Bullous Disease of Dialysis Clinical Presentation

  • Author: Maureen B Poh-Fitzpatrick, MD; Chief Editor: William D James, MD   more...
 
Updated: Apr 3, 2012
 

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.

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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. Blistering limited to volar fingertips was observed in one case.[9] Healing of crusted erosions leaves atrophic scars. Milia, dyspigmentation, and hypertrichosis occur infrequently.

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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,[10] 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 etiologies[11] but remain unproven.

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Contributor Information and Disclosures
Author

Maureen B Poh-Fitzpatrick, MD  Professor Emerita of Dermatology and Special Lecturer, Columbia University College of Physicians and Surgeons; Professor of Medicine (Dermatology), University of Tennessee Health Science Center College of Medicine

Maureen B Poh-Fitzpatrick, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and New York Academy of Medicine

Disclosure: Clinuvel Pharmaceuticals, Ltd. Honoraria Consulting

Specialty Editor Board

Shyam Verma  MBBS, DVD, FAAD, Clinical Associate Professor, Department of Dermatology, University of Virginia; Adjunct Associate Professor, Department of Dermatology, State University of New York at Stonybrook, Adjunct Associate Professor, Department of Dermatology, University of Pennsylvania

Shyam Verma is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

References

  1. Gilchrest B, Rowe JW, Mihm MC Jr. Bullous dermatosis of hemodialysis. Ann Intern Med. Oct 1975;83(4):480-3. [Medline].

  2. Keczkes K, Farr M. Bullous dermatosis of chronic renal failure. Br J Dermatol. Nov 1976;95(5):541-6. [Medline].

  3. 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].

  4. Brivet F, Drüeke T, Guillemette J, Zingraff J, Crosnier J. Porphyria cutanea tarda-like syndrome in hemodialyzed patients. Nephron. 1978;20(5):258-66. [Medline].

  5. Chazot C, Chazot I, Charra B, et al. Functional study of hands among patients dialysed for more than 10 years. Nephrol Dial Transplant. 1993;8(4):347-51. [Medline].

  6. Griffon-Euvrard S, Thivolet J, Laurent G, et al. [Detection of pseudo-porphyria cutanea tarda in 100 hemodialyzed patients (author's transl)]. Dermatologica. 1977;155(4):193-9. [Medline].

  7. Amblard P, Cordonnier D, Reymond JL, Beani JC, Elsener M, Guffon MP. [Pseudo-pseudo-porphyria tarda in hemodialyzed patients]. Ann Dermatol Venereol. 1981;108(12):1019-20. [Medline].

  8. Gibson GE, McGinnity E, McGrath P, et al. Cutaneous abnormalities and metabolic disturbance of porphyrins in patients on maintenance haemodialysis. Clin Exp Dermatol. May 1997;22(3):124-7. [Medline].

  9. Shelley WB, Shelley ED. Blisters of the fingertips: a variant of bullous dermatosis of hemodialysis. J Am Acad Dermatol. Nov 1989;21(5 Pt 2):1049-51. [Medline].

  10. 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].

  11. 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].

  12. 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].

  13. 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].

  14. 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].

  15. 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].

  16. 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].

 
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