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Updated: Jul 3, 2025

Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Vasculitis and vasculopathy associated with inborn errors of immunity: an overview
Silvia Federici1, Bianca Laura Cinicola2,3, Francesco La Torre4
1Division of Rheumatology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Abstract:
Systemic autoinflammatory diseases (SAIDs) are disorders of innate immunity, which are characterized by unprovoked recurrent flares of systemic inflammation often characterized by fever associated with clinical manifestations mainly involving the musculoskeletal, mucocutaneous, gastrointestinal, and nervous systems. Several conditions also present with varied, sometimes prominent, involvement of the vascular system, with features of vasculitis characterized by variable target vessel involvement and organ damage. Here, we report a systematic review of vasculitis and vasculopathy associated with inborn errors of immunity.
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