A Case of Erythropoietic Protoporphyria / 대한피부과학회지

ABSTRACT

A 26-year-old male has experienced repeated pruritic and burning erythema and edema over the exposed areas of the skin, especially on the face and hands, after sunlight exposure. He stated that his father, three aunts, and three sisters had similar sunlight sensitivity. The face showed tiny whitish scars. And also there were hypopigmented scars on the dorsum of the hands. Characteristic laboratory findings were as followa RBC, plasma and stool fluoresced coral red color under the Woods light. 100ml of the RBC contained 245 pg of protoporphyrin. On photosensitive test using sunlight for 60 rninutes, there developed painful sensation at 4 minutes and 40 seconds after exposure, erythema at 12 minutes after, and edema at 20 minutes after. Pathological findings of the specimen obtained from erythematous lesion of the forehead revealed hypergranulosis and acanthosis in the epidermis and perivascular thickening and hyalin deposit around the capillaries in the upper dermis. For treatment, avoidance of exposure to sunlight was recomrnended and symptomatic therapy was tried.