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J. Am. Acad. Dermatol. · Jan 2016
Case ReportsFailure to thrive, interstitial lung disease, and progressive digital necrosis with onset in infancy.
- Justin Chia, Fehime Kara Eroglu, Seza Özen, Dicle Orhan, Gina Montealegre-Sanchez, Adriana A de Jesus, Raphaela Goldbach-Mansky, and Edward W Cowen.
- Division of Dermatology, Department of Medicine, University of Calgary, Calgary, Alberta, Canada.
- J. Am. Acad. Dermatol. 2016 Jan 1; 74 (1): 186-9.
AbstractKey teaching points • SAVI is a recently described interferonopathy resulting from constitutive action of STING and up-regulation of IFN-β signaling. • SAVI is characterized by facial erythema with telangiectasia, acral/cold-sensitive tissue ulceration and amputations, and interstitial lung disease. It has overlapping features with Aicardi-Goutières syndrome and familial chilblain lupus. • Traditional immunosuppressive medications and biologic therapies appear to be of limited benefit, but JAK inhibitors may impact disease progression.Published by Elsevier Inc.
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