Paraneoplastic Dermatomyositis in Patient with Primary Biliary Cirrhosis and Hepatocellular Carcinoma: A Case Report.

BACKGROUND Dermatomyositis is an autoimmune disease characterized by distinctive skin changes, muscle involvement, and, in many cases, damage to various organs, such as interstitial lung disease, arthritis, and myocarditis. The association between dermatomyositis and malignancies is well established. The most common cancers that manifest with a dermatomyositis are neoplasms of the breast, lungs, cervix, and gastrointestinal tract. Primary biliary cirrhosis is a slowly progressive autoimmune disease characterized by a triad of chronic cholestasis, positive specific autoantibodies, and characteristic imaging or pathomorphological findings. Regardless of etiology, cirrhosis is considered as a major risk factor for development of hepatocellular carcinoma. This type of liver neoplasm is rarely associated with dermatomyositis. CASE REPORT We report the case of a 64-year-old woman with comorbid primary biliary cirrhosis, hepatocellular carcinoma, and dermatomyositis as a paraneoplastic syndrome. Pathognomonic skin lesions of dermatomyositis, such as heliotrope rash, Gottron papules, Gottron sign, V-sign, and proximal muscle weakness preceded the diagnosis of biliary cholangitis and hepatocellular carcinoma by more than a year but were not initially recognized. The failure to identify the symptoms of dermatomyositis, combined with the lack of pronounced and specific signs of primary biliary cirrhosis and hepatocellular carcinoma, contributed to delayed diagnoses. The patient died due to severe, decompensated heart failure. CONCLUSIONS This case report emphasized the importance of precise interpretation of pathogenetic findings by all specialists involved. In patients with multiple comorbidities, close collaboration between specialists and an interdisciplinary approach are essential for timely diagnosis and the selection of appropriate treatment, which can significantly improve prognosis and outcome.