A Porphyria Cutanea Tarda-like Distribution Pattern of Porphyrins in Plasma, Hemodialysate, Hemofiltrate, and Urine of Patients on Chronic Hemodialysis

Abstract

The porphyrin investigations of plasma, dialysate, hemofiltrate, and urine in 50 chronic renal patients without bullae undergoing hemodialysis imply that a hyperporphyria exists. All 50 dialysis patients without blisters had significantly elevated plasma porphyrins with a mean of 27.4 micrograms/liter (SD: 10.0). Normal values were established in 51 nonporphyric ambulatory patients with normal renal function. The mean plasma porphyrin content in this group was 10.6 micrograms/liter (SD: 1.7). Porphyrin values in dialysates of 41 patients with chronic renal failure ranged from 2.6-6.3 micrograms/liter (mean, 3.9 micrograms/liter) and were much lower than the porphyrin content in hemofiltrates of 9 patients which ranged from 10-27 micrograms/liter with a mean of 20 micrograms/liter. In addition, plasma, hemofiltrate, and urine of 5 dialysis patients with blisters and porphyria cutanea tarda (PCT) were investigated. The plasma values were extremely elevated and exceeded those of PCT patients with normal renal function. Whether the dermatologic manifestations of chronic hemodialysis can be interpreted as a "forme fruste" of PCT should be subjected to further investigations.

Plasma Porphyrins in Chronic Renal Failure

“…Seubert et al [14] found that uroporphyrin I was the main isomer in patients with CRF, unlike Day and Eales [5] who found mainly isomer III. We could only detect uroporphyrin I and not uroporphyrin III in the plasma of our patients.…”

Laboratory Investigation of the Porphyrias

“…Only small amounts of intermediates are lost from the pathway and excreted either unchanged or after oxidation to porphyrins, the (2), PBG-deaminase (3). uroporphyrinogen-Hl synthase (4), uroporphyrinogen decarboxylase (5), coproporphyrinogen oxidase (6), protoporphyrinogen oxidase (7) and ferrochelatase (8). In normal circumstances, less than 1% 0/ hydroxymethylbilane is cyclized non-enzymatically to uroporphyrinogen I.…”

Pseudoporphyria cutanea tarda Induced by Furosemide in a Patient Undergoing Peritoneal Dialysis

“…Nalidixic acid [5]dyazide [18]tetracycline [14], chlortalidone [14]bumetanide [14], amiodarone [14], etretinate [14], isotretinoin [19], pyridoxine [14], cyclosporin [20]5-fluorouracil [21]hemodialysis [1, 4, 9, 10]or peritoneal dialysis [9, 10, 15]alone have also been reported to induce PP [14, 15]. Drug-induced PP is interpreted by some authors as a phototoxic-mediated disorder [12, 14].…”

“…This entity is characterized by the sudden occurrence of blisters in the UV-exposed skin and an increased skin fragility [2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13]. The etiology of the disease is not yet clarified.…”