Absolute light intolerance: Erythropoietic protoporphyria (EPP)
Erythropoietic protoporphyria (EPP) is a rare and severe UV/light related skin disorder.
The Australian outdoor lifestyle has ingrained in us a distinct awareness and understanding of the dangers of sunlight and UV exposure. This unique insight makes knowledge of photodermatoses (UV/Light related skin disorders) all the more profound.
Erythropoietic protoporphyria (EPP) is a rare genetic disease where patients have a deficiency of an enzyme called ferrochelatase. These patients aren’t able to convert protoporphyrin IX (PPIX) into heme and so they accumulate PPIX in their body. When exposed to light and UV, PPIX causes a phototoxic reaction, resulting in intolerable burning pain and causing EPP patients to avoid light/UV exposure.
CLINUVEL, an Australian Company, understands the needs of Australians to enjoy the great outdoors and the needs of people globally suffering from acute UV and light related disorders.
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- Marko PB et al., (2007). “Erythropoietic protoporphyria patients in Slovenia”, Acta Dermatoven. Vol 16;3, pp99-104.
Murphy, G M (2003). ‘Diagnosis and Management of the Erythropoitetic Porphyrias’, Dermatologic Therapy. Vol 16, pp57-64.