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Giornale Italiano di Dermatologia e Venereologia 2020 Jul 02

DOI: 10.23736/S0392-0488.20.06709-7

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English

Type I interferonopathies: cutaneous vasculopathy, chilblains, panniculitis-induced lipodystrophyand others skin manifestations

Riccardo PAPA 1, 2, Stefano VOLPI 1, 2 , Marco GATTORNO 1

1 Autoinflammatory Diseases and Immunodeficiencies Center, Giannina Gaslini Institute, Genoa, Italy; 2 Department of Neuroscience, Rehabilitation, Ophthalmology, Genetic and Maternal-Infant Sciences, University of Genoa, Genoa, Italy


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Type I interferonopathies are a clinically heterogeneous group of inherited disorders of the innate immune system characterized by constitutive activation of the type I interferon-signaling pathway. Cutaneous vasculopathy, lipodystrophy, interstitial lung disease and brain calcifications are the typical manifestations characterizing affected patients. The pathogenic mechanism commonly underlying these disorders is the abnormal activation of immune pathways involved in recognition of non-self-oligonucleotides. These natural defenses against virus consent humans to survive the infections. Target therapies capable of inhibiting type I interferon signaling pathway seems effective in these patients, albeit with possible incomplete responses and severe side effects.


KEY WORDS: Rheumatic diseases; Hereditary autoinflammatory diseases; Lupus erythematosus, systemic

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