CLCN5 and nevus comedonicus syndrome: Dent disease 2 (OMIM phenotype number 300555) is an XLR condition resulting from inactivating variants in OCRL. Clinical features are shared with that of Dent disease 1 caused by variants in CLCN5 with progressive proximal renal tubulopathy with LMW proteinuria, hypercalciuria, NC, NL, and progressive ESKD (Claverie-Martín et al., 2011).