Pseudoporphyria E80.25

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 26.03.2024

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Definition
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Polyetiologic, blistering skin disease on sun-exposed skin areas, which leads to a porphyria cutanea tarda-like skin appearance without pathologic porphyrin chemistry. It is characterized by the formation of blisters after bagel injuries, possibly also after sun exposure with subsequent ulcers, hyperkeratosis and scarring.

Occurrence/Epidemiology
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haemodialysis (in about 16% of patients). Medicines: in 10-12% of children treated with naproxen.

Etiopathogenesis
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Mostly in chronic renal insufficiency, hemodialysis patients or kidney transplant patients. The existence of pseudoporphyria (in dialysis) has been questioned, as some authors have succeeded in demonstrating an increase in plasma porphyrins (uroporphyrin) in almost all dialysis patients.

Triggering by drugs such as amiodarone, nalidixic acid, diuretics (especially furosemide, bumetanide, thiazides) DADPS, tetracyclines, isotretinoin, 5-fluorouracil, St. John's wort, Ciclosporin A, naproxen (most frequent drug trigger, manifestation often only several months after the start of therapy) and voriconazole has been described.

Intensive UV exposure (solariums) is also the subject of discussion as a triggering co-factor.

Clinical features
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Increased photosensitivity with erythema and burning in the area of exposed skin areas, increased skin vulnerability with post-traumatic blistering, erosions, hyperkeratosis, scars, milia.

Histology
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Subepidermal blistering as well as perivascular lymphohistiocyte, eosinophil-rich infiltrate.

Differential diagnosis
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Manifestation of a latent, hereditary porphyria cutanea tarda under dialysis (accumulation of porphyrins, iron as a realisation factor).

Therapy
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Skin care measures, light protection. In case of drug-induced pseudoporphyria, discontinue the medication in question.

Literature
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  1. Caroppo F et al. (2023) Pseudoporphyria Induced by Voriconazole in a 10-year-old Boy: A Case Report and Review of the Literature. Acta Derm Venereol 103:adv10286.
  2. Goerz G et al. (1985) Porphyria cutanea tarda (PCT). Z Hautkr 60: 137-146
  3. Green JJ et al (2001) Pseudoporphyria. J Am Acad Dermatol 44: 100-108
  4. Hivnor C et al (2003) Cyclosporine-induced pseudoporphyria. Arch Dermatol 139: 1373-1374
  5. LaDuca JR et al (2002) Nonsteroidal anti-inflammatory drug-induced pseudoporphyria: a case series. J Cutan Med Surg 6: 320-326
  6. Maerker JM et al (2001) Naproxen-induced pseudoporphyria. Dermatology 52: 1026-1029
  7. Schad SG et al. (2002) Bullae, erosions and scars in the face of 2 girls with idiopathic arthritis. Naproxen-induced pseudoporphyria. Dermatologist 53: 51-53
  8. Schaller J (1992) Pseudoporphyria in hemodialysis-A histologic and immunohistologic case study. Z Hautkr 67: 557-562
  9. Senger E et al. (1991) Porphyria cutanea uraemica: an obligate systemic disease in chronic renal insufficiency? Dermatologist 42: 764-769
  10. Thivolet J (1979) Pseudoporphyria cutanea tarda in hemodialysis patients. Dermatologist 30: 154-157
  11. Tremblay JF et al. (2003) Pseudoporphyria associated with hemodialysis treated with N-acetylcysteine. J Am Acad Dermatol 49: 1189-1190
  12. Velander MJ et al.(2015) Clinical review of pseudoporphyria]. Ugeskr Laeger 177:V11140599.

  13. Weidner T (2018) Naproxen-induced pseudoporphyria. J Dtsch Dermatol Ges 16: 88-91

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Last updated on: 26.03.2024