A case of extensive hyaline deposition in facial skin caused by erythropoietic protoporphyria.

[erythropoietic protoporphyria]

Although erythropoietic protoporphyria (EPP ) is relatively uncommon , affecting approximately 1 in 140 Â 000 individuals in the U .K ., it is an important disease not to miss owing to the risk of acute severe liver disease in 2 % of cases . EPP occurs with clinical and histological changes in the skin associated with free-radical-associated dermal vascular damage . This also mediates the painful photosensitivity . Severe and disfiguring hyaline deposition is extremely rare . We demonstrate that severe EPP can cause disfiguring hyaline infiltration of the skin on the hands and face , which sheds light on the mechanism of photosensitivity in EPP ; it must also be differentiated from conditions such as lipoid proteinosis .