title: Successful outcome of haemodialysis-induced pseudoporphyria after short-term oral N-acetylcysteine and switch to high-flux technique dialysis.
abstract: Pseudoporphyria is a blistering disease with skin fragility and shallow scarring that clinically and histopathologically closely resembles porphyria cutanea tarda. The two conditions can be distinguished by porphyrin levels that typically are elevated in porphyria cutanea tarda, but not or only slightly in pseudoporphyria. Pseudoporphyria can be induced by various medications (e.g. non-steroidal anti-inflammatory drugs, antibiotics, diuretics, retinoids), intense UV(A) exposure, or haemodialysis. Treatment of haemodialysis-associated pseudoporphyria is not yet standardized. We report here a 65-year-old male patient with chronic renal failure due to Waldenström's macroglobulinaemia who was treated with conventional 3 times/week haemodialysis. He developed blistering skin changes on both hands, which were diagnosed as pseudoporphyria based on clinical, histopathological, and laboratory findings, and could be successfully managed with initial oral N-acetylcysteine and a switch from low-flux to high-flux membrane haemodialysis. The beneficial effect of the high-flux membrane technique in haemodialysis-associated pseudoporphyria has not been previously reported.
authors.affiliation: Department of Dermatology, Medical University of Graz, Austria. Cesare.Massone@klinikum-graz.at
authors.forename: Cesare
authors.initials: C
authors.lastname: Massone
authors.forename: Christina M
authors.initials: CM
authors.lastname: Ambros-Rudolph
authors.forename: Alessandro
authors.initials: A
authors.lastname: Di Stefani
authors.forename: Robert R
authors.initials: RR
authors.lastname: Müllegger
mesh_terms: D000111:Acetylcysteine / Q000627:therapeutic use*; D000284:Administration, Oral; D000368:Aged; D000998:Antiviral Agents / Q000627:therapeutic use*; D006229:Hand Dermatoses / Q000209:etiology / Q000628:therapy*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000628:therapy; D008297:Male; D011164:Porphyrias / Q000209:etiology / Q000628:therapy*; D006435:Renal Dialysis / Q000009:adverse effects* / Q000379:methods*
chemical_list: D000998:Antiviral Agents; D000111:Acetylcysteine