Patient Presentation
A 44-year-old man presented with an asymptomatic change of the dorsum of the tongue, which he called “a bit unsightly appearing.” On examination of the oral cavity, there was a raised oval–to-rhomboidal-shaped pinkish area with a shiny smooth surface localized to the medial aspect of the dorsum of the tongue. There were no other abnormal findings on examination of the oral mucosa or the rest of the skin. Family history was non-contributory.
What’s Your Diagnosis?
Diagnosis: Median Rhomboid Glossitis
Median rhomboid glossitis (MRG) is an oral lesion that is believed to be a developmental anomaly due to the persistence of a midline embryonic structure known as the tuberculum impar.1,2 The traditional developmental etiology of the entrapment of the tuberculum impar between the lateral halves of the tongue adequately explains the typical midline location, size, and lack of filiform papillae.3
Currently, the etiology of MRG is no longer thought to be a developmental anomaly, but rather a persistent inflammatory process secondary to chronic infection by Candida albicans,1,4,5 with a location anterior to the circumvallate papillae at the junction between the anterior two-thirds and posterior one-third of the tongue, at the midline of the dorsum. Indeed, it is far more probable that the lesion is an inflammatory process, infectious process, or even a degenerative process.3 Wright and Fenwick postulated that MRG is the clinical manifestation of a localized chronic fungal infection of the Candida species.4 Although generally benign in nature, there have been reports of carcinoma arising from MRG.6
History
Included in the earliest literature on MRG is a series of 17 cases presented in 1914 by Brocq and Pautrier under the name glossite losangique médiane de la face dorsale de la langue.3,7 In 1924, Lane shortened the name to glossitis rhombica mediana.8 We are more familiar with the anglicanized counterpart of Lane’s term: median rhomboid glossitis.
Subsequently, Loos and Horbst were the first to postulate an etiology based on an embryonic study of the tongue in 1934 in their extensively detailed reports on eight patients.9 Nonetheless, Martin and Howe were credited with promulgating the once-accepted traditional developmental/embryonic theory in 1938, proposing a connection with the persistence of the tuberculum impar developmentally.10,11 Eventually, with a dearth of congenital cases and mounting evidence for an inflammatory/infectious etiology, the belief in the persistence of the tuberculum impar gave way to the theory of a localized candidal infection.3,12
In 1965, Cernea and colleagues published a case series of 16 patients with multifocal oral candidosis in which 13 of the 16 patients had MRG. Cernea and his co-authors compared these cases with Brocq and Pautrier’s original patients.13 Furthermore, they were able to culture Candida albicans from all of the tongue lesions.13
In 1975, Cooke published a case series of 10 patients with MRG, all of whom had fungal hyphae in the keratin layer of the tongue on histologic sections.14 Farman and Nutt published a paper on atrophic tongue lesions and diabetes, affirming that while a cause-and-effect relationship between the atrophic tongue lesions and the candida species was not confirmed, there was considerable statistical correlation between the two.15
In 1978, Wright studied 28 patients in whom 24 (85%) demonstrated the presence of fungal hyphae in two or more serial sections;4 the one patient who did not show all of the histologic features generally associated with MRG did reveal the presence of numerous yeast cells and hyphae.4
Clinical Presentation
MRG is typically benign, rhomboid or oval-shaped, present on the dorsum of the tongue, and midline in location.11 The following characteristics are usually noted as well:
- The lesion is usually raised and situated anterior to the “V” formed by the circumvallate papillae
- The lesion is distinct from the normal tongue surface by being devoid of papillae, with a brownish-red color consistent with the presence of mild inflammation.11
- Furthermore, the lesion is classically asymptomatic except for some mild erythema or inflammatory reaction.11
In 1939, Sammett developed the following six diagnostic qualities of MRG that are applicable:
1. the midline location in the median raphe of the dorsum of the tongue, anterior to the vallate papillae
2. a rhomboid shape
3. its red color
4. the smooth, fissured, or nodular appearance, devoid of papillae
5. erythema
6. its asymptomatic nature and chance discovery.16
MRG typically presents in males in their fourth decade,11 with an age range from 15 years (a patient described by Brocq and Pautrier) to 60 years of age (reported by Sammett).7,16
Mechanism
The pathogenic mechanism in MRG could include a direct toxic action or a hypersensitivity response;17 such factors may include toxins or hydrolytic enzymes that are derived from some pathogenic strains of C. albicans.18 The palatal inflammation would be initiated by the continual exposure of the mucosa to candidotoxins resulting from the infection.17 Evidence supporting this proposition has been established in experimental attempts to produce candidiasis by placing aqueous suspensions of Candida species in the hamster cheek pouch.19 These topical applications consistently produced a highly inflamed mucosa without infestation by hyphae, indicating that the inflammation was mediated by factors produced by the Candida species.19
Because MRG is causally related to candidal infections, the workup related to the expression of the candidal hyphae is fairly straight-forward.
After taking a careful history with special attention to the signs and symptoms of oral candidiasis, an accurate description of the clinical lesion is critical, as MRG can have multiple presentations.20
Candida hyphae can be established with periodic acid-Schiff (PAS) staining of a cytology smear of the pseudomembrane.20 Further delineation of the diagnosis by quantifying the fungal burden can be useful, because the normal carriage in 50% of the population is less than 1000 CFU/ml, yet in infected patients, the burden may range from 4000 to 20,000 CFU/ml.20
For potential cases of chronic hyperplastic candidosis or MRG, the biopsy of relevant tissues is the most exact way to confirm the diagnosis.20 Typically, biopsy results demonstrate hyperparakeratosis, with vertically invading hyphae that are PAS or silver stain positive.20
Differential Diagnosis
The clinical significance of this benign lesion is to recognize it and distinguish it from carcinoma.11 (See Table 1.) Sometimes a biopsy or an excision may be needed to allay the concerns or fears of the patient wary of cancer.11 However, once the attention of the examiner is drawn to this condition, a precise diagnosis is possible.11
Management
The management of MRG is akin to that of other oral candidal infections.
Topical antifungals are the first line of treatment, delivered as troches, rinses, or creams, with systemic antifungals reserved for refractory or extensive lesions, although they increase the risk of adverse effects and drug interactions.20
Most C. albicans infections may be simply treated with topical applications of the polyenes (nystatin and amphotericin),22 or specifically, with nystatin oral suspension (100,000 U/ml) or nystatin pastille (100,000 IU) four times daily after meals.22
Amphotericin suspensions (100 mg/ml) or amphotericin lozenges (10 mg) four times daily after meals are also effective.22
At times, MRG may require systemic administration of ketoconazole, fluconazole, or itraconazole.22
Prognosis and Risk of Malignancy
MRG is an infrequent but not rare anomaly of the dorsum of the tongue. At best, carcinoma of the dorsal surface of the tongue is an extremely rare phenomenon.4,11 The prognosis for patients with MRG is exceptionally positive with little cause for alarm on behalf of the patient and clinician.3
However, in the literature on MRG, there were two reported patients with carcinomatous transformations in lesions.3 Specifically, Sharp and Bullock’s patient was a 51-year-old woman who had no node involvement and was free of disease 2 years after radium needle treatment,23 whereas Ol’Shanetskii’s patient was a 48-year-old man who again had no node involvement but had the lesion excised with recurrence, followed by treatment with radiation and surgery.24 At best, carcinoma of the dorsal surface of the tongue is extremely rare and is usually associated with syphilitic glossitis.25
Conclusion
Once believed to be a developmental anomaly of the tuberculum impar, MRG is currently considered to be a chronic inflammatory process as a result of candidal infection. Diagnosis can be confirmed with a cytology smear and quantification of the fungal burden, and treatment should follow the guidelines of oral candidal infections, with topical or systemic antifungals.
Our patient was treated with a topical “swish and spit” antifungal, and advised to keep the area clean. Three months later, upon follow up examination, the lesion had healed completely.
Patient Presentation
A 44-year-old man presented with an asymptomatic change of the dorsum of the tongue, which he called “a bit unsightly appearing.” On examination of the oral cavity, there was a raised oval–to-rhomboidal-shaped pinkish area with a shiny smooth surface localized to the medial aspect of the dorsum of the tongue. There were no other abnormal findings on examination of the oral mucosa or the rest of the skin. Family history was non-contributory.
What’s Your Diagnosis?
Diagnosis: Median Rhomboid Glossitis
Median rhomboid glossitis (MRG) is an oral lesion that is believed to be a developmental anomaly due to the persistence of a midline embryonic structure known as the tuberculum impar.1,2 The traditional developmental etiology of the entrapment of the tuberculum impar between the lateral halves of the tongue adequately explains the typical midline location, size, and lack of filiform papillae.3
Currently, the etiology of MRG is no longer thought to be a developmental anomaly, but rather a persistent inflammatory process secondary to chronic infection by Candida albicans,1,4,5 with a location anterior to the circumvallate papillae at the junction between the anterior two-thirds and posterior one-third of the tongue, at the midline of the dorsum. Indeed, it is far more probable that the lesion is an inflammatory process, infectious process, or even a degenerative process.3 Wright and Fenwick postulated that MRG is the clinical manifestation of a localized chronic fungal infection of the Candida species.4 Although generally benign in nature, there have been reports of carcinoma arising from MRG.6
History
Included in the earliest literature on MRG is a series of 17 cases presented in 1914 by Brocq and Pautrier under the name glossite losangique médiane de la face dorsale de la langue.3,7 In 1924, Lane shortened the name to glossitis rhombica mediana.8 We are more familiar with the anglicanized counterpart of Lane’s term: median rhomboid glossitis.
Subsequently, Loos and Horbst were the first to postulate an etiology based on an embryonic study of the tongue in 1934 in their extensively detailed reports on eight patients.9 Nonetheless, Martin and Howe were credited with promulgating the once-accepted traditional developmental/embryonic theory in 1938, proposing a connection with the persistence of the tuberculum impar developmentally.10,11 Eventually, with a dearth of congenital cases and mounting evidence for an inflammatory/infectious etiology, the belief in the persistence of the tuberculum impar gave way to the theory of a localized candidal infection.3,12
In 1965, Cernea and colleagues published a case series of 16 patients with multifocal oral candidosis in which 13 of the 16 patients had MRG. Cernea and his co-authors compared these cases with Brocq and Pautrier’s original patients.13 Furthermore, they were able to culture Candida albicans from all of the tongue lesions.13
In 1975, Cooke published a case series of 10 patients with MRG, all of whom had fungal hyphae in the keratin layer of the tongue on histologic sections.14 Farman and Nutt published a paper on atrophic tongue lesions and diabetes, affirming that while a cause-and-effect relationship between the atrophic tongue lesions and the candida species was not confirmed, there was considerable statistical correlation between the two.15
In 1978, Wright studied 28 patients in whom 24 (85%) demonstrated the presence of fungal hyphae in two or more serial sections;4 the one patient who did not show all of the histologic features generally associated with MRG did reveal the presence of numerous yeast cells and hyphae.4
Clinical Presentation
MRG is typically benign, rhomboid or oval-shaped, present on the dorsum of the tongue, and midline in location.11 The following characteristics are usually noted as well:
- The lesion is usually raised and situated anterior to the “V” formed by the circumvallate papillae
- The lesion is distinct from the normal tongue surface by being devoid of papillae, with a brownish-red color consistent with the presence of mild inflammation.11
- Furthermore, the lesion is classically asymptomatic except for some mild erythema or inflammatory reaction.11
In 1939, Sammett developed the following six diagnostic qualities of MRG that are applicable:
1. the midline location in the median raphe of the dorsum of the tongue, anterior to the vallate papillae
2. a rhomboid shape
3. its red color
4. the smooth, fissured, or nodular appearance, devoid of papillae
5. erythema
6. its asymptomatic nature and chance discovery.16
MRG typically presents in males in their fourth decade,11 with an age range from 15 years (a patient described by Brocq and Pautrier) to 60 years of age (reported by Sammett).7,16
Mechanism
The pathogenic mechanism in MRG could include a direct toxic action or a hypersensitivity response;17 such factors may include toxins or hydrolytic enzymes that are derived from some pathogenic strains of C. albicans.18 The palatal inflammation would be initiated by the continual exposure of the mucosa to candidotoxins resulting from the infection.17 Evidence supporting this proposition has been established in experimental attempts to produce candidiasis by placing aqueous suspensions of Candida species in the hamster cheek pouch.19 These topical applications consistently produced a highly inflamed mucosa without infestation by hyphae, indicating that the inflammation was mediated by factors produced by the Candida species.19
Because MRG is causally related to candidal infections, the workup related to the expression of the candidal hyphae is fairly straight-forward.
After taking a careful history with special attention to the signs and symptoms of oral candidiasis, an accurate description of the clinical lesion is critical, as MRG can have multiple presentations.20
Candida hyphae can be established with periodic acid-Schiff (PAS) staining of a cytology smear of the pseudomembrane.20 Further delineation of the diagnosis by quantifying the fungal burden can be useful, because the normal carriage in 50% of the population is less than 1000 CFU/ml, yet in infected patients, the burden may range from 4000 to 20,000 CFU/ml.20
For potential cases of chronic hyperplastic candidosis or MRG, the biopsy of relevant tissues is the most exact way to confirm the diagnosis.20 Typically, biopsy results demonstrate hyperparakeratosis, with vertically invading hyphae that are PAS or silver stain positive.20
Differential Diagnosis
The clinical significance of this benign lesion is to recognize it and distinguish it from carcinoma.11 (See Table 1.) Sometimes a biopsy or an excision may be needed to allay the concerns or fears of the patient wary of cancer.11 However, once the attention of the examiner is drawn to this condition, a precise diagnosis is possible.11
Management
The management of MRG is akin to that of other oral candidal infections.
Topical antifungals are the first line of treatment, delivered as troches, rinses, or creams, with systemic antifungals reserved for refractory or extensive lesions, although they increase the risk of adverse effects and drug interactions.20
Most C. albicans infections may be simply treated with topical applications of the polyenes (nystatin and amphotericin),22 or specifically, with nystatin oral suspension (100,000 U/ml) or nystatin pastille (100,000 IU) four times daily after meals.22
Amphotericin suspensions (100 mg/ml) or amphotericin lozenges (10 mg) four times daily after meals are also effective.22
At times, MRG may require systemic administration of ketoconazole, fluconazole, or itraconazole.22
Prognosis and Risk of Malignancy
MRG is an infrequent but not rare anomaly of the dorsum of the tongue. At best, carcinoma of the dorsal surface of the tongue is an extremely rare phenomenon.4,11 The prognosis for patients with MRG is exceptionally positive with little cause for alarm on behalf of the patient and clinician.3
However, in the literature on MRG, there were two reported patients with carcinomatous transformations in lesions.3 Specifically, Sharp and Bullock’s patient was a 51-year-old woman who had no node involvement and was free of disease 2 years after radium needle treatment,23 whereas Ol’Shanetskii’s patient was a 48-year-old man who again had no node involvement but had the lesion excised with recurrence, followed by treatment with radiation and surgery.24 At best, carcinoma of the dorsal surface of the tongue is extremely rare and is usually associated with syphilitic glossitis.25
Conclusion
Once believed to be a developmental anomaly of the tuberculum impar, MRG is currently considered to be a chronic inflammatory process as a result of candidal infection. Diagnosis can be confirmed with a cytology smear and quantification of the fungal burden, and treatment should follow the guidelines of oral candidal infections, with topical or systemic antifungals.
Our patient was treated with a topical “swish and spit” antifungal, and advised to keep the area clean. Three months later, upon follow up examination, the lesion had healed completely.
Patient Presentation
A 44-year-old man presented with an asymptomatic change of the dorsum of the tongue, which he called “a bit unsightly appearing.” On examination of the oral cavity, there was a raised oval–to-rhomboidal-shaped pinkish area with a shiny smooth surface localized to the medial aspect of the dorsum of the tongue. There were no other abnormal findings on examination of the oral mucosa or the rest of the skin. Family history was non-contributory.
What’s Your Diagnosis?
Diagnosis: Median Rhomboid Glossitis
Median rhomboid glossitis (MRG) is an oral lesion that is believed to be a developmental anomaly due to the persistence of a midline embryonic structure known as the tuberculum impar.1,2 The traditional developmental etiology of the entrapment of the tuberculum impar between the lateral halves of the tongue adequately explains the typical midline location, size, and lack of filiform papillae.3
Currently, the etiology of MRG is no longer thought to be a developmental anomaly, but rather a persistent inflammatory process secondary to chronic infection by Candida albicans,1,4,5 with a location anterior to the circumvallate papillae at the junction between the anterior two-thirds and posterior one-third of the tongue, at the midline of the dorsum. Indeed, it is far more probable that the lesion is an inflammatory process, infectious process, or even a degenerative process.3 Wright and Fenwick postulated that MRG is the clinical manifestation of a localized chronic fungal infection of the Candida species.4 Although generally benign in nature, there have been reports of carcinoma arising from MRG.6
History
Included in the earliest literature on MRG is a series of 17 cases presented in 1914 by Brocq and Pautrier under the name glossite losangique médiane de la face dorsale de la langue.3,7 In 1924, Lane shortened the name to glossitis rhombica mediana.8 We are more familiar with the anglicanized counterpart of Lane’s term: median rhomboid glossitis.
Subsequently, Loos and Horbst were the first to postulate an etiology based on an embryonic study of the tongue in 1934 in their extensively detailed reports on eight patients.9 Nonetheless, Martin and Howe were credited with promulgating the once-accepted traditional developmental/embryonic theory in 1938, proposing a connection with the persistence of the tuberculum impar developmentally.10,11 Eventually, with a dearth of congenital cases and mounting evidence for an inflammatory/infectious etiology, the belief in the persistence of the tuberculum impar gave way to the theory of a localized candidal infection.3,12
In 1965, Cernea and colleagues published a case series of 16 patients with multifocal oral candidosis in which 13 of the 16 patients had MRG. Cernea and his co-authors compared these cases with Brocq and Pautrier’s original patients.13 Furthermore, they were able to culture Candida albicans from all of the tongue lesions.13
In 1975, Cooke published a case series of 10 patients with MRG, all of whom had fungal hyphae in the keratin layer of the tongue on histologic sections.14 Farman and Nutt published a paper on atrophic tongue lesions and diabetes, affirming that while a cause-and-effect relationship between the atrophic tongue lesions and the candida species was not confirmed, there was considerable statistical correlation between the two.15
In 1978, Wright studied 28 patients in whom 24 (85%) demonstrated the presence of fungal hyphae in two or more serial sections;4 the one patient who did not show all of the histologic features generally associated with MRG did reveal the presence of numerous yeast cells and hyphae.4
Clinical Presentation
MRG is typically benign, rhomboid or oval-shaped, present on the dorsum of the tongue, and midline in location.11 The following characteristics are usually noted as well:
- The lesion is usually raised and situated anterior to the “V” formed by the circumvallate papillae
- The lesion is distinct from the normal tongue surface by being devoid of papillae, with a brownish-red color consistent with the presence of mild inflammation.11
- Furthermore, the lesion is classically asymptomatic except for some mild erythema or inflammatory reaction.11
In 1939, Sammett developed the following six diagnostic qualities of MRG that are applicable:
1. the midline location in the median raphe of the dorsum of the tongue, anterior to the vallate papillae
2. a rhomboid shape
3. its red color
4. the smooth, fissured, or nodular appearance, devoid of papillae
5. erythema
6. its asymptomatic nature and chance discovery.16
MRG typically presents in males in their fourth decade,11 with an age range from 15 years (a patient described by Brocq and Pautrier) to 60 years of age (reported by Sammett).7,16
Mechanism
The pathogenic mechanism in MRG could include a direct toxic action or a hypersensitivity response;17 such factors may include toxins or hydrolytic enzymes that are derived from some pathogenic strains of C. albicans.18 The palatal inflammation would be initiated by the continual exposure of the mucosa to candidotoxins resulting from the infection.17 Evidence supporting this proposition has been established in experimental attempts to produce candidiasis by placing aqueous suspensions of Candida species in the hamster cheek pouch.19 These topical applications consistently produced a highly inflamed mucosa without infestation by hyphae, indicating that the inflammation was mediated by factors produced by the Candida species.19
Because MRG is causally related to candidal infections, the workup related to the expression of the candidal hyphae is fairly straight-forward.
After taking a careful history with special attention to the signs and symptoms of oral candidiasis, an accurate description of the clinical lesion is critical, as MRG can have multiple presentations.20
Candida hyphae can be established with periodic acid-Schiff (PAS) staining of a cytology smear of the pseudomembrane.20 Further delineation of the diagnosis by quantifying the fungal burden can be useful, because the normal carriage in 50% of the population is less than 1000 CFU/ml, yet in infected patients, the burden may range from 4000 to 20,000 CFU/ml.20
For potential cases of chronic hyperplastic candidosis or MRG, the biopsy of relevant tissues is the most exact way to confirm the diagnosis.20 Typically, biopsy results demonstrate hyperparakeratosis, with vertically invading hyphae that are PAS or silver stain positive.20
Differential Diagnosis
The clinical significance of this benign lesion is to recognize it and distinguish it from carcinoma.11 (See Table 1.) Sometimes a biopsy or an excision may be needed to allay the concerns or fears of the patient wary of cancer.11 However, once the attention of the examiner is drawn to this condition, a precise diagnosis is possible.11
Management
The management of MRG is akin to that of other oral candidal infections.
Topical antifungals are the first line of treatment, delivered as troches, rinses, or creams, with systemic antifungals reserved for refractory or extensive lesions, although they increase the risk of adverse effects and drug interactions.20
Most C. albicans infections may be simply treated with topical applications of the polyenes (nystatin and amphotericin),22 or specifically, with nystatin oral suspension (100,000 U/ml) or nystatin pastille (100,000 IU) four times daily after meals.22
Amphotericin suspensions (100 mg/ml) or amphotericin lozenges (10 mg) four times daily after meals are also effective.22
At times, MRG may require systemic administration of ketoconazole, fluconazole, or itraconazole.22
Prognosis and Risk of Malignancy
MRG is an infrequent but not rare anomaly of the dorsum of the tongue. At best, carcinoma of the dorsal surface of the tongue is an extremely rare phenomenon.4,11 The prognosis for patients with MRG is exceptionally positive with little cause for alarm on behalf of the patient and clinician.3
However, in the literature on MRG, there were two reported patients with carcinomatous transformations in lesions.3 Specifically, Sharp and Bullock’s patient was a 51-year-old woman who had no node involvement and was free of disease 2 years after radium needle treatment,23 whereas Ol’Shanetskii’s patient was a 48-year-old man who again had no node involvement but had the lesion excised with recurrence, followed by treatment with radiation and surgery.24 At best, carcinoma of the dorsal surface of the tongue is extremely rare and is usually associated with syphilitic glossitis.25
Conclusion
Once believed to be a developmental anomaly of the tuberculum impar, MRG is currently considered to be a chronic inflammatory process as a result of candidal infection. Diagnosis can be confirmed with a cytology smear and quantification of the fungal burden, and treatment should follow the guidelines of oral candidal infections, with topical or systemic antifungals.
Our patient was treated with a topical “swish and spit” antifungal, and advised to keep the area clean. Three months later, upon follow up examination, the lesion had healed completely.
