Bullous Disease of Dialysis Workup

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

Laboratory Studies

To exclude a true porphyria, a plasma or serum porphyrin assay is recommended. The test result will be grossly abnormal in the case of any porphyria in an individual with compromised renal function. Fecal porphyrin analyses may also reveal increased porphyrin excretion in dialyzed patients with true porphyria.[12]

Next

Histologic Findings

Light microscopic examination of a biopsy of a blister reveals a subepidermal bulla indistinguishable from that of a true porphyria, with edematous dermal papillae and a scant perivascular lymphocytic infiltrate.[1]

Ultrastructural studies show thickening of the dermal venular walls and dermoepidermal junction due to replicated basal laminae, hypogranulated mast cells, and granulofilamentous hyaline masses in the dermal connective tissue that appear to be secreted by adjacent fibroblasts.[13]

Direct immunofluorescence examinations have revealed the presence of immunoglobulin G and, inconsistently, of immunoglobulin A, immunoglobulin M, fibrin, and complement around the dermal venules, with immunoglobulin G and complement sporadically noted at the dermoepidermal junction or bulla floor.[1, 6, 14]

Previous
Proceed to Treatment & Management
 
 
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].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.