[Bullous pemphigoid and primary biliary cholangitis, a rare association with therapeutic challenge]

The authors hereby present the case study of a 63-year-old female patient who was admitted to the dermatology department due to the presence of tight-walled bullae all over her body. Prior to this admission, the patient had been prescribed low-to-medium dose of steroids in another hospital. Biopsy was taken from the patient's skin lesions, and the diagnosis of bullous pemphigoid was confirmed using direct immunofluorescence testing. The markedly elevated liver function tests observed at admission were hypothesized to be a consequence of steroid administration, and thus, the administration of hepatotoxic agents was withheld pending further investigation. During the examination, primary biliary cholangitis was diagnosed as the cause of the liver function abnormalities. In view of the patient's serious condition and in consideration of the hepatotoxic side effects of first-line treatments, we initiated plasmapheresis. Following a thorough examination of international guidelines, the decision was taken to initiate off-label omalizumab therapy, which subsequently led to the patient becoming asymptomatic from a dermatological perspective. The therapeutic intervention of ursodesoxycholic acid was initiated for hepatological indications. The co-occurrence of bullous pemphigoid and primary biliary cholangitis is an extremely rare condition based on the literature. We present this case because of its rarity and therapeutic difficulty, and emphasize the importance of cooperation between different medical specialties. Orv Hetil. 2026; 167(11): 439-444.