Skin disease is highly prevalent in the United States, and it has been well-documented that patients with skin disease experience financial, psychological, social, and quality-of-life (QoL) burdens beyond those of…
The continuous advance in the search for the cause and pathogenesis of the autoinflammatory syndromes, as well as reports of the efficacy of specific inflammation-mediator suppressors, has changed the way…
The recent molecular, biologic, and genetic understanding of the inflammasome has revolutionized the diagnosis of and therapy for the phenotypically heterogeneous group of rare oligogenic disorders, now recognized to have…
Autoinflammatory diseases (AIDs) are characterized by recurrent episodes of systemic and organ-specific inflammation. Many of these diseases share fever as a common presenting feature. Physicians need to consider AIDs in…
Autoinflammatory syndromes comprise a diagnostically challenging group of systemic inflammatory disorders uniquely related by (1) dysregulation of innate immunity, (2) inflammasome activation, (3) dramatic clinical features (high fevers, neutrophilic rashes,…
Autoinflammatory disorders are disorders of the innate immune system that are distinct from autoimmune disorders. Dysregulation of the innate immune system, specifically an increase in interleukin-1 beta (IL-1β), gives rise…
Interleukin-1 (IL-1) is a potent inflammatory cytokine that plays a central role in the innate immune response. IL-1 mediates the acute phase of inflammation by inducing local and systemic responses,…
This article presents a summary of the evidence for a link between autoinflammatory diseases and psoriasis. The main concepts regarding the disease state of psoriasis are discussed and these lead…
Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerating skin lesions with rapid onset and recalcitrant treatment course. PG treatment targets an array of inflammatory pathways with variable success….
This article provides a new categorization of inflammatory pustular dermatoses in the context of recent genetic and biological insights. Monogenic diseases with pustular phenotypes are discussed, including deficiency of interleukin…
