The University of Alabama at Birmingham, USA.
J Investig Med High Impact Case Rep. 2022 Jan-Dec;10:23247096221089488. doi: 10.1177/23247096221089488.
Primary biliary cholangitis (PBC) is a rare autoimmune disease characterized by intralobular bile duct destruction. Patients typically present with generalized symptoms including fatigue and pruritis, and less commonly, manifestations of lipid deposition including xanthomas and xanthelasmas. We report a case of a 31-year-old female with PBC-associated cirrhosis who had cutaneous xanthelasmas and diffuse gastric xanthomas secondary to hyperlipidemia and lipoprotein X that completely resolved following liver transplantation. While gastric xanthomas have been reported in patients with PBC previously, to our knowledge, this is the first case report of diffuse gastric xanthomas secondary to PBC reported to resolve following liver transplantation, suggesting that liver transplantation is curative for gastric xanthomatosis in patients with PBC-related cirrhosis.
原发性胆汁性胆管炎（PBC）是一种罕见的自身免疫性疾病，其特征为小叶间胆管破坏。患者通常表现为全身性症状，包括疲劳和瘙痒，以及较少见的脂质沉积表现，包括黄斑瘤和黄瘤。我们报告了一例 PBC 相关性肝硬化患者，其患有与高脂血症和脂蛋白 X 相关的皮肤黄斑瘤和弥漫性胃黄斑瘤，这些病变在肝移植后完全消退。虽然之前有报道称 PBC 患者存在胃黄斑瘤，但据我们所知，这是首例报告的 PBC 相关性肝硬化患者的弥漫性胃黄斑瘤在肝移植后消退的病例，表明肝移植对 PBC 相关肝硬化患者的胃黄色瘤病具有治愈作用。
