Parry Romberg综合征伴偶发政变:一例罕见病例报告

Q4 Dentistry Acta Stomatologica Naissi Pub Date : 2021-01-01 DOI:10.5937/asn2184282f
Asan Faizal, S. Babu, V. Shetty, R. Castelino, Vaibhav Pandita
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引用次数: 0

摘要

问题的基础:Parry Romberg综合征(PRS)是一种罕见的发育障碍,表现为面部缓慢和进行性萎缩,通常是单侧的,因此也被称为进行性面部萎缩。萎缩的程度可能不同,包括浅层皮肤延伸到下面的骨头。PRS的临床特征包括颅面、神经、眼和皮肤表现,并导致各种功能和心理问题。PRS与线性硬皮病属于同一疾病谱系。在PRS中可以看到正常和异常皮肤之间的分界线,称为“en coup de sabre”。PRS最常见于女性,涉及左脸。方法:我们在此报告一例年轻女孩的PRS,表现为右侧面部的剑变外观。结果:患侧面部显微外科重建被认为是矫正面部对称性的金标准方法。在我们的病例中,患者目前正在进行长期随访,一旦萎缩达到稳定,将计划进行整形手术治疗。结论:PRS与线性硬皮病的相关性可能存在诊断困难。因此,仔细记录病史和适当的临床检查有助于建立正确的诊断。
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Parry Romberg syndrome with en coup de sabre: A report of a rare case
The basis of the problem: Parry Romberg syndrome (PRS) is a rare developmental disorder manifesting as a slow and progressive atrophy of the face which is often unilateral, hence also termed as rpogressivehemifacial atrophy. The extent of the atrophy may vary, involving the superficial skin extending upto the underlying bone. The clinical features of PRS include craniofacial, neurologic, ophthalmic and dermatological manifestations, which result in various functional and psychological problems. PRS and linear scleroderma belong to the same disease spectrum. There can be demarcating line between the normal and abnormal skin seen in PRS, termed as "en coup de sabre". PRS is most commonly seen in females and involves the left side of the face. Method: We hereby report a case of PRS in a young girl presenting with en coup de sabre appearance on the right side of face. Results: Microsurgical facial reconstruction of the affected side is known to be the gold standard method for correction of the facial symmetry. In our case, the patient is currently kept under long term follow up and cosmetic surgical treatment will be planned once the atrophy attains stability. Conclusion: The association of PRS with linear scleroderma may present difficulty in its diagnosis. Hence, careful recording of the history and clinical examination with appropriate investigations can aid in establishing the correct diagnosis.
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来源期刊
Acta Stomatologica Naissi
Acta Stomatologica Naissi Dentistry-Dentistry (all)
CiteScore
0.10
自引率
0.00%
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0
审稿时长
4 weeks
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