We established the diagnosis of pSS based on ACR-EULAR criteria for the diagnosis of Sjogren syndrome.7 Under-expression of H(+)-ATPase and pendrin is thought to be the main mechanism of distal renal tubular acidosis in patients with SS.8 Usually dRTA is mild in pSS but can be severe in some patients. The gene discussed is SLC26A4; the disease is peeling skin syndrome.