathogenesis-oriented treatment approaches, which are based on recent advances in the pathophysiology of neutrophilic dermatoses and autoinflammatory syndromes. The increasing role of the IL-1 cytokine family in initiating neutrophilic inflammation in both idiopathic and syndromic disease opened the way for the use of targeted biological treatment. Another promising treatment strategy is aimed at blocking downstream effector cytokines, such as IL12/23 and IL-17, involved in the autoinflammatory immune cascade. Expert commentary: In chronic-recurrent and syndromic cases of neutrophilic dermatoses, there is an unmet clinical need for long-term, continuous disease control. Future controlled clinical studies will optimize the use of targeted-biological agents in sequential or combination treatment strategies.