Clinical amyopathic DM (C-ADM), autoantibodies such as anti-aminoacyl tRNA synthetase (ARS) and anti-melanoma differentiation-associated gene 5 (MDA5) antibodies (Ab), high-resolution computed tomography findings such as ground-glass attenuation and reticular opacity, and a decrease in the diffusing capacity on respiratory function tests have been reported as findings related to the severity and prognosis of DM-IP [6]–[12]. Here, IFIH1 is linked to incontinentia pigmenti.