par Barbier, Pierre;Simonis, Andrée ;Dourov, Nicolas ;Duret, Raymond
Référence Archives belges de dermatologie, 30, 2, page (59-66)
Publication Publié, 1974
Article révisé par les pairs
Résumé : The authors study 18 cases of porphyria cutanea tarda and 3 cases of porphyria variegata. After recalling the metabolism of porphyrins, they describe the clinical, biological, endoscopical aspects of the disease, and its optical and electron microscopical characteristics. Elevation of sideremia, BSP retention and a macroscopically dark liver seen at laparoscopy are frequently found. Histological criteria are autofluorescence of hepatocytes seen with U.V. light, steatosis and presence of granules of intrahepatocyte pigment. Electron microscopy shows inclusions of pigment, of fatty vacuoles and of crystalline intramitochondrial inclusions. All these results are studied and compared with the literature.