Bullous Disease of Dialysis
- Author: Maureen B Poh-Fitzpatrick, MD; Chief Editor: William D James, MD more...
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.
Epidemiology
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 100[6] and 6 of 136[7] 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.
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