A Case of Erythropoietic Protoporphyria / 대한피부과학회지
Korean Journal of Dermatology
;
: 1375-1379, 2000.
Article
Dans Coréen
| WPRIM
| ID: wpr-75965
ABSTRACT
Erythropoietic protoporphyria(EPP) is an inherited inborn error of porphyrin metabolism caused by decreased activity of the enzyme ferrochelatase. EPP is characterized clinically by photosensitivity to visible light commencing in childhood, and biochemically by elevated red cell free protoporphyrin levels. We report herein a case of EPP which occurred in a 44-year-old man and his family. He had suffered from immediate photosensitivity since he was 4 years old. He was presented with burning, erythema, scars and waxy thickening of the sun-exposed skin. Red cell free protoporphyrin level was elevated and urinary porphyrins were normal. Histopathologically, homogeneous eosinophilic materials that stained with PAS were deposited in perivascular area of upper dermis. He was managed with light restriction and sunscreen.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Porphyrines
/
Peau
/
Brûlures
/
Cicatrice
/
Derme
/
Protoporphyrie érythropoïétique
/
Granulocytes éosinophiles
/
Érythème
/
Ferrochelatase
/
Lumière
Limites du sujet:
Adulte
/
Enfant d'âge préscolaire
/
Humains
langue:
Coréen
Texte intégral:
Korean Journal of Dermatology
Année:
2000
Type:
Article
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