Open Access

Traumatic lingual ulceration in a newborn: Riga-Fede disease

  • Erik H van der Meij1Email author,
  • Tjalling W de Vries2,
  • Henk F Eggink3 and
  • Jan GAM de Visscher4
Italian Journal of Pediatrics201238:20

DOI: 10.1186/1824-7288-38-20

Received: 27 February 2012

Accepted: 19 April 2012

Published: 23 May 2012

Abstract

Riga Fede disease is a reactive mucosal disease 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 history and clinical features are most often so typical that there is seldom a need for addititonal histopathological examination. Riga Fede disease can most often be treated with conservative measures only.

Beside the presentation of a six-month-old boy with Riga Fede disease, the literature has been reviewed as well. From this review it can be concluded that Riga Fede disease is almost exclusively restricted to the tongue, occurs soon after birth when associated with (neo)natal teeth, has a male predilection, and is in one quarter of the cases associated with neurologic disorders. In the later case, Riga Fede disease develops after the age of 6 months.

Keywords

incisor infant natal teeth oral ulcer tong diseases

Background

Intra-oral tumours in infancy often cause distress in both parents and doctors. The differential diagnosis includes several serious and potential lethal diseases, but also relatively benign disorders. We describe a six-month-old newborn with a benign intra-oral, ulcerating mass mimicking malignancy. The lesion was finally diagnosed as Riga Fede disease. In this treatise, the clinical characteristics, differential diagnosis, histopathological aspects, and treatment options of Riga Fede disease, based on a literature review, will be discussed. This report aims the paediatrician to recognize this entity and to prevent unnecessary invasive procedures.

Case report

A six-month-old boy was referred to the Department of Oral and Maxillofacial Surgery by his dentist because of an ulcerative swelling on the ventral surface of the tongue, noticed by his parents since three months. The lesion seemed not to be painful as there were no feeding difficulties. The relevant medical history did not reveal any abnormalities, especially no neurologic disorders. Family history was negative for developmental disorders and congenital syndromes. The patient did not use any medication at presentation.

Physical examination revealed an indurated, non-tender, ulcerative swelling on the ventral surface of the tongue measuring 1.5 by 1.5 cm. Impressions of the primary lower central incisors were seen in the middle of the lesion (Figure1). On palpation, the lesion seemed to infiltrate deep into the underlying muscle. A close relationship between the tumor and the primary lower central incisors was noticed during swallowing.
https://static-content.springer.com/image/art%3A10.1186%2F1824-7288-38-20/MediaObjects/13052_2012_Article_236_Fig1_HTML.jpg
Figure 1

Indurated, non-tender, ulcerative swelling on the ventral surface of the tongue measuring 1.5 by 1.5 cm. Impressions of the primary lower central incisors were seen in the middle of the lesion.

Excisional biopsy was performed under general anesthesia. The resulting defect was closed primarily. On histopathological examination an ulcerative, inflammatory lesion with granulation tissue was seen. The mixed cellular infiltrate consisted of lymphocytes, neutrophils, plasma cells, and an abundant number of eosinophils (Figure2). Based on the clinicopathological findings a diagnosis of Riga Fede disease was made.
https://static-content.springer.com/image/art%3A10.1186%2F1824-7288-38-20/MediaObjects/13052_2012_Article_236_Fig2_HTML.jpg
Figure 2

Mixed cellular infiltrate consisting of lymphocytes, neutrophils, plasma cells, and an abundant number of eosinophils (haematoxylin-eosin, 10x).

The tongue healed well, and three months post-operatively no signs of recurrence were found.

Review

A literature search was performed of all cases of Riga Fede disease that were published in the English literature using the databases of PubMed, Cochrane, and Sum Search. A summary of all these reported cases is shown in Table1[129].
Table 1

Summary of all reported cases of patients with Riga Fede disease [[129] ]

   

AGE

SITE

(NEO)NATAL

BIOPSY

ASSOCIATED

TREATMENT

AUTHORS

YEAR

GENDER

(MONTHS)

TEETH

DISORDERS

Amberg[1]

1902

M

7

sublingual

no

yes

no

excision

Bray[2]

1927

M

9

sublingual

no

yes

no

excision

Bradley[3]

1932

F

8

sublingual

no

yes

no

excision

Moncrieff[4]

1933

M

6

sublingual

no

yes

no

weaning

Newman[5]

1935

M

6

sublingual

no

no

mentally deficient

smoothening lower incisors

M

8

dorsum of tongue

no

no

no

extraction

Abramson[6]

1944

F

11

sublingual

no

yes

no

excision

F

9

sublingual

no

yes

no

excision

Jacobs[7]

1956

unknown

0.3

sublingual

yes

no

no

extraction

McDaniel et al.[8]

1978

M

6

dorsum of tongue

no

yes

no

excision

Rakocz[9]

1987

M

10

base and dorsum

no

yes

FD*

composite coverage incisors

    

of tongue

    

Eichenfield et al.[10]

1990

F

6

sublingual

no

yes

FD*

none

Goho[11]

1996

F

0.7

sublingual

yes

no

no

extraction

F

0.3

sublingual

yes

no

no

composite coverage incisors

Uzamiş[12]

1999

M

2

sublingual

yes

no

no

extraction

Slayton[13]

2000

M

10

sublingual

no

no

Down

 
       

syndrome

smoothening lower incisors

Toy[14]

2001

M

20

sublingual

no

yes

CADUPL**

unknown

    

lower lip

    

Baghdadi[15]

2001

M

10

sublingual

no

no

no

smoothening lower incisors

        

topical corticosteroid

Baghdadi[16]

2002

F

12

sublingual

no

no

microcephaly

smoothening lower incisors

        

topical corticosteroid

Terzioğlu et al.[17]

2002

M

7

sublingual

no

no

no

none

Zaenglein et al.[18]

2002

M

10

tongue

no

yes

CADUPL**

unknown

    

lower lip

    

Ahmet et al.[19]

2003

F

9

sublingual

no

unknown

no

none

Hegde[20]

2005

F

1

sublingual

yes

no

no

extraction

Campos-Muñoz et al.[21]

2006

M

11

sublingual

no

no

no

nasogastric feeding tube

Baroni et al.[22]

2006

M

11

sublingual

no

no

no

topical odontologic cream

        

teething ring

Domingues-Cruz[23]

2007

M

24

lower lip

no

no

Down

extraction

       

syndrome

 

Narang et al.[24]

2008

M

9

sublingual

no

no

no

teething ring

        

release of tongue tie

Jariwala et al.[25]

2008

F

1.5

sublingual

yes

no

no

extraction

Ceyhan et al.[26]

2009

M

15

sublingual

no

no

no

topical corticosteroid

Taghi et al.[27]

2009

M

8

sublingual

no

yes

cerebral palsy

composite coverage incisors

Choi et al.[28]

2009

M

8

sublingual

no

no

no

composite coverage incisors

F

2

sublingual

yes

no

no

smoothening incisal edges

Eley et al.[29]

2010

F

11

sublingual

no

yes

no

excision

van der Meij et al.

2012

M

6

sublingual

no

yes

no

excision

* familial dysautonomia.

**congenital autonomic dysfunction with universal pain loss.

Discussion

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[26]. 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[1]. 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[30]. 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 Table2. 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.
Table 2

Differential diagnosis of ulcerated, indurated masses of the oral mucosa in infancy

LOCAL NEOPLASIA

· granular cell tumour

· myofibroma

· sarcoma

· extra-nodal lymphoma

INFECTION

· congenital syphilis

· tuberculosis

HEMATOLOGICAL DISORDER

· agranulocytosis

TRAUMATIC

· mechanical (Riga Fede disease)

· electrical

· chemical

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[23].

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.

Conclusion

In conclusion, Riga Fede disease is a reactive mucosal disease 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 history and clinical features are most often so typical that there is seldom a need for addititonal histopathological examination. Riga Fede disease can most often be treated with conservative measures only.

Consent

Written informed consent was obtained from the parents/ guardians of the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Funding

This research received no specific funding.

Declarations

Authors’ Affiliations

(1)
Department of Oral and Maxillofacial Surgery, Medical Center Leeuwarden
(2)
Department of Paediatrics, Medical Center Leeuwarden
(3)
Departmant of Pathology, Medical Center Leeuwarden
(4)
Department of Oral and Maxillofacial Surgery, Medical Center Leeuwarden

References

  1. Amberg S: Sublingual growth in infants. Am J Med Sci. 1902, 126: 257-69.View ArticleGoogle Scholar
  2. Bray CM: Riga’s disease (Cachectic Aphthae). W Va Med J. 1927, 23: 249-50.Google Scholar
  3. Bradley DJ: Sublingual growth-Riga’s or Fede’s disease. J Med. 1932, 13: 473-74.Google Scholar
  4. Moncrieff A: Sublingual ulcer: with special reference to Tiga’s disease. Br J Child Dis. 1933, 30: 268-74.Google Scholar
  5. Newman PH: A case of double Riga’s disease. Br J Child Dis. 1935, 32: 39-41.Google Scholar
  6. Abramson M, Dowrie JO: Sublingual granuloma in infancy (Riga-Fede’s disease). J Pediatr. 1944, 24: 195-98. 10.1016/S0022-3476(44)80125-1.View ArticleGoogle Scholar
  7. Jacobs M: Oral lesions in childhood. Oral Surg. 1956, 9: 871-81.View ArticlePubMedGoogle Scholar
  8. McDaniel RK, Morano PD: Reparative lesion of the tongue. Oral Surg. Oral Surg. 1978, 45: 266-71.View ArticlePubMedGoogle Scholar
  9. Rakocz M, Frand M, Brand N: Familial dysautonomia with Riga-Fede's disease: report of case. ASDC J Dent Child. 1987, 54: 57-9.PubMedGoogle Scholar
  10. Eichenfield LF, Honig PJ, Nelson L: Traumatic granuloma of the tongue (Riga-Fede disease): association with familial dysautonomia. J Pediatr. 1990, 116: 742-4. 10.1016/S0022-3476(05)82663-0.View ArticlePubMedGoogle Scholar
  11. Goho C: Neonatal sublingual traumatic ulceration (Riga-Fede disease): reports of cases. ASDC J Dent Child. 1996, 63: 362-4.PubMedGoogle Scholar
  12. Uzamiş M, Turgut M, Olmez S: Neonatal sublingual traumatic ulceration (Riga-Fede disease): a case report. Turk J Pediatr. 1999, 41: 113-6.PubMedGoogle Scholar
  13. Slayton RL: Treatment alternatives for sublingual traumatic ulceration (Riga-Fede disease). Pediatr Dent. 2000, 22: 413-4.PubMedGoogle Scholar
  14. Toy BR: Congenital autonomic dysfunction with universal pain loss (Riga-Fede disease). Dermatol Online J. 2001, 7: 17-PubMedGoogle Scholar
  15. Baghdadi ZD: Riga-Fede disease: report of a case and review. J Clin Pediatr Dent. 2001, 25: 209-13.View ArticlePubMedGoogle Scholar
  16. Baghdadi ZD: Riga-Fede disease: association with microcephaly. Int J Paediatr Dent. 2002, 12: 442-5. 10.1046/j.1365-263X.2002.00396.x.View ArticlePubMedGoogle Scholar
  17. Terzioğlu A, Bingül F, Aslan G: Lingual traumatic ulceration (Riga-Fede disease). J Oral Maxillofac Surg. 2002, 60: 478-10.1053/joms.2002.32070.View ArticlePubMedGoogle Scholar
  18. Zaenglein AL, Chang MW, Meehan SA, Axelrod FB, Orlow SJ: Extensive Riga-Fede disease of the lip and tongue. J Am Acad Dermatol. 2002, 47: 445-7. 10.1067/mjd.2002.117213.View ArticlePubMedGoogle Scholar
  19. Ahmet T, Ferruh B, Gürcan A: Lingual traumatic ulceration (Riga-Fede disease). Br J Oral Maxillofac Surg. 2003, 41: 201-View ArticlePubMedGoogle Scholar
  20. Hegde RJ: Sublingual traumatic ulceration due to neonatal teeth (Riga-Fede disease). J Indian Soc Pedo Prev Dent. 2005, 23: 51-52.View ArticleGoogle Scholar
  21. Campos-Muñoz L, Quesada-Cortés A, Corral-De la Calle M, Arranz-Sánchez D, Gonzalez-Beato MJ, De Lucas R, Vidaurrázaga C: Tongue ulcer in a child: Riga-Fede disease. J Eur Acad Dermatol Venereol. 2006, 20: 1357-9. 10.1111/j.1468-3083.2006.01715.x.View ArticlePubMedGoogle Scholar
  22. Baroni A, Capristo C, Rossiello L, Faccenda F, Satriano RA: Lingual traumatic ulceration (Riga-Fede disease). Int J Dermatol. 2006, 45: 1096-7. 10.1111/j.1365-4632.2004.02554.x.View ArticlePubMedGoogle Scholar
  23. Domingues-Cruz J, Herrera A, Fernandez-Crehuet P, Garcia-Bravo B, Camacho F: Riga-Fede disease associated with postanoxic encephalopathy and trisomy 21: a proposed classification. Pediatr Dermatol. 2007, 24: 663-5. 10.1111/j.1525-1470.2007.00564.x.View ArticlePubMedGoogle Scholar
  24. Narang T, De D, Kanwar AJ: Riga-Fede disease: trauma due to teeth or tongue tie?. J Eur Acad Dermatol Venereol. 2008, 22: 395-6. 10.1111/j.1468-3083.2007.02347.x.View ArticlePubMedGoogle Scholar
  25. Jariwala D, Graham RM, Lewis T: Riga-Fede disease. Br Dent J. 2008, 204: 171-10.1038/bdj.2008.113.View ArticlePubMedGoogle Scholar
  26. Ceyhan AM, Yildirim M, Basak PY, Akkaya VB, Ayata A: Traumatic lingual ulcer in a child. Clin Exp Dermatol. 2009, 34: 186-8. 10.1111/j.1365-2230.2008.02796.x.View ArticlePubMedGoogle Scholar
  27. Taghi A, Motamedi MHK: Riga-Fede disease: a histological study and case report. Indian J Dent Research. 2009, 20: 227-9. 10.4103/0970-9290.52893.View ArticleGoogle Scholar
  28. Choi SC, Park JH, Choi YC, Kim GT: Sublingual traumatic ulceration (a Riga-Fede disease): report of two cases. Dental Traumatol. 2009, 25: 48-50. 10.1111/j.1600-9657.2009.00773.x.View ArticleGoogle Scholar
  29. Eley KA, Watt-Smith PA, Watt-Smith SR: Deformity of the tongue in an infant: Riga- Fede disease. Paediatr Child Health. 2010, 15: 581-82.PubMed CentralPubMedGoogle Scholar
  30. Elzay RP: Traumatic ulcerative granuloma with stromal eosinophilia (Riga-Fede’s disease and traumatic eosinophilic granuloma). Oral Surg Oral Med Oral Pathol. 1983, 55: 497-506. 10.1016/0030-4220(83)90236-0.View ArticlePubMedGoogle Scholar

Copyright

© van der Meij et al.; licensee BioMed Central Ltd. 2012

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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