Odontostomatologic involvement in juvenile localised scleroderma of the face

Localised scleroderma of the face (LSF) can lead to significant aesthetical and functional abnormalities. Despite their quite frequent clinical observation, the odontostomatologic complications are not thoroughly described. Aim of the study was to describe the clinical features of the most frequent odontostomatologic abnormalities of LSF and to propose clinical and radiologic criteria for the assessment and follow-up of these complications.

We performed a cross-sectional, multicenter study involving a multidisciplinary team formed by paediatric rheumatologists, orthodontists and radiologists. Patients with a diagnosis of LSF underwent a comprehensive rheumatologic evaluation, dental examination, conventional radiology (orthopantomography, teleradiography) and cone beam computed tomography.

16 patients, nine F, seven M, aged 6.5-21.9 years, were investigated. The mean disease duration was 7.7 years, 62.5% had extracutaneous complications. All patients reported at least one odontostomatologic complication. The main alterations were: malocclusion (94%), overgrowth tendency of the anterior lower third of the face (82%), gnatologic alterations (69%), dental anomalies (63%), skeletal asymmetry (56%), bone involvement (50%) and temporomandibular joint involvement (19%).

We found a high incidence of odontostomatologic abnormalities in LSF. Cone beam computed tomography represents a new feasible technique to evaluate and monitor soft and hard tissue changes in LSF. The observed findings highlight the importance of a multidisciplinary and standardised management of this challenging and rare condition.