Abstract
Autoinflammatory diseases (AID) are rare systemic inflammatory disorders due to monogenic or polygenic dysfunction of innate immunity. They affect many organs including the brain and the skin. The spectrum of these diseases has been rapidly expanding recently due to newly developed diagnostic tools. The neuro-immuno-endocrine-cutaneous interactions play an important role in the pathophysiology of these diseases. The skin-brain interplay is not fully investigated in AID and evidence supporting bidirectional communication is examined. This article provides an overview of the current state of the art in the pathophysiology of AID with cutaneous and psychiatric manifestations. Elucidating the neuro-immuno-endocrine-cutaneous dysregulation underlying pathophysiology of AID is promising for determining future biomarkers and therapeutic options.