Pseudoporphyria E80.25

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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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.

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

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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.

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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).

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

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


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Last updated on: 29.10.2020