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Systematic Assessment of Mammalian Skull Specimens for Dental and Temporomandibular Joint Pathology
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Linear scleroderma en coup de sabre including abnormal dental development.

M Hørberg1, S R Lauesen, J Daugaard-Jensen

  • 1Department of Oral and Maxillofacial Surgery, Køge University Hospital, Region Zealand, Lykkebækvej 1, 4600 Køge, Copenhagen, Denmark, mettehoerberg@hotmail.com.

European Archives of Paediatric Dentistry : Official Journal of the European Academy of Paediatric Dentistry
|October 31, 2014
PubMed
Summary
This summary is machine-generated.

Scleroderma en coup de sabre (SCS) is a rare condition causing skin grooves. This case highlights a potential developmental link between SCS skin defects and localized dental malformations, suggesting a shared ectodermal origin.

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Area of Science:

  • Dermatology
  • Pediatric Dentistry
  • Genetics

Background:

  • Scleroderma en coup de sabre (SCS) is a rare linear morphea variant characterized by localized collagen deposition.
  • While SCS can affect the oral cavity, its severity and relationship to dental anomalies remain unclear.
  • Paediatric dentists may be the first to identify SCS through dental involvement, suggesting a potential diagnostic role.

Observation:

  • A case study of a 6-year-old girl with left-sided SCS involving the face and oral region.
  • Dental examination revealed malformed left maxillary incisors with short roots and delayed eruption.
  • The SCS lesion extended from the hairline, across the forehead and nose, to the oral region.

Findings:

  • The patient presented with unilateral facial skin atrophy and ipsilateral dental anomalies.
  • Surgical and orthodontic treatments were performed to address occlusion and alveolar bone development.
  • A possible developmental connection between the left-sided ectodermal defects (skin and dentition) was observed.

Implications:

  • This case suggests a potential shared developmental pathway for SCS and localized dental malformations.
  • Early dental evaluation may aid in the diagnosis of SCS in children.
  • Further research into the ectodermal origins of SCS and associated dental anomalies is warranted.