Riga-Fede Disease With Hematemesis After Breastfeeding

An 8-month-old male infant presented with hematemesis that had occurred after breastfeeding 2 months prior. However, the infant showed a normal nutritional status and normal body height. To investigate the source of the hematemesis, a pediatrician at the postnatal care center consulted an oral and maxillofacial surgeon. A 12 × 10 mm round, yellowish ulcer with induration on the midline of the ventral tongue above the sublingual fold was noted. Four lower deciduous incisors erupted, and their edges were sharp (Fig. 1). Riga-Fede disease, characterized as a traumatic ulcerative granuloma resulting from lower deciduous incisors, was diagnosed, with a differential diagnosis that included malignancies such as squamous cell carcinoma. Exfoliative cytology revealed a class II result according to the Papanicolaou classification, indicating the presence of minor atypical cells but no evidence of malignancy.1 The sharp edges of the 4 lower deciduous incisors were smoothed using a high-speed handpiece on a pediatric restrainer without anesthesia. One month after the procedure, bleeding ceased, and the ulcer resolved within 3 months. Riga-Fede disease, initially reported by Riga in 1881 and Fede in 1890, involves a traumatic ulcerative granuloma associated with natal teeth or early-erupted lower incisors.2 A study conducted in Hong Kong has determined that the prevalence of natal teeth is 1 in 1118, with 6% of these cases exhibiting a traumatized ventral surface of the tongue, necessitating extraction. This finding suggests that the incidence of Riga-Fede disease is 1 in 17,893.3 The variations in regional prevalence remain undetermined. Oral ulcers may also be caused by drug-induced conditions (eg, Stevens–Johnson syndrome), systemic diseases (eg, Behçet disease), viral infections (eg, herpes simplex virus), and recurrent aphthous stomatitis. Autoimmune bullous diseases, such as mucous membrane pemphigoid and pemphigus vulgaris, also present with multiple erosions and ulcers due to the easy rupture of bullae.4 However, these conditions primarily manifest as multiple painful, flat ulcers. Stomatitis typically presents as a single painful ulcer without granuloma formation. Riga-Fede disease is readily diagnosed by the presence of an ulcer with granuloma attached to the lower deciduous incisors. The clinical presentation of Riga-Fede disease may resemble that of squamous cell carcinoma as the ulcer exhibits induration. If no lower deciduous incisors are present, a biopsy is necessary. Treatment of Riga-Fede disease involves eliminating chronic irritation using methods such as smoothing the incisor edges, capping with resin, and using an occlusal plate. In cases of high dental mobility, which increase the risk of dental avulsion and potential tooth ingestion or inhalation, extraction is the preferred therapeutic approach. When nutrition is not compromised and the lower incisor is stable, smoothing the incisor edges is the treatment of choice due to its less invasive nature.2Fig. 1.: Intraoral photograph of an 8-month-old male infant. A 12 × 10 mm, round, yellowish ulcer with induration is observed on the midline of the ventral tongue, above the sublingual fold. Four lower deciduous incisors with sharp edges have erupted.DISCLOSURE The authors have no financial interest to declare in relation to the content of this article. PATIENT CONSENT Patient consent was obtained to publish the study. Written consent was also obtained.