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Bullous Disease of Dialysis
Updated: Dec 12, 2008
Introduction
Background
Bullous dermatosis of dialysis is a syndrome of cutaneous fragility and blistering.1,2 The skin lesions clinically and histologically resemble those of porphyria cutanea tarda. Lesions predominantly occur in sun-exposed skin, most often on the dorsal hands, of individuals treated for chronic renal failure with maintenance dialysis regimens. This mechanobullous disorder has been observed in end-stage renal disease patients treated with chronic ambulatory peritoneal dialysis and with hemodialysis. Porphyrin levels in urine and stool are normal. Plasma porphyrin levels are normal or only minimally elevated, thus excluding a true porphyria, which, in anephric individuals, would result in grossly abnormal values.3
Pathophysiology
Blistering and mechanical fragility of skin subjected to sunlight and incidental trauma typically begin only after several months or years of dialysis therapy.
Frequency
United States
The frequency of this disorder among dialysis populations in the United States has not been accurately determined but may be similar to that reported from several European surveys.
International
In a large French survey, 6 of 500 individuals who underwent hemodialysis were affected.4 Among 66 individuals who incurred hemodialysis for more than 10 years in France, 27% reported cutaneous fragility and pseudoporphyria (presumably bullae) was noted in 13%.5 In 2 additional French surveys, 16 of 1006 and 6 of 1367 dialysis patients were affected. Three patients with blistering were found among 70 patients at an Irish dialysis center.8
Mortality/Morbidity
These cutaneous lesions are cosmetically distressing and interfere with use of the hands. They may be painful, become secondarily infected, and eventuate in scarring, but are not life threatening.
Race
Although no racial predilections have been reported, individuals with less melanin pigmentation of the skin have less natural photoprotection and may be more likely to develop dialysis-related cutaneous fragility and blistering.
Sex
Although some surveys report a predominance of males, a higher female-to-male ratio has also been noted. In none of these surveys was the sex composition of the underlying population stated; thus, the male-to-female data reported may be reflections of different sex frequencies in their source populations.
Age
Most reported cases have involved adults; however, this may reflect the predominance of older individuals with end-stage renal failure among populations treated with chronic dialysis regimens.
Clinical
History
Individuals with chronic renal failure who are afflicted with bullous dermatosis of dialysis typically develop these lesions only after months to years of maintenance dialysis regimens. The lesions are more florid after sunlight exposure; however, patients often are unaware of the role of sunlight in evoking the lesions since they do not note discomfort in the skin during the exposure.
Physical
Vesicles and bullae filled with clear or hemorrhagic fluid and exudative erosions occur chiefly on the dorsal hands, although the scalp, face, and neck also may be affected. Healing of crusted erosions leaves atrophic scars. Milia, dyspigmentation, and hypertrichosis occur infrequently.
Causes
The etiology of bullous dermatosis of dialysis remains unclear, although the propensity for sunlight aggravation of the bullae and fragility suggests a phototoxic mechanism.
Because plasma porphyrin levels in individuals with chronic renal failure may be mildly elevated,9 porphyrin photosensitization might play a contributory role in some cases. However, porphyrin photosensitization is not likely to be the primary cause because many dialysis patients with similarly mild elevations of plasma porphyrin levels do not develop photocutaneous lesions. Speculations that photosensitizers encountered during dialysis (eg, compounds emanating from plastic tubing) are responsible remain unproven. Concomitant use of therapeutic agents with phototoxic potential (eg, furosemide) cannot be identified in most cases. Effects of high aluminum concentrations from therapeutic or environmental sources on enzymes of heme biosynthesis, leading to overproduction of porphyrins, have been suggested as possible etiologies10 but remain unproven.
More on Bullous Disease of Dialysis |
 Overview: Bullous Disease of Dialysis |
| Differential Diagnoses & Workup: Bullous Disease of Dialysis |
| Treatment & Medication: Bullous Disease of Dialysis |
| Follow-up: Bullous Disease of Dialysis |
| References |
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References
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Keczkes K, Farr M. Bullous dermatosis of chronic renal failure. Br J Dermatol. Nov 1976;95(5):541-6. [Medline].
Poh-Fitzpatrick MB, Bellet N, DeLeo VA, Grossman ME, Bickers DR. Porphyria cutanea tardia in two patients treated with hemodialysis for chronic renal failure. N Engl J Med. Aug 10 1978;299(6):292-4. [Medline].
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Chazot C, Chazot I, Charra B, Terrat JC, Vanel T, Calemard E, et al. Functional study of hands among patients dialysed for more than 10 years. Nephrol Dial Transplant. 1993;8(4):347-51. [Medline].
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Gibson GE, McGinnity E, McGrath P, Carmody M, Walshe J, Donohoe J, et al. Cutaneous abnormalities and metabolic disturbance of porphyrins in patients on maintenance haemodialysis. Clin Exp Dermatol. May 1997;22(3):124-7. [Medline].
Poh-Fitzpatrick MB, Sosin AE, Bemis J. Porphyrin levels in plasma and erythrocytes of chronic hemodialysis patients. J Am Acad Dermatol. Jul 1982;7(1):100-4. [Medline].
Gafter U, Mamet R, Korzets A, Malachi T, Schoenfeld N. Bullous dermatosis of end-stage renal disease: a possible association between abnormal porphyrin metabolism and aluminium. Nephrol Dial Transplant. Sep 1996;11(9):1787-91. [Medline].
Perrot H, Germain D, Euvrard S, Thivolet J. Porphyria cutanea tarda-like dermatosis by hemodialysis. Ultrastructural study of exposed skin. Arch Dermatol Res. Aug 22 1977;259(2):177-85. [Medline].
Thivolet J, Euvrard S, Perrot H, Moskovtchenko JF, Claudy A, Ortonne JP. [Pseudo-late onset cutaneous parphyria in haemodialysis patients. Clinical and histological features. 9 cases (author's transl)]. Ann Dermatol Venereol. Jan 1977;104(1):12-7. [Medline].
Vadoud-Seyedi J, de Dobbeleer G, Simonart T. Treatment of haemodialysis-associated pseudoporphyria with N-acetylcysteine: report of two cases. Br J Dermatol. Mar 2000;142(3):580-1. [Medline].
Cooke NS, McKenna K. A case of haemodialysis-associated pseudoporphyria successfully treated with oral N-acetylcysteine. Clin Exp Dermatol. Jan 2007;32(1):64-6. [Medline].
Glynne P, Deacon A, Goldsmith D, Pusey C, Clutterbuck E. Bullous dermatoses in end-stage renal failure: porphyria or pseudoporphyria?. Am J Kidney Dis. Jul 1999;34(1):155-60. [Medline].
Further Reading
Keywords
bullous disease of dialysis, bullous dermatosis of dialysis, dialysis-related pseudoporphyria, cutaneous fragility, blistering, porphyria cutanea tarda, end-stage renal disease, ESRD, dialysis complications, adverse effects of dialysis, end stage renal disease, end-stage kidney disease, blisters
