A 47-year-old man comes to the office due to recurrent blisters on the skin of the back of his hands. He has also been fatigued for the past several months and has had occasional joint pain. The patient was told that his liver enzymes were elevated during a life insurance medical evaluation 2 years ago but did not follow up. He does not use tobacco or alcohol. The patient has no family history of skin disorders. Temperature is 36.8 C (98.2 F), blood pressure is 137/81 mm Hg, and pulse is 78/min. Skin examination shows small vesicles and erosions on the dorsa of both hands. Prior lesions appear healed with scarring and hyperpigmentation. The remainder of the physical examination is unremarkable. The results of liver function studies are as follows: Further evaluation of this patient will most likely reveal which of the following conditions?

Question

A 47-year-old man comes to the office due to recurrent blisters on the skin of the back of his hands.  He has also been fatigued for the past several months and has had occasional joint pain.  The patient was told that his liver enzymes were elevated during a life insurance medical evaluation 2 years ago but did not follow up.  He does not use tobacco or alcohol.  The patient has no family history of skin disorders.  Temperature is 36.8 C (98.2 F), blood pressure is 137/81 mm Hg, and pulse is 78/min.  Skin examination shows small vesicles and erosions on the dorsa of both hands.  Prior lesions appear healed with scarring and hyperpigmentation.  The remainder of the physical examination is unremarkable.  The results of liver function studies are as follows:  Further evaluation of this patient will most likely reveal which of the following conditions?

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11ec25c8_d37c_7a56_bc81_4db0f4ace75b_MD0003_00 This patient has several features suggestive of chronic hepatitis C virus (HCV) infection, including fatigue, elevated transaminases, arthralgias, and porphyria cutanea tarda (PCT). In addition to PCT, other common extrahepatic manifestations of HCV include mixed cryoglobulinemia (eg, palpable purpura, glomerulonephritis, low complement levels) and lichen planus. PCT is due to reduced activity of a hepatic enzyme involved in the heme biosynthesis pathway, leading to the accumulation of porphyrins, which are eventually transported to skin. PCT presents with fragile skin with vesicles and bullae in areas of trauma or sun exposure (eg, dorsa of the hands). Healed lesions typically scar and can form both hypo- and hyperpigmented areas. The diagnosis is supported by elevated plasma and urine porphyrin levels. Because PCT is strongly associated with HCV, all patients with PCT should be screened for HCV. Treatment involves serial phlebotomy or hydroxychloroquine along with management of the underlying causes (eg, antiviral therapy for HCV). dorsa of the hands (Choice A) Alpha-1 antitrypsin deficiency is associated with emphysematous lung disease, chronic hepatitis, cirrhosis, and necrotizing panniculitis (painful, erythematous nodules and plaques on the thighs or buttocks). The lack of lung disease and the vesicular morphology of this patient's skin lesions make this diagnosis less likely. (Choice C) Primary biliary cholangitis (previously primary biliary cirrhosis) can cause fatigue, but liver function studies typically demonstrate a cholestatic pattern (eg, high alkaline phosphatase). Dermatologic manifestations include pruritus, jaundice, hyperpigmentation, and xanthomas but not PCT. (Choice D) Primary sclerosing cholangitis is characterized by fibrosis and obliteration of intrahepatic bile ducts. Laboratory analysis typically demonstrates cholestasis, not elevated aminotransferases. Patients frequently have jaundice and pruritus but not PCT. (Choice E) Wilson disease (inherited disorder of copper accumulation) classically presents in young patients with liver disease, neuropsychiatric manifestations (eg, dysarthria, movement disorders, mood or personality changes), and Kayser-Fleischer rings (green or golden deposits encircling the iris). Blistering skin lesions in sun-exposed areas is not a feature. Kayser-Fleischer rings Educational objective: Porphyria cutanea tarda is characterized by fragile, photosensitive skin with vesicles or bullae in areas of trauma or sun exposure. It is strongly associated with hepatitis C virus infection and warrants screening. References: Systemic manifestations of hepatitis C infection. (http://www.ncbi.nlm.nih.gov/pubmed/27222662) Extrahepatic manifestations of chronic hepatitis C virus infection. (http://www.ncbi.nlm.nih.gov/pubmed/26862398)

A 47-Year-Old Man Comes to the Office Due to Recurrent