GANGRENOUS PYODERMA AS A RARE EXTRAHEPATIC MANIFESTATION OF SERONEGATIVE PRIMARY BILIARY CHOLANGITIS

Abstract

Background. A seronegative form of primary biliary cholangitis presents a diagnostic challenge, as it lacks serological markers and requires morphological verification. Cases of association between primary biliary cholangitis and pyoderma gangrenosum are rare. Objective. To describe a rare extrahepatic manifestation of pyoderma gangrenosum with scleroderma-like skin lesions in a patient with seronegative primary biliary cholangitis. Material and methods. We presented clinical observation data of patient V., aged 38 and treated in the gastroenterology department of the city clinical hospital. Results. The disease first manifested itself in cholestatic syndrome, accompanied by widespread ulcerative skin lesions on the hands and skin scleroderma-like changes. Blood tests revealed cytolysis and cholestasis. Markers for autoimmune liver diseases and hepatitis viruses were negative. Magnetic resonance cholangiopancreatography was normal. Liver morphology was characterized by minimal hepatitis and destructive cholangitis; ductular proliferation, cholestasis, and portal fibrosis were detected. Skin biopsy revealed scleroderma-like changes without vasculitis. Treatment with ursodeoxycholic acid, systemic glucocorticosteroids, and azathioprine resulted in ulcer epithelialization and normalization of biochemical parameters. Conclusion. Based on the examination, seronegative primary biliary cholangitis with extrahepatic manifestations – pyoderma gangrenosum – was diagnosed. The complexity of diagnosing necessitated a multidisciplinary approach and a comprehensive examination of the patient.