Cutaneous photosensitivity results from a heterogeneous group of disorders that drastically impair quality of life(1,2,3). One severe inherited form of photosensitivity is erythropoietic protoporphyria (EPP), which causes life-long painful cutaneous photosensitivity to visible (not UV) light(4,5). In EPP, both the mechanism of pain and the variability in light sensitivity between patients is poorly understood(6,7). While the disease can sometimes be life threatening, there is no food and drug administration (FDA)-approved treatment for EPP(4,8). Clinical trials in EPP have been drastically impaired by the lack of precise endpoints. Recently, a company called SunSense (getsunsense.com) has developed a wearable sensor which provides data on both UV and visible light exposure, including the wavelengths of light to which EPP patients are sensitive(4,9). The overall goal of this project is to test this wearable device for light exposure monitoring in EPP, and this has the potential help patients in two important ways. 1. Analyzing light exposure practices and the resulting symptoms can provide a better understanding of the pathophysiology of phototoxicity in EPP that could inform the development of new therapies. 2. Enabling EPP patients to make informed decisions about sunlight avoidance based on their accumulated light dose will improve quality of life. Ultimately, more accurate dosimetry of light exposure in EPP will improve data collection for clinical trials. While this project focuses on EPP, much of the knowledge gained from this study will also be transferrable to other forms of photosensitivity.
Massachusetts General Hospital, Boston