Protection against visible light by dihydroxyacetone in erythropoietic protoporphyria

Heerfordt, Ida M.; Philipsen, Peter Alshede; Lerche, Catharina M.; Wulf, Hans Christian

doi:10.1016/j.pdpdt.2023.103302

Cited by 2 publications

(1 citation statement)

References 30 publications

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“…The choice of wavelengths is important for the phototest as some treatments, for example, implant afamelanotide or application of dihydroxyacetone, will protect the skin better against some wavelengths than others. 21,27,28 As the symptoms in the patients' everyday life are caused by daylight, we propose using a wide range of visible wavelengths, optimally a continuous light spectrum of 400-750 nm simulating daylight, or alternatively, a light source covering many of PpIX's absorption peaks (Figure 1). Light-emitting diodes with these characteristics are currently in use for indoor gentle PpIX photodynamic therapy.…”

Section: Studymentioning

confidence: 99%

Phototesting in erythropoietic protoporphyria trials: A systematic review

Heerfordt

Philipsen

Lerche

et al. 2023

Experimental Dermatology

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Severe skin pain when exposed to long wave ultraviolet radiation or visible light is the main symptom of erythropoietic protoporphyria (EPP). Treatment options for EPP are inadequate and new treatments are needed but hampered by the lack of valid efficacy outcomes. Phototesting with well‐defined illumination of the skin can be performed reliably. We aimed to provide an overview of phototest procedures used to evaluate EPP treatments. Systematic searches of Embase, MEDLINE and the Cochrane Library were performed. Searches identified 11 studies using photosensitivity as efficacy outcome. The studies used eight different phototest protocols. Illuminations were performed with a filtered high‐pressure mercury arc, or a xenon arc lamp equipped with monochromator or filters. Some used broadband, others narrowband illumination. In all protocols phototests were performed on the hands or the back. Endpoints were minimal dose required to induce either first symptom of discomfort, erythema, urticaria or intolerable pain. Other endpoints were change in erythema intensity or diameter of any type of flare after exposure compared to before. In conclusion, protocols displayed extensive variability in illumination set‐up and evaluation of phototest reactions. Implementation of a standardized phototest method will allow more consistent and reliable outcome evaluation in future therapeutic research of protoporphyric photosensitivity.

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Section: Studymentioning

confidence: 99%

Phototesting in erythropoietic protoporphyria trials: A systematic review

Heerfordt

Philipsen

Lerche

et al. 2023

Experimental Dermatology

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Exploring current and emerging therapies for porphyrias

Jericó,

Córdoba,

Urigo

et al. 2024

Liver International

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Porphyrias are rare, mostly inherited disorders resulting from altered activity of specific enzymes in the haem synthesis pathway that lead to accumulation of pathway intermediates. Photocutaneous symptoms occur when excess amounts of photoreactive porphyrins circulate in the blood to the skin, whereas increases in potentially neurotoxic porphyrin precursors are associated with neurovisceral symptoms. Current therapies are suboptimal and their mechanisms are not well established. As described here, emerging therapies address underlying disease mechanisms by introducing a gene, RNA or other specific molecule with the potential to cure or slow progression of the disease. Recent progress in nanotechnology and nanoscience, particularly regarding particle design and formulation, is expanding disease targets. More secure and efficient drug delivery systems have extended our toolbox for transferring specific molecules, especially into hepatocytes, and led to proof‐of‐concept studies in animal models. Repurposing existing drugs as molecular chaperones or haem synthesis inhibitors is also promising. This review summarizes key examples of these emerging therapeutic approaches and their application for hepatic and erythropoietic porphyrias.

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Protection against visible light by dihydroxyacetone in erythropoietic protoporphyria

Cited by 2 publications

References 30 publications

Phototesting in erythropoietic protoporphyria trials: A systematic review

Phototesting in erythropoietic protoporphyria trials: A systematic review

Exploring current and emerging therapies for porphyrias

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