Khan Mohsin, Khan Mubeen, Negi Raju, Gupta Nikita
M.D.S, Masters Of Dental surgery. Department of Orthodontics and Dentofacial Orthopaedics, Bangalore, Karnataka, India.
M.D.S, Professor and Head, Department of Oral Medicine and Radiology, Government Dental College and Research Institute, Bangalore, Karnataka, India.
J Clin Exp Dent. 2014 Jul 1;6(3):e313-6. doi: 10.4317/jced.51409. eCollection 2014 Jul.
Parry Romberg syndrome(PRS) is a rare acquired poorly understood neurocutaneous syndrome of unknown etiology characterized by slow progressive atrophic changes commonly affecting one half of the face. The exact incidence and etiology towards the syndrome remains unclear. Apart from the multifactorial etiology proposed, the possible primary cause is mainly attributed to the cerebral disturbance of the fat metabolism. The syndrome overlaps with "en coup de sabre" morphea, with an ill defined relationship existing between the two. Parry Romberg Syndrome is an invalidating lesion that may be associated with different neurological, cutaneous, ocular, dental and autoimmune abnormalities. This report presents one rare case of 22 years old female patient with Parry Romberg syndrome associated with localized scleroderma, accompanied by a brief review of literature with classical clinical, radiographic, histological findings and the treatment of progressive hemifacial atrophy. Key words:Parry Romberg syndrome, progressive facial hemiatrophy, morphea, localized scleroderma.
帕里-罗默伯格综合征(PRS)是一种罕见的、病因不明且了解甚少的获得性神经皮肤综合征,其特征为缓慢进展的萎缩性改变,通常累及半侧面部。该综合征的确切发病率和病因尚不清楚。除了所提出的多因素病因外,可能的主要原因主要归因于脂肪代谢的脑功能紊乱。该综合征与“剑伤样”硬斑病重叠,两者之间的关系尚不明确。帕里-罗默伯格综合征是一种致残性病变,可能与不同的神经、皮肤、眼部、牙齿和自身免疫异常有关。本报告介绍了一例罕见的22岁女性帕里-罗默伯格综合征患者,该患者伴有局限性硬皮病,并对有关进行性半侧面部萎缩的经典临床、影像学、组织学表现及治疗的文献进行了简要回顾。关键词:帕里-罗默伯格综合征;进行性面部半侧萎缩;硬斑病;局限性硬皮病