According to Michet et al., patients with suspected RPC must have inflammation in at least two cartilage locations or one chondritis site with at least two of the following features: ocular inflammation, seronegative arthritis, hearing loss, and vestibular dysfunction [7]. There are no specific diagnostic tests to confirm the diagnosis; however, laboratory tests, such as CBC, ESR, CRP, autoimmune and infectious screening, including RF, ANA, anticytoplasmic antibody (c-ANCA), and VDRL test, and urinalysis are commonly requested to rule out concomitant or associated autoimmune diseases [1]. This evidence concerns the gene ESR1 and familial reactive perforating collagenosis.