Persistent lingual ulceration (Riga-Fede disease) in an infant with Down syndrome and natal teeth: a case report

INTRODUCTION: Riga-Fede disease is a rare pediatric condition in which chronic lingual ulceration results from repetitive trauma. Neonatal teeth or underlying neuro-developmental disorders which include Down syndrome are described as causative factors, but to the best of our knowledge, this is the first case report of both Down syndrome and natal teeth coexisting. The need for early extraction in the presence of two risk factors is highlighted in this case report. CASE PRESENTATION: An 18-month-old Sinhalese male presented with an ulcerating lingual mass on the ventral surface of the tongue. The lesion had progressed over the past six months. He also had clinically diagnosed Down syndrome. The ulcer was non-tender, indurated, and had elevated margins. It was not bleeding and two natal teeth in lower central dentition were seen in apposition with the lesion. There was no regional lymphadenopathy but the ulcer was causing concerns as it mimicked a malignant lesion. A clinical diagnosis of Riga-Fede disease caused by raking movements of the tongue against anterior natal teeth by a child who was developmentally delayed and prone to suck on his tongue was made. The mother was reassured and the natal teeth were extracted. CONCLUSIONS: Early extraction of natal teeth is recommended only if there is a risk of aspiration or interference with breast feeding. Although Down syndrome is among the neuro-developmental conditions that lead to this lesion, its occurrence is usually at an older age. The presence of natal teeth together with Down syndrome caused the lesion to occur in infancy. Awareness of the benign nature of this rare condition by pediatricians and dental practitioners is important as it will allay anxiety and avoid unnecessary biopsy. This case also highlights the impact of two risk factors and needs consideration as an added indication for the early extraction of natal teeth.