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.

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.

Linear scleroderma en coup de sabre including abnormal dental development.

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.