Dermatomyositis associated with hepatitis B virus-related hepatocellular carcinoma
A 55-year-old male visited our hospital complaining of progressive weakness in his bilateral arms, hips, and thighs. He had difficulty combing his hair and was experiencing dysphagia and erythematous changes in the skin (his face and a 'V' on his upper chest and neck that had developed 3 weeks earlier) ().\nA physical examination revealed a heliotrope rash with bilateral periorbital edema and poikiloderma on the upper chest and neck. A musculoskeletal examination showed marked weakness of all proximal muscles in the symmetric extremities. Serum laboratory tests revealed the following: white blood cells 9,720/mm3, hemoglobin 13.6 g/dL, platelets 98,000/mm3, creatine kinase 16,440 IU/L (normal range, 0 to 190), lactate dehydrogenase 994 IU/L, aspartate aminotransferase 717 IU/L, alanine transaminase 139 IU/L, total bilirubin 1.4 mg/dL, albumin 2.2 g/dL, alkaline phosphatase 119 IU/L, and prothrombin time (activity) 55%. HBV surface antigen was detected, and the HBV DNA level was 7,380 IU/mL. No antibodies against hepatitis C virus were detected.\nIgM antibodies against Epstein-Barr virus, cytomegalovirus, and herpes simplex virus were not detected. There was no other evidence of infectious mononucleosis. Antinuclear antibody and anti-SS-Ro tests were both positive. Tests for other autoantibodies, including anti-dsDNA, anti-ribonucleoprotein, anti-Sm, anti-Jo-1, and anti-Scl70, were all negative. Electromyography showed a small number of polyphasic waves with a normal interference pattern in all muscles tested. A muscle biopsy of the right upper arm showed a sparse lymphocytic infiltrate around the blood vessels and negative immunofluorescence pathologic findings, compatible with DM (). The patient's levels of carcinoembryonic antigen, carbohydrate antigen 19-9, and prostate-specific antigen were normal, but he had elevated levels of α-fetoprotein (AFP; 448.8 ng/mL) and proteins induced by vitamin K absence-II (> 500 mAU/mL). Liver dynamic computed tomography (CT) revealed a 6.5-cm sized arterial enhancing mass in hepatic segment 7/6 ().\nWe diagnosed the patient with HBV-related HCC based on his status as an HBV carrier, elevated AFP level (> 200 ng/mL), and typical radiographic findings (e.g., arterial enhancing features on liver dynamic CT).\nOptimal management procedures for HCC such as transcatheter arterial chemoembolization could not be performed because of the patient's poor general condition. We started prophylactic antiviral therapy with lamivudine for the prevention of viral reactivation. A total of 60 mg of oral prednisolone was administered daily. After steroid therapy, the patient's skin rash, muscle strength, and dysphagia showed slight improvement, and his elevated muscle enzyme levels returned to the near-normal range. However, his underlying HCC progressed rapidly: the size of the cancer grew up to 11 cm with portal vein thrombosis and multiple lymph node metastasis. The patient ultimately died due to hepatic failure after 4 months.