Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®


Craig A Elmets, MD
Section Editors
Jean-Claude Roujeau, MD
Maja Mockenhaupt, MD, PhD
Deputy Editor
Rosamaria Corona, MD, DSc


Pseudoporphyria is a bullous photodermatosis with the clinical and histologic features of porphyria cutanea tarda (PCT) in the absence of abnormalities in porphyrin metabolism [1]. Pseudoporphyria has been associated with medications, chronic renal failure and hemodialysis, and tanning beds. Patients typically present with skin fragility, bullae, milia, and scarring on the dorsum of the hands and other sun-exposed areas. Histologically, there is a noninflammatory subepidermal blister.

This topic will discuss the pathogenesis, clinical manifestations, diagnosis, and treatment of pseudoporphyria. PCT and other types of porphyrias associated with photosensitivity are discussed separately. (See "Porphyria cutanea tarda and hepatoerythropoietic porphyria: Clinical manifestations and diagnosis" and "Erythropoietic protoporphyria and X-linked protoporphyria".)


The incidence of pseudoporphyria is unknown. It occurs at any age and more frequently in women than in men. Pseudoporphyria has been reported in approximately 10 percent of children taking naproxen, a nonsteroidal antiinflammatory drug routinely used in the therapy of juvenile idiopathic arthritis. Among children treated with naproxen, risk factors for the development of pseudoporphyria include skin phototypes I or II (table 1), blue/gray eyes, and the concurrent use of chloroquine [2-4]. In one study of 363 hemodialysis patients, pseudoporphyria occurred in two (0.6 percent) [5].


Four factors have been implicated in the etiology of pseudoporphyria: ultraviolet (UV) radiation, medications, chronic renal failure and hemodialysis, and tanning beds.

UV radiation — Although the exact pathogenetic mechanism is unknown, drug-induced pseudoporphyria is considered a photodynamic phototoxic drug reaction. In this type of reaction, the photosensitizing compound, upon absorption of the appropriate wavelength radiation, changes to an excited state and reacts with oxygen to form reactive oxygen species (ROS) such as singlet oxygen, hydrogen peroxide, or superoxide anion. ROS can damage cell components such as nucleic acids, membranes, lipids, and proteins. Mediators of inflammation and inflammatory cells participate in tissue injury, including products of complement activation, proinflammatory cytokines, arachidonic acid metabolites, proteases, and polymorphonuclear leukocytes.


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Sep 2016. | This topic last updated: Feb 10, 2015.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
  1. Green JJ, Manders SM. Pseudoporphyria. J Am Acad Dermatol 2001; 44:100.
  2. De Silva B, Banney L, Uttley W, et al. Pseudoporphyria and nonsteroidal antiinflammatory agents in children with juvenile idiopathic arthritis. Pediatr Dermatol 2000; 17:480.
  3. Levy ML, Barron KS, Eichenfield A, Honig PJ. Naproxen-induced pseudoporphyria: a distinctive photodermatitis. J Pediatr 1990; 117:660.
  4. Schäd SG, Kraus A, Haubitz I, et al. Early onset pauciarticular arthritis is the major risk factor for naproxen-induced pseudoporphyria in juvenile idiopathic arthritis. Arthritis Res Ther 2007; 9:R10.
  5. Masmoudi A, Ben Hmida M, Mseddi M, et al. [Cutaneous manifestations of chronic hemodialysis. Prospective study of 363 cases]. Presse Med 2006; 35:399.
  7. Ramsay CA. Photosensitivity from nalidixic acid. Proc R Soc Med 1973; 66:747.
  8. Ramsay CA, Obreshkova E. Photosensitivity from nalidixic acid. Br J Dermatol 1974; 91:523.
  9. Frank J, Poblete-Gutierrez R. Porphyria. In: Dermatology, 2nd ed, Bolognia JL, Jorizzo JL, Rapini RP, et al (Eds), Mosby Elsevier, St. Louis 2008. p.648.
  10. Sharp MT, Horn TD. Pseudoporphyria induced by voriconazole. J Am Acad Dermatol 2005; 53:341.
  11. Kwong WT, Hsu S. Pseudoporphyria associated with voriconazole. J Drugs Dermatol 2007; 6:1042.
  12. Tolland JP, McKeown PP, Corbett JR. Voriconazole-induced pseudoporphyria. Photodermatol Photoimmunol Photomed 2007; 23:29.
  13. Dolan CK, Hall MA, Blazes DL, Norwood CW. Pseudoporphyria as a result of voriconazole use: a case report. Int J Dermatol 2004; 43:768.
  14. Lenfestey A, Friedmann D, Burke WA. Metformin-induced pseudoporphyria. J Drugs Dermatol 2012; 11:1272.
  15. Beer K, Applebaum D, Nousari C. Pseudoporphyria: discussion of etiologic agents. J Drugs Dermatol 2014; 13:990.
  16. Berghoff AT, English JC 3rd. Imatinib mesylate-induced pseudoporphyria. J Am Acad Dermatol 2010; 63:e14.
  17. Timmer-de Mik L, Kardaun SH, Kramer MH, et al. Imatinib-induced pseudoporphyria. Clin Exp Dermatol 2009; 34:705.
  18. Mahon C, Purvis D, Laughton S, et al. Imatinib mesylate-induced pseudoporphyria in two children. Pediatr Dermatol 2014; 31:603.
  19. Pérez NO, Esturo SV, Viladomiu Edel A, et al. Pseudoporphyria induced by imatinib mesylate. Int J Dermatol 2014; 53:e143.
  20. Sanz-Motilva V, Martorell-Calatayud A, Llombart B, et al. Sunitinib-induced pseudoporphyria. J Eur Acad Dermatol Venereol 2015; 29:1848.
  21. Lang BA, Finlayson LA. Naproxen-induced pseudoporphyria in patients with juvenile rheumatoid arthritis. J Pediatr 1994; 124:639.
  22. Girschick HJ, Hamm H, Ganser G, Huppertz HI. Naproxen-induced pseudoporphyria: appearance of new skin lesions after discontinuation of treatment. Scand J Rheumatol 1995; 24:108.
  23. Creemers MC, Chang A, Franssen MJ, et al. Pseudoporphyria due to naproxen. A cluster of 3 cases. Scand J Rheumatol 1995; 24:185.
  24. Burns DA. Naproxen pseudoporphyria in a patient with vitiligo. Clin Exp Dermatol 1987; 12:296.
  25. Diffey BL, Farr PM. Naproxen pseudoporphyria in a patient with vitiligo. Clin Exp Dermatol 1988; 13:207.
  26. Rivers JK, Barnetson RS. Naproxen-induced bullous photodermatitis. Med J Aust 1989; 151:167.
  27. Judd LE, Henderson DW, Hill DC. Naproxen-induced pseudoporphyria. A clinical and ultrastructural study. Arch Dermatol 1986; 122:451.
  28. Suarez SM, Cohen PR, DeLeo VA. Bullous photosensitivity to naproxen: "pseudoporphyria". Arthritis Rheum 1990; 33:903.
  29. Taylor BJ, Duffill MB. Pseudoporphyria from nonsteroidal antiinflammatory drugs. N Z Med J 1987; 100:322.
  30. Cummins R, Wagner-Weiner L, Paller A. Pseudoporphyria induced by celecoxib in a patient with juvenile rheumatoid arthritis. J Rheumatol 2000; 27:2938.
  31. LaDuca JR, Bouman PH, Gaspari AA. Nonsteroidal antiinflammatory drug-induced pseudoporphyria: a case series. J Cutan Med Surg 2002; 6:320.
  32. Cron RQ, Finkel TH. Nabumetone induced pseudoporphyria in childhood. J Rheumatol 2000; 27:1817.
  33. Checketts SR, Morgan GJ Jr. Two cases of nabumetone induced pseudoporphyria. J Rheumatol 1999; 26:2703.
  34. Meggitt SJ, Farr PM. Pseudoporphyria and propionic acid non-steroidal anti-inflammatory drugs. Br J Dermatol 1999; 141:591.
  35. Magro CM, Crowson AN. Pseudoporphyria associated with Relafen therapy. J Cutan Pathol 1999; 26:42.
  36. Krischer J, Scolari F, Kondo-Oestreicher M, et al. Pseudoporphyria induced by nabumetone. J Am Acad Dermatol 1999; 40:492.
  37. Varma S, Lanigan SW. Pseudoporphyria caused by nabumetone. Br J Dermatol 1998; 138:549.
  38. Al-Khenaizan S, Schechter JF, Sasseville D. Pseudoporphyria induced by propionic acid derivatives. J Cutan Med Surg 1999; 3:162.
  39. Antony F, Layton AM. Nabumetone-associated pseudoporphyria. Br J Dermatol 2000; 142:1067.
  40. O'Hagan AH, Irvine AD, Allen GE, Walsh M. Pseudoporphyria induced by mefenamic acid. Br J Dermatol 1998; 139:1131.
  41. Epstein JH, Tuffanelli DL, Seibert JS, Epstein WL. Porphyria-like cutaneous changes induced by tetracycline hydrochloride photosensitization. Arch Dermatol 1976; 112:661.
  42. Gilchrest B, Rowe JW, Mihm MC Jr. Bullous dermatosis of hemodialysis. Ann Intern Med 1975; 83:480.
  43. Harvey E, Bell CH, Paller AS, et al. Pseudoporphyria cutanea tarda: two case reports on children receiving peritoneal dialysis and erythropoietin therapy. J Pediatr 1992; 121:749.
  44. Glynne P, Deacon A, Goldsmith D, et al. Bullous dermatoses in end-stage renal failure: porphyria or pseudoporphyria? Am J Kidney Dis 1999; 34:155.
  45. Brivet F, Drüeke T, Guillemette J, et al. Porphyria cutanea tarda-like syndrome in hemodialyzed patients. Nephron 1978; 20:258.
  46. Griffon-Euvrard S, Thivolet J, Laurent G, et al. [Detection of pseudo-porphyria cutanea tarda in 100 hemodialyzed patients (author's transl)]. Dermatologica 1977; 155:193.
  47. Attia EA, Hassan SI, Youssef NM. Cutaneous disorders in uremic patients on hemodialysis: an Egyptian case-controlled study. Int J Dermatol 2010; 49:1024.
  48. Bencini PL, Montagnino G, Citterio A, et al. Cutaneous abnormalities in uremic patients. Nephron 1985; 40:316.
  49. Neergaard J, Nielsen B, Faurby V, et al. On the exudation of plasticizers from PVC haemodialysis tubings. Nephron 1975; 14:263.
  50. Schettler V, Wieland E, Methe H, et al. Oxidative stress during dialysis: effect on free radical scavenging enzyme (FRSE) activities and glutathione (GSH) concentration in granulocytes. Nephrol Dial Transplant 1998; 13:2588.
  51. Anderson CD, Rossi E, Garcia-Webb P. Porphyrin studies in chronic renal failure patients on maintenance hemodialysis. Photodermatol 1987; 4:14.
  52. Fevang SA, Kroon S, Skadberg Ø. Pseudoporphyria or porphyria cutanea tarda? Diagnostic and treatment difficulties. Acta Derm Venereol 2008; 88:426.
  53. Murphy GM, Wright J, Nicholls DS, et al. Sunbed-induced pseudoporphyria. Br J Dermatol 1989; 120:555.
  54. Wilson CL, Mendelsohn SS. Identical twins with sunbed-induced pseudoporphyria. J R Soc Med 1992; 85:45.
  55. Stenberg A. Pseudoporphyria and sunbeds. Acta Derm Venereol 1990; 70:354.
  56. Thomsen K. Solarium pseudoporphyria. Photodermatol 1989; 6:61.
  57. Kochs C, Mühlenstädt E, Neumann NJ, Hanneken S. [Solarium-induced pseudoporphyria and variegate porphyria as rare differential diagnoses of porphyria cutanea tarda]. Hautarzt 2009; 60:790.
  58. Maynard B, Peters MS. Histologic and immunofluorescence study of cutaneous porphyrias. J Cutan Pathol 1992; 19:40.
  59. Fung MA, Murphy MJ, Hoss DM, et al. The sensitivity and specificity of "caterpillar bodies" in the differential diagnosis of subepidermal blistering disorders. Am J Dermatopathol 2003; 25:287.
  60. Egbert BM, LeBoit PE, McCalmont T, et al. Caterpillar bodies: distinctive, basement membrane-containing structures in blisters of porphyria. Am J Dermatopathol 1993; 15:199.
  61. Schanbacher CF, Vanness ER, Daoud MS, et al. Pseudoporphyria: a clinical and biochemical study of 20 patients. Mayo Clin Proc 2001; 76:488.
  62. Dabski C, Beutner EH. Studies of laminin and type IV collagen in blisters of porphyria cutanea tarda and drug-induced pseudoporphyria. J Am Acad Dermatol 1991; 25:28.
  63. Motley RJ. Pseudoporphyria due to dyazide in a patient with vitiligo. BMJ 1990; 300:1468.
  64. Wallace CA, Farrow D, Sherry DD. Increased risk of facial scars in children taking nonsteroidal antiinflammatory drugs. J Pediatr 1994; 125:819.
  65. Horner ME, Alikhan A, Tintle S, et al. Cutaneous porphyrias part I: epidemiology, pathogenesis, presentation, diagnosis, and histopathology. Int J Dermatol 2013; 52:1464.
  66. Schulenburg-Brand D, Katugampola R, Anstey AV, Badminton MN. The cutaneous porphyrias. Dermatol Clin 2014; 32:369.
  67. Poh-Fitzpatrick MB, Sosin AE, Bemis J. Porphyrin levels in plasma and erythrocytes of chronic hemodialysis patients. J Am Acad Dermatol 1982; 7:100.
  68. Sebaratnam DF, Murrell DF. Bullous systemic lupus erythematosus. Dermatol Clin 2011; 29:649.
  69. Massone C, Ambros-Rudolph CM, Di Stefani A, Müllegger RR. Successful outcome of haemodialysis-induced pseudoporphyria after short-term oral N-acetylcysteine and switch to high-flux technique dialysis. Acta Derm Venereol 2006; 86:538.
  70. Tremblay JF, Veilleux B. Pseudoporphyria associated with hemodialysis treated with N-acetylcysteine. J Am Acad Dermatol 2003; 49:1189.
  71. Vadoud-Seyedi J, de Dobbeleer G, Simonart T. Treatment of haemodialysis-associated pseudoporphyria with N-acetylcysteine: report of two cases. Br J Dermatol 2000; 142:580.
  72. Cooke NS, McKenna K. A case of haemodialysis-associated pseudoporphyria successfully treated with oral N-acetylcysteine. Clin Exp Dermatol 2007; 32:64.
  73. Kasuya A, Hashizume H, Hirakawa S, Tokura Y. Haemodialysis-induced pseudoporphyria successfully treated with glutathione. Eur J Dermatol 2012; 22:137.
  74. Guiotoku MM, Pereira Fde P, Miot HA, Marques ME. Pseudoporphyria induced by dialysis treated with oral N-acetylcysteine. An Bras Dermatol 2011; 86:383.
  75. Azak A, Yenigün E, Koçak G, et al. Pseudoporphyria in a hemodialysis patient successfully treated with oral glutamine. Hemodial Int 2013; 17:466.