Cutaneous Manifestations of Autoinflammatory Diseases.

Abstract

Autoinflammatory diseases (AIDs) are a heterogeneous group of disorders in which recurrent or continuous aseptic inflammation arises primarily through antigen-independent hyperactivation of the innate immune system. The skin is frequently involved with a wide variety of cutaneous manifestations, most of which are non-specific. Recognition of skin lesions in AIDs may sometimes provide clues for a correct diagnosis. In this review, the cutaneous involvements of >20 selected AIDs were summarized and organized into different categories based on their characteristic manifestations, such as urticarial dermatosis, neutrophilic dermatosis, granulomatosis, chilblain, lipodystrophy, and hyperkeratosis. With this classification scheme, cutaneous manifestations in AIDs could be more easily identified to facilitate diagnosis in clinical practice.