Dixit Shantanu, Kalkur Chaithra, Sattur Atul P, Bornstein Michael M, Melton Fred
Department of Oral Medicine and Radiology, Dhulikhel hospital, Kathmandu University School of Medical Sciences, Dhulikhel, Nepal.
Department of Oral Medicine and Radiology, Century International Institute of Dental Science and Research Centre, Kasargod, Kerala, India.
J Med Case Rep. 2016 Oct 24;10(1):297. doi: 10.1186/s13256-016-1086-1.
Scleroderma is a chronic connective tissue disorder with unknown etiology. It is characterized by excessive deposition of extracellular matrix in the connective tissues causing vascular disturbances which can result in tissue hypoxia. These changes are manifested as atrophy of the skin and/or mucosa, subcutaneous tissue, muscles, and internal organs. Such changes can be classified into two types, namely, morphea (localized) and diffuse (systemic). Morphea can manifest itself as hemifacial atrophy (Parry-Romberg syndrome) although this remains debatable. Hence, we present a case of morphea, associated with Parry-Romberg syndrome, and a second case with the classical signs of progressive systemic sclerosis.
Case one: A 20-year-old man of Dravidian origin presented to our out-patient department with a complaint of facial asymmetry, difficulty in speech, and loss of taste sensation over the last 2 years. There was no history of facial trauma. After physical and radiological investigations, we found gross asymmetry of the left side of his face, a scar on his chin, tongue atrophy, relative microdontia, thinning of the ramus/body of his mandible, and sclerotic lesions on his trunk. Serological investigations were positive for antinuclear antibody for double-stranded deoxyribonucleic acid and mitochondria. A biopsy was suggestive of morphea. Hence, our final diagnosis was mixed morphea with Parry-Romberg syndrome. Case two: A 53-year-old woman of Dravidian origin presented to our out-patient department with a complaint of gradually decreasing mouth opening over the past 7 years. Her medical history was noncontributory. On clinical examination, we found her perioral, neck, and hand skin to be sclerotic. Also, her fingers exhibited bilateral telangiectasia. An oral examination revealed completely edentulous arches as well as xerostomia and candidiasis. Her serological reports were positive for antinuclear antibodies against centromere B, Scl-70, and Ro-52. A hand and wrist radiograph revealed acro-osteolysis of the middle finger on her right hand. Hence, our final diagnosis was progressive systemic sclerosis.
Through this article, we have tried to emphasize the importance of a general examination when diagnosing rare systemic diseases such as scleroderma and the role of the general dentist when caring for such patients, even though they can be quite rare in general practice.
硬皮病是一种病因不明的慢性结缔组织疾病。其特征是结缔组织中细胞外基质过度沉积，导致血管紊乱，进而引起组织缺氧。这些变化表现为皮肤和/或黏膜、皮下组织、肌肉及内脏的萎缩。此类变化可分为两种类型，即局限性硬皮病（硬斑病）和弥漫性（系统性）硬皮病。硬斑病可表现为半侧面部萎缩（帕里 - 龙贝格综合征），尽管这一点仍存在争议。因此，我们报告一例伴有帕里 - 龙贝格综合征的硬斑病病例，以及另一例具有进行性系统性硬化症典型体征的病例。
病例一：一名20岁的达罗毗荼族男性因面部不对称、言语困难及味觉丧失前来我院门诊就诊，症状持续2年。无面部外伤史。经过体格检查和影像学检查，我们发现其左侧面部严重不对称、下巴有瘢痕、舌萎缩、相对牙过小、下颌支/体变薄以及躯干有硬化性病变。血清学检查显示抗双链脱氧核糖核酸和线粒体的抗核抗体呈阳性。活检提示为硬斑病。因此，我们最终诊断为伴有帕里 - 龙贝格综合征的混合型硬斑病。病例二：一名53岁的达罗毗荼族女性因过去7年开口度逐渐减小前来我院门诊就诊。她的病史无特殊意义。临床检查发现，她的口周、颈部及手部皮肤硬化。此外，她的手指出现双侧毛细血管扩张。口腔检查发现牙弓完全无牙，伴有口干和念珠菌病。她的血清学报告显示抗着丝粒B、Scl - 70和Ro - 52的抗核抗体呈阳性。手部及腕部X线片显示其右手中指有肢端骨质溶解。因此，我们最终诊断为进行性系统性硬化症。
通过本文，我们试图强调在诊断硬皮病等罕见系统性疾病时全面检查的重要性，以及全科牙医在照料此类患者时所起的作用，尽管这类疾病在一般临床实践中相当罕见。
