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The causes of liver disease, ranging from fatty changes to cirrhosis and hepatocellular carcinoma, in porphyria cutanea tarda (PCT) remain unclear. We tested 100 consecutive PCT patients for antibodies to hepatitis C virus (HCV) by enzyme-linked immunosorbent assay and a recombinant immunoblot assay. 75 (79%) patients with sporadic PCT but none of 5 with… (More)
BACKGROUND AND OBJECTIVES Dermatitis herpetiformis is a chronic bullous disease that is currently considered a cutaneous expression of gluten hypersensitivity. The aim of this study was to analyze and describe the clinical, histological, and immunopathological characteristics of patients with dermatitis herpetiformis assessed at Hospital Clinic de… (More)
CT and MR findings in two patients with hepatoerythropoietic porphyria are presented. CT scans showed atrophy and cortical mineralization at the same level. MR examination performed in one of the two patients showed mainly frontal cortical atrophy and punctate bright signal on T1- and T2-weighted sequences.
Paraneoplastic pemphigus is a life-threatening autoimmune bullous disease associated with neoplasia, generally of lymphoid origin. Immunosuppressive therapy is often disappointing and there are only a few reports of patients surviving more than 2 years. These cases were generally associated with benign neoplasms. We report here the case of a patient with… (More)
Uroporphyrinogen decarboxylase levels were measured in haemolysed whole blood or fibroblasts from 3 unrelated patients with hepatoerythropoietic porphyria (HEP) and in 4 unrelated patients with familial porphyria cutanea tarda, a condition in which the enzyme is defective. In HEP patients enzyme activities were 7% of normal in erythrocytes and 8% of normal… (More)
Chronic graft-vs-host disease (GVHD) remains the most common late complication of allogeneic hematopoietic stem cell transplantation (HSCT), despite improvements in its prophylaxis. Although the graft-vs-leukemia effect associated with the development of chronic GVHD may be beneficial in controlling the underlying disease, patients with chronic GVHD are… (More)