Baskan Emel Bulbul, Kaçar Seval Doğruk, Turan Ayşegül, Saricaoglu Hayriye, Tunali Sukran, Adim Saduman Balaban
Dermatology Department, Uludag University Medical Faculty, Bursa, Turkey.
Photodermatol Photoimmunol Photomed. 2006 Oct;22(5):259-61. doi: 10.1111/j.1600-0781.2006.00238.x.
Parry-Romberg syndrome (PRS) or progressive hemifacial atrophy is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue and sometimes bone and cartilage. Although this syndrome has overlapping features of scleroderma 'en coup de sabre', it shows little or no sclerosis and may affect the entire distribution of the trigeminal nerve including the eye and tongue. As the pathogenesis is unknown, no effective therapy exists. We present here the third case of PRS associated with borreliosis and more interestingly two cases whose progressive course have been stabilized with gel PUVA therapy.
帕里-罗姆伯格综合征(PRS)或进行性面部半侧萎缩是一种罕见病症,其特征为皮肤、皮下组织,有时还包括骨骼和软骨的单侧萎缩。尽管该综合征具有硬皮病“剑伤状”的重叠特征,但几乎没有或不存在硬化现象,且可能影响三叉神经的整个分布区域,包括眼睛和舌头。由于发病机制尚不清楚,目前尚无有效治疗方法。我们在此报告第三例与莱姆病相关的PRS病例,更有趣的是,还有两例病例经凝胶补骨脂素紫外线A光疗法(gel PUVA therapy)后病情进展得以稳定。