A heterozygous LOF variant in MAPK8, the gene encoding the c-Jun N-terminal kinase 1 (JNK1) was recently reported in a three-generation kindred with a complex syndrome combining chronic mucocutaneous candidiasis and a connective tissue disorder but no evidence of aortic aneurysm or dissection (5). Here, MAPK8 is linked to Chronic mucocutaneous candidosis.