Aicardi-Goutières syndrome: a possible explanation of angiokeratoma of Mibelli

Aicardi-Goutieres syndrome (AGS) is a rare autosomal recessive disorder first described by Jean Aicardi and Francoise Goutieres in 19841 . Given the elevated serum and cerebrospinal fluid (CSF) levels of interferon-α (IFNα), it belongs to the group of type 1 interferonopathies2 . Cutaneous manifestations are the most frequent extra neurologic findings and include chilblain-like lesions (CLL), acrocyanosis, distal tapering of digits, and nail abnormalities3-5 .