Correspondence

Renal Transplantation for Porphyria Cutanea Tarda

N Engl J Med 1997; 336:811March 13, 1997

Article

To the Editor:

Porphyria cutanea tarda can occur in patients with chronic renal failure who are treated with hemodialysis.1,2 These patients are probably predisposed to porphyria cutanea tarda because of a preexisting decrease in hepatic uroporphyrinogen activity.3 Iron overload due to renal failure further decreases enzyme activity, leading to clinical disease.

The treatment options are limited for patients with chronic renal failure who have porphyria cutanea tarda. Because porphyrins are bound with high affinity to plasma proteins, they are not dialyzed well.4 Chloroquine cannot be given because the complexes it forms with porphyrins are not filtered by hemodialysis, and the presence of anemia secondary to renal failure may preclude phlebotomy. Treatment with erythropoietin in conjunction with phlebotomy may be beneficial.5 We describe a patient with porphyria cutanea tarda and renal failure who did not respond to treatment with erythropoietin plus phlebotomy but had a complete remission after renal transplantation.

A 40-year-old man with renal failure due to drug abuse who had been treated with hemodialysis for one year was referred to the dermatology clinic in September 1990 because of a one-year history of cutaneous fragility and blisters on his arms and hands. Physical examination revealed tense blisters on the backs of both hands and forearms, with milia and anterior displacement of the temporal hairline. The patient's erythrocyte free porphyrin concentration was 120 μg per deciliter (normal, <50), and the plasma total porphyrin concentration was 220 μg per deciliter (normal, <1). On fluorescence analysis, the plasma porphyrin spectrum showed a pattern consistent with the presence of porphyria cutanea tarda.

The patient was treated for 11 months with erythropoietin plus weekly phlebotomy to decrease iron stores, with little clinical or laboratory change. Cadaveric renal transplantation was then performed. The patient's serum creatinine concentration decreased from 8.9 mg per deciliter preoperatively to 2.3 mg per deciliter on postoperative day 31, and his plasma porphyrin concentration decreased from 69 to 2 μg per deciliter. His cutaneous signs and symptoms resolved almost completely over the subsequent six months.

In patients with porphyria cutanea tarda who are currently on hemodialysis and who do not respond to other therapies, renal transplantation should be considered as an effective and potentially curative treatment.

Shannon Ewing
David L. Crosby, M.D.
Medical College of Wisconsin, Milwaukee, WI 53226

5 References

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   Stevens BR, Fleischer AB Jr, Piering F, Crosby DL. Porphyria cutanea tarda in the setting of renal failure: response to renal transplantation. Arch Dermatol 1993;129:337-339
   CrossRef | Web of Science | Medline

2. 2

   Garcia Parrilla J, Ortega R, Pena ML, et al. Porphyria cutanea tarda during maintenance haemodialysis. BMJ 1980;280:1358-1358
   CrossRef | Web of Science | Medline

3. 3

   Elder GH, Lee GB, Tovey JA. Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda. N Engl J Med 1978;299:274-278
   Full Text | Web of Science | Medline

4. 4

   Day RS, Eales L. Porphyrins in chronic renal failure. Nephron 1980;26:90-95
   CrossRef | Medline

5. 5

   Anderson KE, Goeger DE, Carson RW, Lee S-MK, Stead RB. Erythropoietin for the treatment of porphyria cutanea tarda in a patient on long-term hemodialysis. N Engl J Med 1990;322:315-317[Erratum, N Engl J Med 1990;322:1616.]
   Full Text | Web of Science | Medline

Citing Articles (1)

Citing Articles

1. 1

   Erich Köstler, Uwe Wollina. (2005) Therapy of porphyria cutanea tarda. Expert Opinion on Pharmacotherapy 6:3, 377-383
   CrossRef