Riga Fede disease is a reactive traumatic mucosal disease characterized by persistent ulceration of the oral mucosa. It develops as a result of repetitive trauma of the tongue by the anterior primary teeth during forward and backward movement
]. Although the aspect of the lesion might be impressive, its nature is relatively benign. The lesion was first described by Antonio Riga, an Italian physician, in 1881. Fede, the founder of Italian pediatrics, subsequently published histological studies and additional cases in 1890
]. It has therefore become known as Riga Fede disease. A broad variety of terms have been used to describe Riga Fede disease, such as eosinophilic ulcer of the oral mucosa, sublingual fibrogranuloma, sublingual growth in infants, sublingual ulcer, reparative lesion of the tongue, (neonatal) lingual traumatic ulceration, traumatic atrophic glossitis, and traumatic granuloma of the tongue.
In 1983, Elzay coined the term ‘traumatic ulcerative granuloma with stromal eosinophilia’ (TUGSE) for those chronic ulcerative lesions of the oral mucosa that histopathologically consist mainly of eosinophils
]. As TUGSE and Riga Fede disease have the same histologic features and are often associated with a history of trauma it was suggested by Elzay that they might be considered as one entity. Although TUGSE has been mainly reported to occur in late adulthood, and not restricted in location to the tongue, it may occur in the buccal mucosa, the vestibule, gingiva, or palate, Riga Fede disease is almost exclusively restricted to the tongue. In the present literature review twenty-nine lesions appeared as ulcerations on the ventral surface of the tongue associated with repetitive trauma of the primary lower incisors, three lesions appeared on the dorsal of the tongue caused by trauma of the upper incisors, and three lesions were found on the lower lip. In seven patients the symptoms were seen soon, within two months, after birth. All these cases were associated with (neo)natal teeth. The remaining twenty-seven patients developed lesions after eruption of the lower incisors, at the age of six to twenty-four months, with a mean age of ten months. The male-to-female ratio appeared to be 1.8:1.
Riga Fede disease begins as an ulcerated area with prominent raised edges. With repeated trauma, it may progress to an enlarged, fibrous mass with the appearance of an ulcerative granuloma with superficial necrosis. Based on these characteristics the differential diagnosis of Riga Fede disease should include those entities mentioned in Table
. Once the clinician is familiar with the diagnosis Riga Fede disease, the history and clinical features are most often so typical that there is seldom a need for addititonal histopathological examination. In the present literature review histopathological examination was performed in fourteen cases. In the remaining twenty cases a diagnosis of Riga Fede disease was made on history and clinical features alone. In our patient biopsy was performed because of unawareness of the entity of Riga Fede disease. Riga Fede disease is histopathologically characterized by an ulcerated mucosa with granulation tissue and a mixed inflammatory infiltrate consisting of lymphocytes, macrophages, mast cells and an abundant number of eosinophils, the latter being the most typical of this entity.
Differential diagnosis of ulcerated, indurated masses of the oral mucosa in infancy
In the present literature review one quarter of the patients suffered from a neurologic disorders, i.c. familial dysautonomia, congenital autonomic dysfunction with universal pain loss, Down syndrome, microcephaly, and cerebral palsy. Interestingly, all seven patients with (neo)natal teeth developed Riga Fede disease before the age of six months and did not suffer from neurologic disorders. According to these findings Domingues–Cruz et al. proposed using a classification of the disease wherin ‘precocious Riga fede disease’ defines those occurrences associated with (neo)natal teeth in the first 6
months of life, where no relation with neurologic disorders was found, and ‘late Riga Fede disease’ refers to those instances which typically start after 6–8
months of life, with the first dentition, usually the lower incisors. In the former, the existence of (neo)natal teeth, together with the instinctive sucking reflex and the tendency for the tongue to protrude favor the development of the disease. In the latter, the importance of recognition of the condition is due to its possible relationship to neurologic disease
Several treatments for Riga Fede disease have been described, all of which aim to eliminate the source of trauma so healing can take place. It is preferably to start treatment conservatively such as smoothening off the incisor edges, covering the rough incisor edges with composite resin, changing feeding habits by using a bottle with a larger hole in the nipple, placing a nasogastric tube, or relieving symptoms by application of a local corticosteroid. If conservative methods fail to resolve the lesion, or when the child is severely dehydrated or malnourished extraction of the incisors might be considered. Alternatively, excision of the lesion itself might be performed.