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

Type I interferonopathies: cutaneous vasculopathy, chilblains, panniculitis-induced lipodystrophyand others skin manifestations. G Ital Dermatol Venereol. 2020 Jul 02;: Authors: Papa R, Volpi S, Gattorno M Abstract 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. PMID: 32618445 [PubMed - as supplied by publisher]
Source: Giornale Italiano di Dermatologia e Venereologia - Category: Dermatology Tags: G Ital Dermatol Venereol Source Type: research

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Source: Expert Review of Clinical Immunology - Category: Allergy & Immunology Tags: Expert Rev Clin Immunol Source Type: research
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