Background
Bullous disease of dialysis, or 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 occur predominantly in sun-exposed skin—most often on the dorsal hands—of individuals treated for chronic renal failure with maintenance dialysis regimens. (See Presentation.) See the image below.
Hands of a transfusion-dependent patient on long-term hemodialysis. Several uremia-related cutaneous disorders are visible. The pigmentary alteration results from retained urochromes and hemosiderin deposition. The large bullae are consistent with either porphyria cutanea tarda or the bullous disease of dialysis. All nails show the distal brown-red and proximal white coloring of half-and-half nails.
This mechanobullous disorder has been observed in patients with end-stage renal disease who were treated with chronic ambulatory peritoneal dialysis or with hemodialysis. Typically, however, however, the condition develops only after several months or years of dialysis therapy. (See Presentation.)
Porphyrin levels in urine and stool are normal in patients with bullous disease of dialysis; plasma porphyrin levels are also normal or are most often only minimally elevated. Such findings in a patient would exclude the presence of a true porphyria, which, in anephric individuals, would result in grossly abnormal porphyrin values. (See Workup.) [3]
The cutaneous lesions of bullous disease of dialysis are cosmetically distressing and interfere with the use of the hands. They may be painful, become secondarily infected, and eventuate in scarring, but they are not life threatening. (See Treatment and Medication.)
Etiology
Because plasma porphyrin levels in individuals with chronic renal failure may be mildly elevated, [4] or occasionally high enough to overlap levels seen in porphyria patients with normal renal function, [5] porphyrin photosensitization may play a contributory role in some cases. It is not likely to be the primary cause, however, because many dialysis patients with similarly mild elevations of plasma porphyrin levels do not develop photocutaneous lesions.
Speculation that photosensitizers encountered during dialysis (eg, compounds emanating from plastic tubing) are responsible for the disorder remains unproven. The concomitant use of therapeutic agents with phototoxic potential (eg, furosemide) cannot be identified in most cases.
A possible, but unproven, cause of bullous disease of dialysis may be overproduction of porphyrins resulting from effects of high aluminum concentrations (from therapeutic or environmental sources) on enzymes of heme biosynthesis. [5] In 6 dialyzed patients with bullous dermatoses and high serum aluminum levels, Gafter et al found elevated plasma porphyrins approaching or surpassing those seen in true porphyria cutanea tarda. [5] Potential benefit from aluminum chelation with desferrioxamine in such cases was considered.
Epidemiology
The frequency of bullous disease of dialysis among dialysis populations in the United States has not been accurately determined but may be similar to that reported from several European surveys.
In a large French survey, 6 of 500 individuals who underwent hemodialysis were affected by the disease after between 2 and 54 months of dialysis. [6] Among 66 individuals who underwent hemodialysis for more than 10 years, 27% reported cutaneous fragility, and pseudoporphyria (presumably bullae) was noted in 13%. [7] In 2 additional French surveys, 16 of 100 [8] and 6 of 136 dialysis patients were affected. [9] Three patients with blistering were found among 70 patients at an Irish dialysis center. [10]
Race-, sex-, and age-related demographics
Although no race predilections have been reported for bullous disease of dialysis, 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.
Although some surveys have reported a male predominance for the disease, a higher female-to-male ratio has also been noted. In none of these surveys, however, was the male/female composition of the underlying population stated. Thus, the male-to-female data reported may reflect differences in the frequency with which men and women occurred in the source populations.
Most reported cases of bullous disease of dialysis have involved adults. However, this may reflect the predominance of older individuals with end-stage renal failure among populations treated with chronic dialysis regimens.
Patient Education
Advise patients of the role of sunlight in aggravating their propensity for blistering. Review measures for reduction of sunlight exposure and of mechanical stress to light-exposed skin. Instruct patients to use topical sunscreen formulations designed to reduce the transmission of long ultraviolet and visible light. In addition, inform patients that they should use gloves during activities likely to traumatize the hands
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Hands of a transfusion-dependent patient on long-term hemodialysis. Several uremia-related cutaneous disorders are visible. The pigmentary alteration results from retained urochromes and hemosiderin deposition. The large bullae are consistent with either porphyria cutanea tarda or the bullous disease of dialysis. All nails show the distal brown-red and proximal white coloring of half-and-half nails.
