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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2013  |  Volume : 24  |  Issue : 1  |  Page : 121-123
Porphyria cutanea tarda in a hemodialysis patient with hepatitis C virus: Efficacy of treatment with multiple phlebotomies and erythropoietin

1 Department of Nephrology, Dialysis and Renal Transplantation, Military Training Hospital Mohamed V, Rabat, Morocco
2 Hemodialysis Center, The First Medical-Surgical Center, Agadir, Morocco

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Date of Web Publication22-Jan-2013

How to cite this article:
Hamzi MA, Alayoud A, Asseraji M, Akhmouch I, Oualim Z. Porphyria cutanea tarda in a hemodialysis patient with hepatitis C virus: Efficacy of treatment with multiple phlebotomies and erythropoietin. Saudi J Kidney Dis Transpl 2013;24:121-3

How to cite this URL:
Hamzi MA, Alayoud A, Asseraji M, Akhmouch I, Oualim Z. Porphyria cutanea tarda in a hemodialysis patient with hepatitis C virus: Efficacy of treatment with multiple phlebotomies and erythropoietin. Saudi J Kidney Dis Transpl [serial online] 2013 [cited 2020 Jun 2];24:121-3. Available from: http://www.sjkdt.org/text.asp?2013/24/1/121/106305
To the Editor,

Porphyria cutanea tarda (PCT) is a metabolic disease with cutaneous expression. It is caused by a deficiency of the enzyme uroporphyrinogen decarboxylase (UPD), which results in accumulation of an endogenous photo-sensitizing substance, uroporphyrin. PCT exists in three forms: two inherited autosomal-dominant forms (types II and III) and a sporadic acquired one (a pure hepatic form, type I). [1] The sporadic form, the most common, is associated with multiple factors of hepatotoxicity. [2] PCT has been described in patients receiving maintenance hemodialysis (HD). [3]

We herewith report a case of PCT in a patient on HD who also had infection with the hepatitis C virus (HCV). The patient was treated with removal of small amounts of blood along with administration of a higher dose of erythropoietin. A 60-year-old woman undergoing HD for end-stage renal disease for 27 years presented with recurrent vesiculo-bullous skin eruption of both hands of six months duration. She was positive for anti-HCV antibody for many years. There was no history of similar cases in the family or alcohol ingestion. Her medications included alfacalcidol and calcium carbonate. Clinical examination showed fragility of the skin and bullae on the back of both hands [Figure 1]. Small ulcerative lesions and milia were also noted. The remaining part of the physical examination was normal. A skin biopsy specimen revealed regular epidermal papillae with discrete non-specific interstitial dermatitis without any vascular anomalies.
Figure 1: Bullous lesions on the back of the hand in the study patient.

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The diagnosis of PCT was confirmed by the determination of elevated levels of porphyrins in the blood: uroporphyrin was 166 nmol/L (normal = 0) and coproporphyrin was 67 nmol/L (normal <150). In the feces, uroporphyrin was 367 nmol/g dry weight (normal <150) and coproporphyrin was 897 nmol/g dry weight (normal <750). Serological tests for hepatitis B and human immunodeficiency virus (HIV) were negative. The serum iron status was normal: serum iron was 87 μg/dL (normal = 50-160) and ferritin was 267 ng/mL (normal = 200-500).

Other laboratory data were normal, including normal hemoglobin level (12.7 g/dL with 100 μg/month of Methoxy Polyethylene Glycol-Epoetin Beta (Mircera®)) and normal liver enzymes.

The patient was treated with a higher dose of erythropoietin (100-200 μg of Mircera per month) associated with multiple small amounts of bleeding (300 mL per month). Measures like photo-protection (avoidance of sunlight, protective clothing and opaque sun screens) and avoidance of trauma or inducing drugs were followed.

With this treatment, the patient's hemoglobin remained stable (10.6-12.2 g/dL). Serum ferritin at three months was 126 μg/mL. The evolution of the skin lesion was favorable. All bullous lesions and erosions healed with minor scarring, and no new lesion had occurred after seven months of follow-up.

The development of skin lesions in PCT is the result of the interaction between solar radiation and accumulated porphyrins in the skin. The clinical expression requires the association of an additional risk factor: iron over-load, viral infection (hepatitis B or C, HIV), chronic alcoholism, estrogen therapy or HD. Genetic factors are associated with an increased risk of occurrence of sporadic PCT from three- to 48 times.

Isolated cases of PCT in HD patients with HCV have been reported. [3],[4],[5] Infection with the HCV occurs due to reduction of the UPD enzymatic activity and also through an immune mechanism. [6]

In patients on HD, several patho-physiological mechanisms are proposed: UPD inhibition due to nitrogen retention, loss of urinary excretion and improper clearance by dialysis of porphyrins (high molecular weight substances). Cases of PCT in patients on peritoneal dialysis have also been described. [7] The diagnosis is based on several criteria: PCT is often expressed as skin fragility with blisters and erosions of the uncovered parts. The lesions are provoked by even minor trauma. They persist for several weeks before giving way to scarring scabs. Hypertrichosis and hyperpigmentation of the photo-exposed areas are also common. Histology is non-specific. It usually shows sub-epidermal bubbles with low inflammation. The presence of hyaline deposits in vessel walls is common. Skin biopsy is not necessary for etiological diagnosis.

Laboratory tests show an elevation of porphyrins in plasma, urine and feces. In sporadic PCT, the activity of UPD is reduced only in the liver. The UPD is normal in erythrocytes while it is frequently (but not constantly) decreased in hereditary forms. Complete blood count, liver function tests, serum iron, ferritin and hepatitis B, C and HIV serologies should be performed. [1],[8] The transferrin saturation coefficient, serum iron and serum ferritin are frequently increased.

In HD patients, the main differential diagnosis is pseudo-porphyria, clinically identical to PCT, but the assay of porphyrins (which are increased only in PCT) can distinguish the two.

In anuric patients, the determination of urinary porphyrins is not feasible thus necessitating the determination of blood and fecal porphyrins. This should be interpreted with reference to specific standards in this population. Indeed, 60% of the dialysis patients have moderately increased plasma porphyrins without clinical signs indicating PCT. [9]

The aim of treatment is to promote the elimination of accumulated porphyrins and reduce the iron overload observed in most patients. Besides preventive measures (photo protection, avoidance of alcohol ingestion and hepatotoxic or photo-sensitizer substance), several therapies have been proposed, including anti-malarial drugs, phlebotomy and deferoxamine.

Phlebotomy is an effective treatment in cases with hemochromatosis or when the serum iron and ferritin are slightly elevated. [2] Phlebotomies (350-500 mL each week) are continued until normalization of iron stores occurs, ensuring that the hemoglobin levels are greater than 11 g/dL.

In patients on chronic HD, co-existing anemia does not allow high volume bleed, although small repetitive phlebotomies are possible. [10] Erythropoietin, by mobilizing iron stores, can be effective, either alone or in association with bleeding. Anti-malarial drugs are ineffective or even dangerous. They form a hydrosoluble complex with porphyrins, facilitating their excretion, but are not eliminated by dialysis. Furthermore, they can lead to hepato-toxicity. [1] Deferoxamine may be useful at a dose of 2-4 g intravenously after each dialysis. [11]

Plasma exchange is a possible but risky and expensive alternative. [12] Kidney transplantation remains a last resort treatment in refractory forms. In patients with HCV infection, remissions have been reported with treatment by interferon alfa. [13]

Our patient was treated by small phlebotomies (300 mL per month) and increased doses of erythropoietin. The disease appears to be stabilized without any new lesion after a follow-up of five months. A full recovery will require a longer time.

   References Top

1.Bessis D. porphyria cutanea tarda.. Ann Dermatol Vénéréol 2001;128:1068-74.   Back to cited text no. 1
2.Bulaj ZJ, Phillips JD, Ajioka RS, et al. Hemochromatosis genes and other factors contributing to the pathogenesis of porphyria cutanea tarda. Blood 2000;95:1565-71.  Back to cited text no. 2
3.Albalate M, Farinas MC, Octavio JG, Perez JH, Goicoechea M, Caramelo C. Development of porphyria cutanea tarda in a hemodialysis patient after reactivation of hepatitis C virus infection. Nephron 2001;88:170-3.  Back to cited text no. 3
4.Lee PK, Abrahams I, Bickers DR. Porphyria cutanea tarda occuring in a patient with chronic renal failure, systemic lupus erythematous and chronic hepatitis C infection treated with hemodialysis. Cutis 1999;64:237-9.  Back to cited text no. 4
5.Albitar S, Bourgeon B, Chuet C. Porphyria cutanea tarda, hepatitis C infection and iron overload in a patient in hemodialysis. Am J Med 1999;106:266-7.  Back to cited text no. 5
6.Cribier B, Petiau P, Stoll-keller F, et al. Porphyria cutanea tarda and hepatitis C virus infection. Clinical and virological study. Ann Dermatol Venereol 1996;123:200-2.  Back to cited text no. 6
7.Kelly MA, O'Rourke KD. Treatment of Porphyria cutanea tarda with phlebotomy in a patiemt on peritoneal dialysis. J Am Acad Dermatol 2001;44(2 suppl):336-8.  Back to cited text no. 7
8.Lecha M, Herrero C, Ozalla D. Diagnosis and treatment of the hepatic porphyrias. Dermatol Ther 2003;16:65-72.  Back to cited text no. 8
9.Glynne P, Deacon A, Goldsmith D, Pusey C, Clutterbuck E. Bullous dermatoses in end-stage renal failure: Porphyria or pseudoporphyria? Am J Kidney Dis 1999;34:155-60.  Back to cited text no. 9
10.Poux JM, Demontis R,Cadranel JF,Ghazali A, Fievet P, Nordmann Y. Porphyria cutanea tarda in a dialyzed patient with hepatitis C virus infection: Dramatic efficacy of small repeated phlebotomies. Am J Med 1997;103 :163-4.  Back to cited text no. 10
11.Stockenhuber F, Kurz R, Grimm G, Moser G, Balcke R. Successful treatment of hemodialysis-related porphyria cutanea tarda with deferoxamine. Nephron 1990;55:321-4.  Back to cited text no. 11
12.Disler P, Day R, Burman N, Blekkenhorst G, Eales L. Treatment of hemdialysis-related porphyria cutanea tarda with plasma exchange. Am J Med 1982;72:989-93.  Back to cited text no. 12
13.Sheikh MY, Wright RA, Buruss JB. Dramatic resolution of skin lesions associated with porphyria cutanea tarda after interferon alpha therapy in a case of chronic hepatitis C. Dig Dis Sci 1998;43:529-33.  Back to cited text no. 13

Correspondence Address:
Mohamed Amine Hamzi
Department of Nephrology, Dialysis and Renal Transplantation, Military Training Hospital Mohamed V, Rabat
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DOI: 10.4103/1319-2442.106305

PMID: 23354206

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