DOI: https://doi.org/10.36347/sjmcr.2025.v13i08.033

Pages: 1882-1885

Primary biliary cholangitis (PBC), a chronic autoimmune liver disease, is frequently associated with other autoimmune disorders such as Sjögren's syndrome and thyroid diseases. However, its coexistence with superficial pemphigus is extremely rare, with no prior cases reported in the literature. We present a case of a 49-year-old woman diagnosed with PBC who developed superficial pemphigus during the course of her disease, treated with corticoids followed by RITUXIMAB as background treatment with good clinical evolution. This case emphasizes the importance of considering rare autoimmune associations in PBC and highlights the need for coordinated care between hepatology and other specialists.