帕里-龙贝格综合征
Parry-Romberg syndrome.
作者信息
Lazaridou Elizabeth, Giannopoulou Christina, Apalla Zoi, Fotiadou Christina, Trigoni Anastasia, Ioannides Demetris
机构信息
First Department of Dermatology and Venereology, Aristotle University Medical School, Thessaloniki, Greece.
出版信息
J Dermatol Case Rep. 2010 Nov 19;4(2):30-2. doi: 10.3315/jdcr.2010.1049.
BACKGROUND
Parry-Romberg syndrome (PRS) or idiopathic hemifacial atrophy is a rare neurocutaneous syndrome. It is characterized by slowly progressive atrophy, located on one side of the face, primarily involving the skin, fat and connective tissue. PRS seems to overlap with "en coupe de sabre" morphea.
MAIN OBSERVATIONS
We present a case of hemifacial atrophy in a 14-year-old boy treated with topical calcipotriol-betamethasone ointment. The diagnosis of PRS was established mainly based on the clinical findings and histological picture. The time to diagnosis was almost 9 years, similar to the mean time reported in the literature.
CONCLUSIONS
Understanding the pathogenesis and stopping disease progression is important as it can cause severe disfigurement and has neurological and psychiatric complications. Not much is known about the efficacy of agents used in the treatment of this syndrome making treatment decision very difficult. Possible complications, pathophysiology and therapeutic options are being discussed.
背景
帕里-罗姆伯格综合征（PRS）或特发性半侧面部萎缩是一种罕见的神经皮肤综合征。其特征为缓慢进展的萎缩，位于面部一侧，主要累及皮肤、脂肪和结缔组织。PRS似乎与“剑砍状”硬斑病重叠。
主要观察结果
我们报告一例14岁男孩的半侧面部萎缩，采用外用卡泊三醇-倍他米松软膏治疗。PRS的诊断主要基于临床发现和组织学表现。诊断时间将近9年，与文献报道的平均时间相似。
结论
了解发病机制并阻止疾病进展很重要，因为它可导致严重毁容，并伴有神经和精神并发症。对于用于治疗该综合征的药物疗效知之甚少，这使得治疗决策非常困难。文中讨论了可能的并发症、病理生理学和治疗选择。
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