What is Riga-Fede disease?
Riga-Fede disease is a mouth ulcer seen in infants and is usually caused by rubbing the tongue over teeth. The ulcer may be uncomfortable but it is otherwise harmless. Less commonly other parts of the mouth may be involved. It is also known as sublingual ulceration, sublingual traumatic ulceration, traumatic lingual ulceration, sublingual traumatic atrophic glossitis, sublingual fibrogranuloma, traumatic granuloma of the tongue, traumatic ulcerative granuloma with stromal eosinophilia, eosinophilic granuloma, traumatic eosinophilic ulceration of the tongue and sublingual growth in infants.
Who gets Riga-Fede disease and why?
Riga-Fede disease is rare. It usually develops when the tongue is rubbed repeatedly over the teeth, usually the lower front teeth. It most commonly complicates natal or neonatal teeth as the newborn baby pushes the tongue forward when feeding (infantile tongue thrusting reflex) and the tongue lies between the alveolar ridges. It can also develop in older babies when their lower front teeth erupt at the normal time. The usual age range is 1 week to 1 year, with peaks in early infancy and 6-8 months of age. However there have been rare reports of it affecting older children and adults.
There is a strong association with cerebral palsy, especially when the tongue is affected by spasticity, in which case the course can be prolonged.
Other associations reported include:
- Lesch-Nyhan syndrome (MIM300322)
- Tourette syndrome (MIM137580)
- Familial dysautonomia (Riley-Day syndrome, MIM223900)
- Microcephaly (small head)
- Macroglossia (large tongue) with tongue protrusion
- Tongue biting
Males more commonly develop Riga-Fede disease than females.
Clinical features of Riga-Fede disease
Riga-Fede disease usually affects the tip and under surface of the tongue, matching the location of the lower front teeth. Less commonly the top of the tongue is involved due to repeated contact with upper front teeth. The gum opposite a natal/neonatal tooth can be affected. Any location in the mouth can be affected, including the lips and inside the cheeks due to repetitive biting.
Riga-Fede disease begins as an ulcer and it can sometimes look like the tip of the tongue has been eaten away. The base of the ulcer is covered by a yellow fibrinopurulent membrane that can be wiped off. There is usually surrounding redness and often a raised white edge immediately next to the ulcer. Less commonly an ulcerated raised fibrous mass develops.
As the ulcer can be very painful, Riga-Fede disease may affect feeding and therefore dehydration, malnutrition and failure-to-thrive can result if unrecognised and untreated.
Spontaneous recovery can occur.
What investigations should be done?
Histopathology shows an ulcerated surface with an underlying mixed inflammatory infiltrate including lymphocytes, macrophages, mast cells and a predominance of eosinophils. Riga-Fede disease is therefore a cause of eosinophilic ulcer. Atypical histiocytic granulomas may also be seen.
Biopsy may be required to exclude other conditions affecting the tongue including:
- Infections – ulcerative candidiasis, bacterial infections, fungal infections, tuberculosis, primary syphilis
- Malignancies – lymphoma, sarcoma
- Agranulocytosis (blood condition in which there are no white blood cells)
Neurological evaluation may be required to look for one of the reported associations, as Riga Fede disease can be an early sign of a neurological or developmental problem.
Treatment of Riga-Fede disease
Conservative treatment may include:
- Smoothing the rough edges of the teeth
- Protective covering of the teeth e.g. with rounded smooth composite resin, Stomahesive wafers or rings
- Change of feeding method
- Topical cortisone application to the ulcer
Extraction of teeth is rarely indicated and may be complicated by movement of adjacent teeth to fill the space and therefore overcrowding later