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.
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PseudoporphyriaE80.25
DefinitionThis section has been translated automatically.
Occurrence/EpidemiologyThis section has been translated automatically.
haemodialysis (in about 16% of patients). Medicines: in 10-12% of children treated with naproxen.
EtiopathogenesisThis section has been translated automatically.
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 featuresThis section has been translated automatically.
HistologyThis section has been translated automatically.
Subepidermal blistering as well as perivascular lymphohistiocyte, eosinophil-rich infiltrate.
Differential diagnosisThis section has been translated automatically.
Manifestation of a latent, hereditary porphyria cutanea tarda under dialysis (accumulation of porphyrins, iron as a realisation factor).
TherapyThis section has been translated automatically.
Skin care measures, light protection. In case of drug-induced pseudoporphyria, discontinue the medication in question.
LiteratureThis section has been translated automatically.
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Velander MJ et al.(2015) Clinical review of pseudoporphyria]. Ugeskr Laeger 177:V11140599.
- Weidner T (2018) Naproxen-induced pseudoporphyria. J Dtsch Dermatol Ges 16: 88-91