TY - JOUR

T1 - Protection against visible light by dihydroxyacetone in erythropoietic protoporphyria

AU - Heerfordt, Ida M.

AU - Philipsen, Peter A.

AU - Lerche, Catharina M.

AU - Wulf, Hans Christian

N1 - Funding Information: This work was supported by an unrestricted grant from the Alfred Benzon Foundation, Denmark. The funder had no role in designing this paper or the decision to publish it. The funder will have no role in the further execution of the project. We thank Louise Holbæk Kaihøi, Copenhagen University Hospital, for secretarial assistance. Photo credit to Colourbox.dk. Drawing in Fig. 1 was created with BioRender.com. Funding Information: This work was supported by an unrestricted grant from the Alfred Benzon Foundation, Denmark. The funder had no role in designing this paper or the decision to publish it. The funder will have no role in the further execution of the project. We thank Louise Holbæk Kaihøi, Copenhagen University Hospital, for secretarial assistance. Photo credit to Colourbox.dk. Drawing in Fig. 1 was created with BioRender.com.

PY - 2023

Y1 - 2023

N2 - Background: Patients with erythropoietic protoporphyria (EPP) are hypersensitive to long wave ultraviolet (UVA) radiation and visible light and they experience severe skin pain by light exposure. The patients have very limited treatment options. Sunless skin tanning with dihydroxyacetone (DHA) is now being investigated as a possible treatment modality of skin photosensitivity in EPP. Methods: We simulated the theoretical light protection factor provided by DHA application. In addition, we present 19 cases with EPP who were treated at our department with DHA weekly during spring and summer from 2018 to 2021 inclusive. Results: The protection factor against UVA and visible light was estimated to approximately two. Out of the 19 patients with EPP who were treated with DHA in 2018, 11 patients experienced a sustained good effect and continued to use the treatment on a weekly basis in the spring and summer of 2019, 2020, and 2021. Conclusion and perspectives: Both the theoretical estimates and the uncontrolled study suggest that sunless tanning with DHA reduces photosensitivity in patients with EPP. Our hypothesis is that skin treated with DHA can tolerate twice the daylight dose compared to untreated skin before onset of skin symptoms. To validate this conclusion, we plan a randomized clinical trial to determine the effect of DHA application to reduce photosensitivity in patients with EPP under controlled clinical conditions. The study protocol for this trial is presented in the paper.

AB - Background: Patients with erythropoietic protoporphyria (EPP) are hypersensitive to long wave ultraviolet (UVA) radiation and visible light and they experience severe skin pain by light exposure. The patients have very limited treatment options. Sunless skin tanning with dihydroxyacetone (DHA) is now being investigated as a possible treatment modality of skin photosensitivity in EPP. Methods: We simulated the theoretical light protection factor provided by DHA application. In addition, we present 19 cases with EPP who were treated at our department with DHA weekly during spring and summer from 2018 to 2021 inclusive. Results: The protection factor against UVA and visible light was estimated to approximately two. Out of the 19 patients with EPP who were treated with DHA in 2018, 11 patients experienced a sustained good effect and continued to use the treatment on a weekly basis in the spring and summer of 2019, 2020, and 2021. Conclusion and perspectives: Both the theoretical estimates and the uncontrolled study suggest that sunless tanning with DHA reduces photosensitivity in patients with EPP. Our hypothesis is that skin treated with DHA can tolerate twice the daylight dose compared to untreated skin before onset of skin symptoms. To validate this conclusion, we plan a randomized clinical trial to determine the effect of DHA application to reduce photosensitivity in patients with EPP under controlled clinical conditions. The study protocol for this trial is presented in the paper.

KW - Dihydroxyacetone

KW - Erythropoietic protoporphyria

KW - Light protection

KW - Protoporphyrin IX

KW - Self-tanning

KW - Skin

U2 - 10.1016/j.pdpdt.2023.103302

DO - 10.1016/j.pdpdt.2023.103302

M3 - Journal article

C2 - 36690194

AN - SCOPUS:85147118219

VL - 41

JO - Photodiagnosis and Photodynamic Therapy

JF - Photodiagnosis and Photodynamic Therapy

SN - 1572-1000

M1 - 103302

ER -