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.