Phototesting in erythropoietic protoporphyria trials: A systematic review

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 ill...

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Bibliographic Details
Published in:Experimental dermatology 2023-07, Vol.32 (7), p.1042-1047
Main Authors: Heerfordt, Ida M., Philipsen, Peter A., Lerche, Catharina M., Wulf, Hans Christian
Format: Article
Language:English
Subjects:
Erythema
Erythropoietic protoporphyria
Humans
Illumination
Pain
photodynamic reaction
Photosensitivity
Photosensitivity Disorders
phototest
Protoporphyria, Erythropoietic - therapy
protoporphyrin IX
Skin
Ultraviolet radiation
Ultraviolet Rays
Urticaria
Xenon
X‐linked protoporphyria
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Summary: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.
ISSN:0906-6705
1600-0625
DOI:10.1111/exd.14809