RESUMO

This is a case report about Porphyria cutanea tarda (PCT) and its relationship with the infection caused by the human immunodeficiency virus (HIV). Cutaneous porphyria is an illness caused by enzymatic modification that results in partial deficiency of uroporphyrinogen decarboxylase (Urod), which may be hereditary or acquired. Several studies suggest that HIV infection associated with cofactors might trigger the development of porphyria cutanea tarda. In this case report, we present a patient infected with HIV, who after the introduction of antiretroviral therapy (ART) enjoyed clinical improvement of porphyria cutanea tarda symptoms.

Assuntos

RESUMO

Abstract: This is a case report about Porphyria cutanea tarda (PCT) and its relationship with the infection caused by the human immunodeficiency virus (HIV). Cutaneous porphyria is an illness caused by enzymatic modification that results in partial deficiency of uroporphyrinogen decarboxylase (Urod), which may be hereditary or acquired. Several studies suggest that HIV infection associated with cofactors might trigger the development of porphyria cutanea tarda. In this case report, we present a patient infected with HIV, who after the introduction of antiretroviral therapy (ART) enjoyed clinical improvement of porphyria cutanea tarda symptoms.

Assuntos

RESUMO

Porphyria cutanea tarda (PCT) is considered an extra-hepatic manifestation of HCV infection. The frequency of this association varies according to different authors and the mechanism by which the virus can trigger this disease is not yet clear. We present a 47-year-old-man with chronic hepatitis C genotype 1b who, during the treatment with peg-interferón alfa 2b plus ribavirina, with no detectable viremia at weeks 12th, 24th, and 48th, developed dermatological photosensitive lesions at week 44th. With a presumptive diagnosis of PCT a cutaneous/skin biopsy was performed as well as a porphyrin dosage with urine porphyirins of 4185 microg/24 hs (nv<250). The chromatographic analysis revealed the typical PCT pattern thus confirming the diagnosis. The hemochromatosis HFE gen evaluation showed heterozigotus character mutations (H63D and C282Y) a frequent association in patients with iron overload and PCT. The antiviral treatment of the HCV infection can improve the clinical-humoral manifestations of PCT. The novo occurrence of PCT was recently reported during chronic hepatitis C treatment with interferón and ribavirin, but no cases of late appearance of PCT in patients with no detectable viremia were reported. The mutation of the gen HFE in our patient and the hemolysis caused by ribavirin can be related to the development of the disease, but the iron overload because of ribavirin use is also controversial. This is another example of the complexity of this association.

Assuntos

RESUMO

La porfiria cutánea tarda (PCT) es considerada una manifestación extrahepática de la infección por el virusde la hepatitis C (HCV). La frecuencia de esta asociación es variable y no es claro el mecanismo por el cual el virus la desencadena. Se presenta un hombre de 47 años con hepatitis C, genotipo-1b, que durante el tratamiento con peg-interferón alfa-2b más ribavirinasin viremia detectable en las semanas 12, 24 y 48, en la semana 44 consulta por presentar lesiones dermatológicasfotosensibles. Con el diagnóstico presuntivo de PCT se realizó biopsia cutánea y el dosaje de porfirinasurinarias fue 4185 ug/24hs (vn: < 250). El análisis cromatográfico reveló el típico patrón de PCT, confirmando el diagnóstico. El gen HFE de la hemocromatosis mostró mutaciones de carácter heterocigoto (H63D y C282Y), asociación frecuente en los pacientescon sobrecarga de hierro y PCT. El tratamiento antiviral de la infección por HCV puede mejorar las manifestacionesde la PCT. La ocurrencia de novo de éstafue reportada recientemente durante el tratamiento de la hepatitis C crónica con interferón y ribavirina pero no hay casos relatados de aparición tardía de PCT enpacientes HCV con viremia no detectable. La presencia de la mutación del gen HFE y la posibilidad de unaumento de oferta de hierro por la acción hemolítica de la ribavirina podrían explicar un exceso de hierrocapaz de desencadenar la crisis de PCT. Tampoco sonconcluyentes los estudios con respecto al aumento o no de la concentración de hierro en hígado en pacientes con anemia que reciben ribavirina.(AU)

Porphyria cutanea tarda (PCT) is considered an extra-hepatic manifestation of HCV infection. The frequency of this association varies according to different authors and the mechanism by which the virus can trigger this disease is not yet clear. We present a 47-year-old-man with chronic hepatitis C genotype 1b who, during the treatment with peg-interferón alfa 2b plus ribavirina, with no detectable viremia at weeks 12th, 24th, and 48th, developed dermatological photosensitive lesions at week 44th. With a presumptive diagnosis of PCT a cutaneous/skin biopsy was performed as well as a porphyrin dosage with urine porphyirins of 4185 microg/24 hs (nv<250). The chromatographic analysis revealed the typical PCT pattern thus confirming the diagnosis. The hemochromatosis HFE gen evaluation showed heterozigotus character mutations (H63D and C282Y) a frequent association in patients with iron overload and PCT. The antiviral treatment of the HCV infection can improve the clinical-humoral manifestations of PCT. The novo occurrence of PCT was recently reported during chronic hepatitis C treatment with interferón and ribavirin, but no cases of late appearance of PCT in patients with no detectable viremia were reported. The mutation of the gen HFE in our patient and the hemolysis caused by ribavirin can be related to the development of the disease, but the iron overload because of ribavirin use is also controversial. This is another example of the complexity of this association.(AU)

Assuntos

RESUMO

La porfiria cutánea tarda (PCT) es considerada una manifestación extrahepática de la infección por el virusde la hepatitis C (HCV). La frecuencia de esta asociación es variable y no es claro el mecanismo por el cual el virus la desencadena. Se presenta un hombre de 47 años con hepatitis C, genotipo-1b, que durante el tratamiento con peg-interferón alfa-2b más ribavirinasin viremia detectable en las semanas 12, 24 y 48, en la semana 44 consulta por presentar lesiones dermatológicasfotosensibles. Con el diagnóstico presuntivo de PCT se realizó biopsia cutánea y el dosaje de porfirinasurinarias fue 4185 ug/24hs (vn: < 250). El análisis cromatográfico reveló el típico patrón de PCT, confirmando el diagnóstico. El gen HFE de la hemocromatosis mostró mutaciones de carácter heterocigoto (H63D y C282Y), asociación frecuente en los pacientescon sobrecarga de hierro y PCT. El tratamiento antiviral de la infección por HCV puede mejorar las manifestacionesde la PCT. La ocurrencia de novo de éstafue reportada recientemente durante el tratamiento de la hepatitis C crónica con interferón y ribavirina pero no hay casos relatados de aparición tardía de PCT enpacientes HCV con viremia no detectable. La presencia de la mutación del gen HFE y la posibilidad de unaumento de oferta de hierro por la acción hemolítica de la ribavirina podrían explicar un exceso de hierrocapaz de desencadenar la crisis de PCT. Tampoco sonconcluyentes los estudios con respecto al aumento o no de la concentración de hierro en hígado en pacientes con anemia que reciben ribavirina.(AU)

Porphyria cutanea tarda (PCT) is considered an extra-hepatic manifestation of HCV infection. The frequency of this association varies according to different authors and the mechanism by which the virus can trigger this disease is not yet clear. We present a 47-year-old-man with chronic hepatitis C genotype 1b who, during the treatment with peg-interferón alfa 2b plus ribavirina, with no detectable viremia at weeks 12th, 24th, and 48th, developed dermatological photosensitive lesions at week 44th. With a presumptive diagnosis of PCT a cutaneous/skin biopsy was performed as well as a porphyrin dosage with urine porphyirins of 4185 microg/24 hs (nv<250). The chromatographic analysis revealed the typical PCT pattern thus confirming the diagnosis. The hemochromatosis HFE gen evaluation showed heterozigotus character mutations (H63D and C282Y) a frequent association in patients with iron overload and PCT. The antiviral treatment of the HCV infection can improve the clinical-humoral manifestations of PCT. The novo occurrence of PCT was recently reported during chronic hepatitis C treatment with interferón and ribavirin, but no cases of late appearance of PCT in patients with no detectable viremia were reported. The mutation of the gen HFE in our patient and the hemolysis caused by ribavirin can be related to the development of the disease, but the iron overload because of ribavirin use is also controversial. This is another example of the complexity of this association.(AU)

Assuntos

RESUMO

La porfiria cutánea tarda (PCT) es considerada una manifestación extrahepática de la infección por el virusde la hepatitis C (HCV). La frecuencia de esta asociación es variable y no es claro el mecanismo por el cual el virus la desencadena. Se presenta un hombre de 47 años con hepatitis C, genotipo-1b, que durante el tratamiento con peg-interferón alfa-2b más ribavirinasin viremia detectable en las semanas 12, 24 y 48, en la semana 44 consulta por presentar lesiones dermatológicasfotosensibles. Con el diagnóstico presuntivo de PCT se realizó biopsia cutánea y el dosaje de porfirinasurinarias fue 4185 ug/24hs (vn: < 250). El análisis cromatográfico reveló el típico patrón de PCT, confirmando el diagnóstico. El gen HFE de la hemocromatosis mostró mutaciones de carácter heterocigoto (H63D y C282Y), asociación frecuente en los pacientescon sobrecarga de hierro y PCT. El tratamiento antiviral de la infección por HCV puede mejorar las manifestacionesde la PCT. La ocurrencia de novo de éstafue reportada recientemente durante el tratamiento de la hepatitis C crónica con interferón y ribavirina pero no hay casos relatados de aparición tardía de PCT enpacientes HCV con viremia no detectable. La presencia de la mutación del gen HFE y la posibilidad de unaumento de oferta de hierro por la acción hemolítica de la ribavirina podrían explicar un exceso de hierrocapaz de desencadenar la crisis de PCT. Tampoco sonconcluyentes los estudios con respecto al aumento o no de la concentración de hierro en hígado en pacientes con anemia que reciben ribavirina.

Porphyria cutanea tarda (PCT) is considered an extra-hepatic manifestation of HCV infection. The frequency of this association varies according to different authors and the mechanism by which the virus can trigger this disease is not yet clear. We present a 47-year-old-man with chronic hepatitis C genotype 1b who, during the treatment with peg-interferón alfa 2b plus ribavirina, with no detectable viremia at weeks 12th, 24th, and 48th, developed dermatological photosensitive lesions at week 44th. With a presumptive diagnosis of PCT a cutaneous/skin biopsy was performed as well as a porphyrin dosage with urine porphyirins of 4185 microg/24 hs (nv<250). The chromatographic analysis revealed the typical PCT pattern thus confirming the diagnosis. The hemochromatosis HFE gen evaluation showed heterozigotus character mutations (H63D and C282Y) a frequent association in patients with iron overload and PCT. The antiviral treatment of the HCV infection can improve the clinical-humoral manifestations of PCT. The novo occurrence of PCT was recently reported during chronic hepatitis C treatment with interferón and ribavirin, but no cases of late appearance of PCT in patients with no detectable viremia were reported. The mutation of the gen HFE in our patient and the hemolysis caused by ribavirin can be related to the development of the disease, but the iron overload because of ribavirin use is also controversial. This is another example of the complexity of this association.

Assuntos

RESUMO

In 1989, the main agent causing non A non B hepatitis was identified as a RNA virus of the flavivirus family, with several serotypes, and was denominated virus C. At the present moment, the knowledge about the infection features and diseases that it causes has expanded thanks to the availability of reliable laboratory techniques to detect the antibody and the virus. The prevalence of infection and the frequency of serotypes varies in different regions of the world. Chile is a country with a low prevalence. The detection of infected blood in blood banks has reduced the spreading of the disease. Other means of infection such as the use of intravenous drugs, hemodialysis and transplantation have acquired greater importance. Sexual, maternal and familial transmission is exceptional. Infected people develop an acute hepatitis, generally asymptomatic. Eighty percent remain with a chronic hepatic disease, that can be mild or progressive, evolving to cirrhosis or hepatic carcinoma. Chronic hepatitis, closely resembling an autoimmune disease, can be caused by the virus. Alcohol intake increases viral activity causing severe hepatic diseases, refractory to treatments. Several non hepatic diseases are associated to hepatitis C virus infection such as essential mixed cryoglobulinemia, mesangiocapillary glomerulonephritis, porphyria cutanea tarda, dysglobulinemias and probably type 2 diabetes mellitus. The only available treatment is interferon, that is successful in a minority of patients, frequently causing a transient improvement. The use of Ribaravine associated to interferon improve the effectiveness of therapy. Liver transplantation is the only therapy for severe hepatic disease. The use of new antiviral drugs should improve the prognosis of the disease.

Assuntos