Abstract
Porphyria cutanea tarda is a disease characterized by skin lesion, hepatic disorder, diabetes mellitus, and increase of serum iron. The disease is said to be caused by hereditary enzyme deficiency decreased activity of uroporphyrinogen decarboxylase (UD) J and manifested when acquired factors are also present. Our case is a 56-years-old male whose acquired factor may be the intake of a large amount of alcohol over many years. As the skin lesion, facial hypertrichosis, skin pigmentation, bulla formation, erosion, scar and skin incrassation were observed, while no difference from the control was found in the photosensitivity test with 400 nm UV. In PAS stain of skin tissue for biopsy incrassation of basement membrane and of vascular wall, and PAS positive substance around them were observed. Fluorescent antibody method showed deposition mainly of IgG on these areas. Hepatic function is slightly disordered; deposition of hemosiderin and deposition of porphyrin body in the hepatic tissue was observed in biopsy of the liver by laparoscopy and by 400 nm UV irradiation, respectively. In addition UD activity in hepatic tissue was remarkably decreased, which is said to be the cause. Diabetes mellitus was recognized while insulinogenic index (1 hr) was as low as 0.1, and therefore the disease is difficult to be considered secondary to the hepatic disorder. Both reserve iron and serum iron were increased; urine was reddish brown; Δ-Ala level in urine was normal; levels of uroporphyrin and coproporphyrin were high, the former being predominant. At present the patient is being subjected to blood-letting; the clinical symptoms have not been improved yet.
