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Localised scleroderma en coup de sabre affecting the skin, dentition and bone tissue within craniofacial neural crest fields. Clinical and radiographic study of six patients

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Abstract

Purpose

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.

Methods

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.

Results

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.

Conclusion

SCS conditions seem to extend from the skin in the depth to the sella turcica area within neural crest fields.

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Acknowledgements

Consent and approval for face photograph with closed eyes, Fig. 1, was obtained from the patient and her parents. Other photographs from the patient were demonstrated by Hørberg et al. (2015).

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Authors and Affiliations

  1. Department of Odontology, Faculty of Health and Medical Sciences, University of Copenhagen, 20 Nørre Allé, 2200, Copenhagen, Denmark

    S. R. Lauesen & I. Kjær

  2. Department of Oral and Maxillofacial Surgery and Rare Oral Diseases, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark

    J. Daugaard-Jensen & E. F. Lauridsen

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  1. S. R. Lauesen
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  2. J. Daugaard-Jensen
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  3. E. F. Lauridsen
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  4. I. Kjær
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Correspondence to I. Kjær.

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Lauesen, S.R., Daugaard-Jensen, J., Lauridsen, E.F. et al. Localised scleroderma en coup de sabre affecting the skin, dentition and bone tissue within craniofacial neural crest fields. Clinical and radiographic study of six patients. Eur Arch Paediatr Dent 20, 339–350 (2019). https://doi.org/10.1007/s40368-019-00427-7

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