Riga-Fede disease :A rare case report

IntroductionRiga-Fede disease is a traumatic ulceration of ventral surface of tongue, most often associated with natal or neonatal teeth or erupted primary lower incisors1. The self mutilating behaviour is quite common in children with lower intelligence, congenital insensitivity to pain (familial dysautonomia), CIPA (congenital insensitivity to pain with anhidrosis) and with a habit of repetitive tongue thrusting against lower central incisors2.Antonia Riga an Italian physician, was the first to report the malady in 1881, while the first histologic examination of this oral phenomenon was described by Fede in 18903.There has been only 11 cases reported in literature4. Trauma to oral mucosa may result in surface ulceration but most heal within days The ulceration in Riga-Fede disease may remain for a long time, resulting in inadequate food intake, retardation of growth and sometimes results in dehydration, feeding difficulties and failure to thrive in an infant5.Case report An 11 month old female child reported to outpatient department with cleft like deformity of tongue present since 5 months . Her mother reported that an ulcer had appeared  after the  child used to rubbed her tongue against the erupted incisors very often and caused difficulty in feeding. The parents were illiterate, have never visited any paediatrician in the past to assess her medical condition. Patient's mother gave a history of consanguineous marriage and death of her first female child within one year of age which was mentally retarded.On general physical examination  the child  was distressed, weak, malnourished, deaf, mentally retarded by birth, unable to hold her neck and all milestones delayed, continuous uncoordinated movements of hands, feet and abdomen. On consultation with paediatrician, child was  diagnosed with cerebral palsy.On intraoral examination an ulcer was present on the tip of tongue covering dorsal and ventral surface measuring approx 4.5x 3 cm with irregular borders covered with pseudo membranous slough .The ulcer had partially healed creating a cleft like deformity on anterior one third of tongue giving the tongue a bifid appearance(fig 1).The lower central incisors 71, 81 and 51,61,52,62 were erupted. On palpation of ulcer the child did not elicit significant painful responseOn thorough   history and examination the condition was diagnosed as Riga-Fede disease.The child was prescribed topical corticosteroid application and extraction of the mandibular incisors. On consultation with the child's parents it was confirmed that the ulcer was healing, but unfortunately the child died due to high grade fever and an  episode of seizure after 15 days. DiscussionRiga-Fede disease is of particular concern because it is frequently associated with malnourished infants.  And in conditions like severe cerebral palsy they cannot control spasticity of the tongue , hence the repeated ulcerations3,6. Self mutilation or self injurious behaviour is most common in mental retardation, Lesch-Nyhan syndrome7, familial dysautonomia2 and congenital insensitivity to pain. This is a rare disease first to be reported in an Indian child. The Riga-Fede disease persisted for a long time which resulted in inadequate food intake and retardation of growth, dehydration and failure to thrive1. Cases have been reported of Riga- Fede disease associated with natal or neonatal teeth4,5,8, 9,but we report this case associated with erupted primary dentition. Treatment of this disease has varied from excision of the lesion to smoothening of sharp incisal edges8,9 .The  conservative treatment involving use of emollient  corticosteroid orabase1 , and stoma adhesive wafer10 has also have proved beneficial. Behaviour modification like use of protective dental appliances has also been the cornerstone of treatment11. In our case though we had planned the appropriate treatment, the child couldn't  survive. The consequence could be attributed to  poverty, illiteracy of the parents, and failure to seek medical attention for the underlying nutritional deficiency and associated high grade fever and seizures.References:1. Rebecca L. Slayton; Treatment alternatives for sublingual traumatic ulceration (Riga Fede disease) . American Academy of Paediatric Dentistry 22:413-414,2000.2.Meir Rakocz, Mira Frand, Natan Brand. Familial dysautonomia with Riga-Fede disease: report of a case. Journal of dentistry for children 57-59, 1987.3.Ziad D Baghdadi. Riga Fede disease: report of a case and review. Journal of Clinical Paediatric Dentistry 25 (3): 209-213, 2001.4.Ahmet Terzioglu, Ferrech Bingul, Gurean Asian. Lingual traumatic ulceration (Riga Fede disease) Letter to editor. Journal of oral and maxillofacial surgery 60:478, 2002,5.Terzioglu Ahmet, Bingul Ferrech, Asian Gurean. Lingual Traumatic ulceration (Riga Fede disease) Letters to Editor. British Journal of oral and maxillofacial surgery 41: 201-207, 2003.6.Ziad D Baghdadi. Riga-Fede disease: association with microcephaly. International journal of Pediatric Dentistry 12: 442-445, 2002.7.Natan Gadoth, Eliyah Mass. Hereditary neuropathies with self mutilation. J of Pediatric Neurology 2 no 4: 205-211, 2004.8.Hegde RJ. Sublingual traumatic ulceration due to neonatal teeth (Riga- Fede disease). J Indian Soc Pedod Prev Dent 23: 51-52, 2005{cited 2009}9.D Jariwala, R M Graham and T Lewis. Letter . Riga- Fede disease. British Dental Journal 204:171, 2008.10.Susan Buchanan , Cristopher R Jenkins .Riga-Fede syndrome: natal or neonatal teeth associated with tongue ulceration. Case report. Australian dental journal 42 (4):225-227, 2008.11.Brian R Toy. Congenital autonomic dysfunction with universal pain loss (Riga- Fede disease). Dermatology online Journal 7(2): 17, 2001.                                             Fig 1; Intraoral photograph showing Ulceration                       and cleft like  deformity on the ventral surface of tongue.