Novel use of Autoinflammatory Diseases Activity Index (AIDAI) captures skin and extracutaneous features to help manage pediatric DITRA: A case report and a proposal for a modified disease activity index in autoinflammatory keratinization disorders

• DOI: 10.1111/pde.14155
• Publication Date: 2020-04-17T07:03:51Z
• Authors: Chloe Stephenson; Vimal H. Prajapati; Charlene Hunter; Paivi Miettunen
• Keywords: Generalized pustular psoriasis

Abstract Generalized pustular psoriasis (GPP) is a severe form of psoriasis, which rare in pediatric and adult patients. It characterized by sterile lesions that appear on erythematous skin, associated with systemic features. A recent identification mutations the IL36RN gene some GPP patients has led to diagnosis new autoinflammatory disease, interleukin‐36‐receptor antagonist deficiency (DITRA). DITRA represents an emerging group diseases hyperkeratotic skin involvement, called keratinization (AIKD). management are challenging as neither DITRA‐specific clinical assessment tools nor treatment trials exist. Autoinflammatory Diseases Activity Index (AIDAI) validated tool originally developed evaluate disease activity response other inherited involvement. We report first use AIDAI patient following goals: (a) describe contribution our patient's management; (b) identify potential limitations DITRA; (c) review literature for current tools; (d) propose preliminary DITRA/AIKD index (DITRA/AIDAI) be future studies.