Aicardi–Goutières syndrome

Aicardi–Goutières syndrome (AGS), which is completely distinct from the similarly named Aicardi syndrome, is a rare, usually early onset childhood, inflammatory disorder most typically affecting the brain and the skin (neurodevelopmental disorder).The majority of affected individuals experience significant intellectual and physical problems, although this is not always the case. The clinical features of AGS can mimic those of in utero acquired infection, and some characteristics of the condition also overlap with the autoimmune disease systemic lupus erythematosus.

Disease classification WHO

93.4 Aicardi–Goutières syndrome

ICD-10 online

https://www.wikidata.org/wiki/Q403453 Aicardi–Goutières syndrome