Pseudoporphyria E80.25

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 29.10.2020

Definition
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Polyetiological, blistering skin disease on sun-exposed skin areas, which leads to a porphyria cutanea tarda-like skin appearance without pathological porphyrin chemistry. Blistering is characteristic 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.

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Etiopathogenesis
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Usually in chronic renal failure, haemodialysis patients or kidney transplant patients. The existence of pseudoporphyria (in dialysis) has been questioned, as some authors have been able to demonstrate an increase in plasma porphyrins (uroporphyrin) in almost all dialysis patients.

Triggered 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 common drug trigger, manifestation often several months after the start of therapy) is described.

Intensive UV-exposure (solariums) is also discussed 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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Goerz G et al (1985) Porphyria cutanea tarda (PCT). Z Hautkr 60: 137-146
Green JJ et al (2001) Pseudoporphyria. J Am Acad Dermatol 44: 100-108
Hivnor C et al (2003) Cyclosporine-induced pseudoporphyria. Arch Dermatol 139: 1373-1374
LaDuca JR et al (2002) Nonsteroidal antiinflammatory drug-induced pseudoporphyria: a case series. J Cutan Med Surg 6: 320-326
Maerker JM et al (2001) Naproxen-induced pseudoporphyria. dermatologist 52: 1026-1029
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
Schaller J (1992) Pseudoporphyria on hemodialysisA histological and immunohistological case study. Z Hautkr 67: 557-562
Senger E et al (1991) Porphyria cutanea uraemica: an obligatory systemic disease in chronic renal failure? dermatologist 42: 764-769
Thivolet J (1979) Pseudoporphyria cutanea tarda in hemodialysis patients. dermatologist 30: 154-157
Tremblay JF et al (2003) Pseudoporphyria associated with hemodialysis treated with N-acetylcysteine. J Am Acad Dermatol 49: 1189-1190
Weidner T (2018) Naproxen-induced pseudoporphyria. J Dtsch Dermatol Ges 16: 88-91