Vafa Atefeh, Gevorgyan Ofelya, De Debalina, Hassan Sobia
Department of Internal Medicine, John H Stroger Hospital of Cook County, Illinois, US.
Department of Rheumatology, Rush University Medical Center, Illinois, US.
Eur J Rheumatol. 2019 May 20;6(4):219-222. doi: 10.5152/eurjrheum.2019.18100.
Retinal vasculitis is a sight-threatening condition that can occur as an isolated ocular disorder or in association with a number of systemic diseases. Parry-Romberg syndrome, also known as progressive hemifacial atrophy (PHA), is a rare disorder of unknown etiology characterized by unilateral facial atrophy and is associated with multiple ophthalmologic and neurologic manifestations. Here we report the case of a 17-year-old man with no prior diagnosis of PHA, who presented with a sudden onset of floaters and decreased vision in the right eye; he was found to have retinal vasculitis and uveitis in the right eye. Routine workup did not reveal the cause of retinal vasculitis. However, thorough physical examination demonstrated features of PHA overlapping with linear scleroderma en coup de sabre. The patient was started on treatment with systemic steroids with a later addition of methotrexate; he responded to treatment with considerable improvement in his symptoms and ophthalmologic examination.
视网膜血管炎是一种可导致视力受损的疾病，可作为一种孤立的眼部疾病出现，或与多种全身性疾病相关。帕里-罗姆伯格综合征，也称为进行性半侧面部萎缩（PHA），是一种病因不明的罕见疾病，其特征为单侧面部萎缩，并伴有多种眼科和神经学表现。在此，我们报告一例17岁男性病例，该患者既往未诊断为PHA，此次因右眼突然出现飞蚊症和视力下降就诊；检查发现其右眼患有视网膜血管炎和葡萄膜炎。常规检查未发现视网膜血管炎的病因。然而，全面的体格检查显示出PHA的特征，与剑伤样线状硬皮病重叠。患者开始接受全身性类固醇治疗，随后加用甲氨蝶呤；他对治疗有反应，症状和眼科检查有显著改善。
