Dermatology Department, Hospital Universitario de La Princesa, Madrid, Spain.
Photodermatol Photoimmunol Photomed. 2012 Oct;28(5):261-3. doi: 10.1111/j.1600-0781.2012.00677.x.
Erythropoietic protoporphyria arises from an inherited disorder of porphyrin metabolism which leads to an accumulation of protoporphyrin IX in the erythropoietic system and other tissues. It is characterized by cutaneous photosensitivity, usually difficult to keep under control. Among the scant therapeutic options proposed to reduce photosensitivity in erythropoietic protoporphyria, narrow-band UVB phototherapy has occasionally been used to induce sunlight tolerance. We report an adult case of erythropoietic protoporphyria with a severe photosensitivity treated with narrow-band UVB that developed an appropriate sunlight phototolerance, without adverse events during phototherapy.
红细胞生成性原卟啉症是一种遗传性卟啉代谢紊乱,导致原卟啉 IX 在红细胞生成系统和其他组织中积累。其特征是皮肤光敏性,通常难以控制。在为减少红细胞生成性原卟啉症的光敏性而提出的少数治疗选择中,窄带 UVB 光疗偶尔被用于诱导阳光耐受。我们报告了一例成人红细胞生成性原卟啉症,该患者光敏性严重,接受了窄带 UVB 治疗,从而获得了适当的阳光光耐受,光疗期间无不良反应。