Riga-Fede disease in an infant

A 10-month-old female baby was brought by her mother for an asymptomatic solitary verrucous plaque over the ventral aspect of the tongue in the midline since the age of 6 months, not interfering with breastfeeding. Local examination also revealed upper and lower central incisors with sharp edges, which erupted at the age of 6 months [Figure 1]. Riga-Fede disease (RFD) was diagnosed. Magnetic resonance imaging (MRI) brain and electroencephalography (EEG) were not advised as the child was asymptomatic. Parents were counselled and reassured about the benign nature of this condition and advised to seek dental and otorhinolaryngology opinions with regular follow-up.

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Figure 1:

Solitary yellow to white verrucous plaque over the ventral aspect of the tongue in the midline.

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RFD is caused by trauma from the sharp margins of natal and neonatal teeth or from teeth erupting at the normal time. This trauma can lead to ulcers on the tongue, lips, or other areas of the oral cavity. The base of the ulcer is covered with a yellow-to-white fibrinopurulent membrane with surrounding erythema. RFD can be associated with cerebral palsy, Lesch-Nyhan syndrome, familial dysautonomia, etc. An MRI brain is recommended for children with neurological signs and symptoms to confirm further cerebral palsy. EEG and genetic testing can be advised to patients with the Lesch-Nyhan syndrome associated with RFD.