Lauesen S R, Daugaard-Jensen J, Lauridsen E F, Kjær I
Department of Odontology, Faculty of Health and Medical Sciences, University of Copenhagen, 20 Nørre Allé, 2200, Copenhagen, Denmark.
Department of Oral and Maxillofacial Surgery and Rare Oral Diseases, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Eur Arch Paediatr Dent. 2019 Aug;20(4):339-350. doi: 10.1007/s40368-019-00427-7. Epub 2019 Mar 7.
When localised scleroderma occurs in the face, neck and scalp area, it is called scleroderma en coup de sabre (SCS) for its resemblance to the stroke of a sabre. Most observed characteristics: abnormal skin and dental development, facial atrophy and neurological complications. The aim was to evaluate the extent of SCS in the underlying subcutis, including teeth/bone tissues. The goal was to solve, how far the external visual skin abnormality extends in depth, and if the condition appears within and limited to craniofacial neural crest fields.
Photographic and radiographic materials from six patients (one male, five females, aged 5-39 years) were included. The cases were divided in three groups, two in each, according to similarity in location of SCS in the skin. Dentition and gingiva were analysed clinically and from intra-oral photos, dental radiographs and orthopantomograms. Agenesis, dental maturity stage (root length), deviation in crown and root morphology (size and shape), and eruption disturbances were registered. Profile and frontal radiographs were analysed cephalometrically for jaw relationships and bone structures.
In SCS, skin affection corresponds to the neural crest regions/fields. A close spatial association between skin, teeth and bone affections within neural crest fields was found. No common traits in profile analyses were observed. Asymmetry from minor to severe appears in the frontal analyses. A malformation in planum sphenoidale was observed in two individuals with the same location of skin affections.
SCS conditions seem to extend from the skin in the depth to the sella turcica area within neural crest fields.
局限性硬皮病发生于面部、颈部和头皮区域时，因其外观类似剑伤而被称为剑伤性硬皮病（SCS）。最常见的特征包括：皮肤和牙齿发育异常、面部萎缩以及神经并发症。本研究旨在评估剑伤性硬皮病在皮下组织（包括牙齿/骨组织）中的累及范围。研究目的是明确外部可见的皮肤异常在深度上延伸多远，以及该病症是否出现在颅面神经嵴区域内并局限于此。
纳入6例患者（1例男性，5例女性，年龄5 - 39岁）的照片和影像学资料。根据剑伤性硬皮病在皮肤中的位置相似性，将病例分为三组，每组2例。对牙列和牙龈进行临床检查，并分析口腔内照片、牙科X光片和曲面断层片。记录牙齿缺失、牙齿成熟阶段（牙根长度）、牙冠和牙根形态偏差（大小和形状）以及萌出障碍情况。对侧位片和正位片进行头影测量分析，以评估颌骨关系和骨结构。
在剑伤性硬皮病中，皮肤病变与神经嵴区域相对应。发现在神经嵴区域内，皮肤、牙齿和骨骼病变之间存在紧密的空间关联。在侧位分析中未观察到共同特征。在正位分析中，出现了从轻度到重度的不对称情况。在两名皮肤病变位置相同的个体中，观察到蝶骨平面畸形。
剑伤性硬皮病似乎从皮肤向深部延伸至神经嵴区域内的蝶鞍区。
