Manifestación de porfiria cutánea tardía en pacientes infectados con el virus de la inmunodeficiencia humana

摘要

Porphyrias are metabolic disorders caused by a decreased activity of one of the haem enzymes. Porphyria Cutanea Tarda (PCT) is due to subnormal activity of Uroporphyrinogen Decarboxylase (URO-D), and its value in erythrocytes allows differentiation between familiar and sporadic PCT, leading to increased levels of highly carboxylated porphyrins in liver, plasma and urine. Clinical symptoms include hyperpigmentation, hyperthricosis, blisters, skin photosensitivity and fragility. Factors such as alcohol, estrogens, polyhalogenated compounds, iron overload and infections with hepatitis viruses (HBV and HCV) and human immunodeficiency virus (HIV), have been implicated in its pathogenesis. All Argentine PCT-HIV cases studied from 1990 to 2004 were rewiewed. A group of 91 HIV positive subjects were diagnosed as PCT patients (20 to 50 years). Only 3 were women; 48 alcohol and drug abusers, 16 only heavy drinkers, 10 drug abusers and 41 were also coinfected with HCV. In 25 patients an overlapping of alcohol, drug abuse and HCV infection was found. In only 2 of the PCT-HIV studied URO-D was diminished. Treatment with low doses of chloroquine or the combined therapy (SAMe and chloroquine) is well tolerated, with normalization of clinical and biochemical signs. It can be concluded that HIV infected subjects should be considered a risk group, highly susceptible to developing Porphyria.