Abstract：
Severe skin pain when exposed to long wave ultraviolet radiation or visible light is the main symptom of erythropoietic protoporphyria (EPP). Treatment options for EPP are inadequate and new treatments are needed but hampered by the lack of valid efficacy outcomes. Phototesting with well-defined illumination of the skin can be performed reliably. We aimed to provide an overview of phototest procedures used to evaluate EPP treatments. Systematic searches of Embase, MEDLINE and the Cochrane Library were performed. Searches identified 11 studies using photosensitivity as efficacy outcome. The studies used eight different phototest protocols. Illuminations were performed with a filtered high-pressure mercury arc, or a xenon arc lamp equipped with monochromator or filters. Some used broadband, others narrowband illumination. In all protocols phototests were performed on the hands or the back. Endpoints were minimal dose required to induce either first symptom of discomfort, erythema, urticaria or intolerable pain. Other endpoints were change in erythema intensity or diameter of any type of flare after exposure compared to before. In conclusion, protocols displayed extensive variability in illumination set-up and evaluation of phototest reactions. Implementation of a standardized phototest method will allow more consistent and reliable outcome evaluation in future therapeutic research of protoporphyric photosensitivity.
摘要：
当暴露于长波紫外线辐射或可见光时，严重的皮肤疼痛是促红细胞生成原卟啉症（EPP）的主要症状。EPP的治疗选择不足，需要新的治疗方法，但由于缺乏有效的疗效结果而受到阻碍。可以可靠地执行具有明确定义的皮肤照明的光测试。我们旨在提供用于评估EPP治疗的光测程序的概述。Embase的系统搜索，进行了MEDLINE和Cochrane图书馆。搜索确定了11项使用光敏性作为疗效结果的研究。研究使用了八种不同的光测方案。用过滤的高压汞弧进行照明，或配有单色器或过滤器的氙弧灯。一些使用宽带，其他窄带照明。在所有方案中,在手或背部进行照相检验。终点是诱发不适的第一个症状所需的最小剂量，红斑,荨麻疹或难以忍受的疼痛。其他终点是暴露后与暴露前相比任何类型的耀斑的红斑强度或直径的变化。总之,协议在照明设置和光测反应评估中显示出广泛的可变性。标准化的光测方法的实施将在未来的原卟啉光敏性治疗研究中允许更一致和可靠的结果评估。
