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
| العنوان: | 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 |
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| المؤلفون: | Charlene Hunter, Vimal H. Prajapati, Chloe Stephenson, Paivi Miettunen |
| المصدر: | Pediatric Dermatology. 37:670-676 |
| بيانات النشر: | Wiley, 2020. |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | Treatment response, medicine.medical_specialty, Future studies, Adult patients, business.industry, Dermatology, Activity index, medicine.disease, Disease activity, 030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, Quality of life, 030220 oncology & carcinogenesis, Psoriasis, Pediatrics, Perinatology and Child Health, medicine, Generalized pustular psoriasis, business |
| الوصف: | Generalized pustular psoriasis (GPP) is a severe form of psoriasis, which is rare in pediatric and adult patients. It is characterized by sterile pustular lesions that appear on erythematous skin, associated with systemic features. A recent identification of mutations in the IL36RN gene in some GPP patients has led to a diagnosis of new autoinflammatory disease, interleukin-36-receptor antagonist deficiency (DITRA). DITRA represents an emerging group of autoinflammatory diseases with hyperkeratotic skin involvement, called autoinflammatory keratinization diseases (AIKD). DITRA diagnosis and management are challenging as neither DITRA-specific clinical assessment tools nor treatment trials exist. Autoinflammatory Diseases Activity Index (AIDAI) is a validated tool originally developed to evaluate disease activity and treatment response in other inherited autoinflammatory diseases with systemic and skin involvement. We report the first use of AIDAI in a pediatric DITRA patient with the following goals: (a) to describe the contribution of AIDAI to our patient's management; (b) to identify potential limitations of AIDAI in DITRA; (c) to review literature for current psoriasis assessment tools; and (d) to propose a preliminary DITRA/AIKD disease activity index (DITRA/AIDAI) to be validated in future studies. |
| تدمد: | 1525-1470 0736-8046 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_________::f587b6d2e3e1caf4faf317d2e140a91bTest https://doi.org/10.1111/pde.14155Test |
| حقوق: | CLOSED |
| رقم الانضمام: | edsair.doi...........f587b6d2e3e1caf4faf317d2e140a91b |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15251470 07368046 |
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