BRAF and familial atrioventricular septal defect: In children, isolated skin JXG lesion are not known to harbor the BRAF V600E mutation (i.e., grouped as “C group” lesions) [49, 56]; however, in adults a cutaneous BRAF V600E xanthogranuloma is highly correlative with ECD, especially xanthelasmas, and should immediately prompt further clinico-radiographic investigation for ECD after biopsy diagnosis [15].