[Porphyria cutanea tarda, pseudoporphyria and hemodialysis of chronic renal failure patients].
With the help of the observation of a bullous dermatosis in a 52-year-old male with cystic kidneys, terminal renal insufficiency and chronic hemodialytic treatment the authors adopt a definite attitude to the pathogenesis of this coincidence frequently reported on. Since the determination of porphyrin in the urine does not reflect the plasma porphyrin content in the combination, after sonography by means of a liver blind puncture and missing fluorescence of the liver bioptate in the long-wave ultraviolet light a porphyria cutanea tarda could be excluded. The bullous dermatosis observed is to be ascribed to the pseudoporphyrias (idiopathically; phototoxically by drugs). Renal insufficiency and long-term haemodialysis apparently on the one hand further the manifestation of genetically conditioned diseases of porphyria cutanea tarda, on the other hand there are references that the chronic renal insufficiency itself leads to the disturbance of the hemobiosynthesis at the level of the uroporphyrinogen decarboxylase and that plasma porphyrins bound to highly molecular proteins are not capable to dialysis. A classification of skin changes accompanied by vesicles in patients with chronic renal insufficiency who were haemodialysed is proposed.
D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications* / Q000628:therapy; D008107:Liver Diseases / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000175:diagnosis*; D011166:Porphyrins / Q000652:urine; D006435:Renal Dialysis; D012871:Skin Diseases / Q000175:diagnosis*
D011166:Porphyrins
Porphyria cutanea tarda, Pseudoporphyrie und Hämodialyse chronisch Niereninsuffizienter.