In the Cox regression model, age (HR = 1.03 (1.01–1.05)) and especially the presence of anti-SSA antibodies in CIE (HR = 4.45 (2.35–8.42)) were significantly associated with the occurrence of extraglandular manifestations (Table 3). The gene discussed is TRIM21; the disease is congenital non-bullous ichthyosiform erythroderma.