Letter
Blue (or purple) toes: Chilblains or chilblain lupus-like lesions are a manifestation of Aicardi–Goutières syndrome and familial chilblain lupus

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Cited by (18)

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    Enhanced interferon signaling may contribute to the variable perivascular lymphocytic infiltrate seen in these seemingly normal skin biopsies in patients with capillary leak syndrome. In all of the aforesaid interferon endotheliopathy syndromes, the endothelial cell injury and vascular inflammation can result in ischemia as evidenced by the pattern of vascular drop out with ensuing fibroplasia observed in the skin in both dermatomyositis and Kohlmeier-Degos disease, the progressive loss of brain substance attributable to a necrotizing lymphocytic vasculitis that characterizes Aicardi-Goutières syndrome, and the painful edematous purpuric papular nodular lesions reflective of lymphocytic vasculitis encountered on the toes and fingers observed in patients with idiopathic perniosis and COVID-19 associated perniosis [21-27]. The potential role of interferon alpha in enhancing endothelial cell vasopermeability was first described by Puri and Rosenberg in their paper entitled “The Combined Effects of Interferon Alpha and Interleukin 2 on the Induction of a Vascular Leak Syndrome in Mice.”

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Conflicts of interest: None declared.

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