A previous study of SSc revealed that Ro52 positive patients with IP showed worse survival after 40 months, especially Ro52 mono-positive patients.(32) In the present study, there was no statistical difference of survival between the Ro52 mono and Ro52 overlap groups up to 2 years, but the Ro52 mono group had significantly worse survival than the Ro52 negative group. The gene discussed is TRIM21; the disease is incontinentia pigmenti.