Liver fibrosis and cirrhosis are predisposing factors for the development of hepatocellular carcinoma (HCC). Hemosiderosis has also been described to trigger carcinogenesis. A significant iron overload, as found in he...Liver fibrosis and cirrhosis are predisposing factors for the development of hepatocellular carcinoma (HCC). Hemosiderosis has also been described to trigger carcinogenesis. A significant iron overload, as found in hereditary hemochromatosis (HHC), is a risk factor for HCC and may also promote the symptoms of porphyria cutanea tarda (PCT). A 68-year old male patient presented to our clinic with a suspected HCC, elevated alpha-fetoprotein but normal liver function tests. He reported a 25 year-old history of vitiligo upon exposure to sunlight. The patient underwent an extended left hemihepatectomy, and the recovery was uneventful, with the exception of a persistent hyperbilirubinemia. Perfusion problems and extrahepatic cholestasis were ruled out by CT-scan with angiography and MR-cholangiopancreatography. However, MRI showed an iron overload. Histology confirmed the HCC (pT3, pN0, G3, R0) and revealed a portal fibrosis and hemosiderosis. Based on the skin lesions we suspected a PCT that was confirmed by laboratory tests showing elevated porphyrin, uroporphyrin, coproporphyrin and porphobilinogen. Concurrently, molecular diagnostics revealed homozygosity for the C282Y mutation within the hemochromatosis HFE gene. After phlebotomy and normalization of liver function tests the patient was discharged. This is the first case ever showing the unusual combination of HCC in a fibrotic liver with HHC and PCT. This diagnosis not only warrants oncological follow-up but also symptomatic therapy to normalize iron metabolism and thereby improve liver function and alleviate the symptoms of HHC and PCT. Thus progression of fibrosis may be prevented and liver regeneration supported.展开更多
Hepatitis C virus (HCV) affects millions of people worldwide,and an estimated 3.2 million people in the United States.HCV is a hepatotropic and lymphotropic virus that causes not only liver disease,but also a signific...Hepatitis C virus (HCV) affects millions of people worldwide,and an estimated 3.2 million people in the United States.HCV is a hepatotropic and lymphotropic virus that causes not only liver disease,but also a significant number of extrahepatic manifestations (EHMs).Up to 74% of patients affected by HCV will have HCV-related EHMs of some severity in their lifetime.The EHMs vary from simple cutaneous palpable purpura to complex lymphoproliferative disorders,including lymphomas and immune-complex deposit diseases causing local and/or systemic complications.Mixed cryoglobulinemia (MC) is manifested by multiple systemic organ involvement,mainly skin,kidney,peripheral nerves,and salivary glands,and less frequently causes widespread vasculitis and malignant lymphoma.MC affects up to 3% of HCV-infected patients with cryoglobulinemia of clinical significance,i.e.>6%.Severe disease requires immunosuppressive or plasma exchange therapy.HCV prevalence in the United States in patients with porphyria cutanea tarda (PCT) was reported to be 66%,much higher than that in general population.Therefore,all patients with PCT should be screened for HCV.The skin rash of PCT varies from large blisters to small vesicles and/or milia on the hands.Skin manifestations due to PCT usually respond to anti-HCV treatment together with reducing skin sun exposure,avoiding triggers,having routine phlebotomy (especially for people with chronic iron overload states),and using chloroquine.Lichen planus (LP),which typically affects both the skin and oral mucosa is a chronic inflammatory disease of squamous cell origin affecting about 1% of the worldwide population.The prevalence of HCV in patients with LP varies based on geographic location.We review here the basic pathophysiology,clinical features,and management of dermatologic manifestations of HCV.展开更多
文摘Liver fibrosis and cirrhosis are predisposing factors for the development of hepatocellular carcinoma (HCC). Hemosiderosis has also been described to trigger carcinogenesis. A significant iron overload, as found in hereditary hemochromatosis (HHC), is a risk factor for HCC and may also promote the symptoms of porphyria cutanea tarda (PCT). A 68-year old male patient presented to our clinic with a suspected HCC, elevated alpha-fetoprotein but normal liver function tests. He reported a 25 year-old history of vitiligo upon exposure to sunlight. The patient underwent an extended left hemihepatectomy, and the recovery was uneventful, with the exception of a persistent hyperbilirubinemia. Perfusion problems and extrahepatic cholestasis were ruled out by CT-scan with angiography and MR-cholangiopancreatography. However, MRI showed an iron overload. Histology confirmed the HCC (pT3, pN0, G3, R0) and revealed a portal fibrosis and hemosiderosis. Based on the skin lesions we suspected a PCT that was confirmed by laboratory tests showing elevated porphyrin, uroporphyrin, coproporphyrin and porphobilinogen. Concurrently, molecular diagnostics revealed homozygosity for the C282Y mutation within the hemochromatosis HFE gene. After phlebotomy and normalization of liver function tests the patient was discharged. This is the first case ever showing the unusual combination of HCC in a fibrotic liver with HHC and PCT. This diagnosis not only warrants oncological follow-up but also symptomatic therapy to normalize iron metabolism and thereby improve liver function and alleviate the symptoms of HHC and PCT. Thus progression of fibrosis may be prevented and liver regeneration supported.
文摘Hepatitis C virus (HCV) affects millions of people worldwide,and an estimated 3.2 million people in the United States.HCV is a hepatotropic and lymphotropic virus that causes not only liver disease,but also a significant number of extrahepatic manifestations (EHMs).Up to 74% of patients affected by HCV will have HCV-related EHMs of some severity in their lifetime.The EHMs vary from simple cutaneous palpable purpura to complex lymphoproliferative disorders,including lymphomas and immune-complex deposit diseases causing local and/or systemic complications.Mixed cryoglobulinemia (MC) is manifested by multiple systemic organ involvement,mainly skin,kidney,peripheral nerves,and salivary glands,and less frequently causes widespread vasculitis and malignant lymphoma.MC affects up to 3% of HCV-infected patients with cryoglobulinemia of clinical significance,i.e.>6%.Severe disease requires immunosuppressive or plasma exchange therapy.HCV prevalence in the United States in patients with porphyria cutanea tarda (PCT) was reported to be 66%,much higher than that in general population.Therefore,all patients with PCT should be screened for HCV.The skin rash of PCT varies from large blisters to small vesicles and/or milia on the hands.Skin manifestations due to PCT usually respond to anti-HCV treatment together with reducing skin sun exposure,avoiding triggers,having routine phlebotomy (especially for people with chronic iron overload states),and using chloroquine.Lichen planus (LP),which typically affects both the skin and oral mucosa is a chronic inflammatory disease of squamous cell origin affecting about 1% of the worldwide population.The prevalence of HCV in patients with LP varies based on geographic location.We review here the basic pathophysiology,clinical features,and management of dermatologic manifestations of HCV.
