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European Archives of Paediatric Dentistry

, Volume 16, Issue 2, pp 227–231 | Cite as

Linear scleroderma en coup de sabre including abnormal dental development

  • M. HørbergEmail author
  • S. R. Lauesen
  • J. Daugaard-Jensen
  • I. Kjær
Case Report

Abstract

Background

Linear scleroderma en coup de sabre (SCS) is a rare skin condition, where dense collagen is deposited in a localised groove of the head and neck area resembling the stroke of a sabre. The SCS may involve the oral cavity, but the severity and relation to this skin abnormality is unknown. A paediatric dentist may be the first medical person to identify SCS by its involvement in dentition. It is assumed that the malformation of a dentition could be associated with the severity of the skin deviation.

Case report

A 6-year and 10-month-old Turkish girl with a history of SCS was referred for dental diagnostics and treatment. The SCS skin lesion affected the left side of her hairline over the forehead and nose, involving the left orbit proceeding towards the left oral region. Dental clinical/radiographic examination revealed malformed left maxillary incisors with short roots and lack of eruption.

Follow-up

The patient has been regularly controlled and treated since she was first diagnosed. A surgical and orthodontic treatment was performed to ensure optimal occlusion, space and alveolar bone development. The present age of the patient is 14 years and 10 months.

Conclusion

This case demonstrated a patient with a left-sided skin defect (SCS) and a left-sided local malformation in her dentition. It is possible that there is a developmental connection between these two left-sided defects, both with an ectodermal origin.

Keywords

Scleroderma en coup de sabre Dental Ectopic eruption 

Notes

Acknowledgments

Consent and approval for the full face photographs, showing the eyes, was obtained from the patient and her parents.

References

  1. David J, Wilson J, Woo P. Scleroderma ‘en coup de sabre’. Ann Rheum Dis. 1991;50:260–2.CrossRefPubMedCentralPubMedGoogle Scholar
  2. Davis WC, Saunders TS. Scleroderma of the face involving the gingiva. Arch Derm Syphilol. 1946;54:133–5.CrossRefPubMedGoogle Scholar
  3. Fischer DJ, Patton LL. Scleroderma: oral manifestations and treatment challenges. Spec Care Dentist. 2000;20:240–4.CrossRefPubMedGoogle Scholar
  4. Fox TC. Note on the history of scleroderma in England. Br J Dermatol. 1892;4:101–4.CrossRefGoogle Scholar
  5. Holland KE, Steffes B, Nocton JJ, et al. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics. 2006;117:e132–6.CrossRefPubMedGoogle Scholar
  6. Jablonska S, Blaszczyk M. Long-lasting follow-up favours a close relationship between progressive facial hemiatrophy and scleroderma en coup de sabre. J Eur Acad Dermatol Venereol. 2005;19:403–4.CrossRefPubMedGoogle Scholar
  7. Looby JP, Burket LW. Scleroderma of the face with involvement of the alveolar process. Am J Orthod Oral Surg. 1942;28:493–8.CrossRefGoogle Scholar
  8. Marzano AV, Menni S, Parodi A, et al. Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol. 2003;13:171–6.PubMedGoogle Scholar
  9. Spackman GK. Scleroderma: what the general dentist should know. Gen Dent. 1999;47:576–9.PubMedGoogle Scholar
  10. Tolle SL. Scleroderma: considerations for dental hygienists. Int J Dent Hyg. 2008;6:77–83.CrossRefPubMedGoogle Scholar
  11. Weibel L, Harper JI. Linear morphoea follows Blaschko’s lines. Br J Dermatol. 2008;159:175–81.CrossRefPubMedGoogle Scholar
  12. Zulian F. Systemic sclerosis and localized scleroderma in childhood. Rheum Dis Clin North Am. 2008;34:239–55.CrossRefPubMedGoogle Scholar

Copyright information

© European Academy of Paediatric Dentistry 2014

Authors and Affiliations

  • M. Hørberg
    • 1
    Email author
  • S. R. Lauesen
    • 2
  • J. Daugaard-Jensen
    • 3
  • I. Kjær
    • 2
  1. 1.Department of Oral and Maxillofacial SurgeryKøge University HospitalCopenhagenDenmark
  2. 2.Department of Odontology, Faculty of Health and Medical SciencesUniversity of CopenhagenCopenhagenDenmark
  3. 3.Centre for Rare Oral DiseasesUniversity Hospital Section 5811CopenhagenDenmark

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