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Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome)

Geographic Tongue




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Author: Robert Kelsch, DMD, Clinical Assistant Professor, Department of Oral Biology and Pathology, State University of New York-Stony Brook; Consulting Staff, Department of Dental Medicine, Division of Oral Pathology, Long Island Jewish Medical Center

Robert Kelsch is a member of the following medical societies: American Academy of Oral and Maxillofacial Pathology and American Dental Association

Editors: Jean Paul Ortonne, MD, Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Drore Eisen, MD, DDS, Consulting Staff, Department of Dermatology, Dermatology Research Associates of Cincinnati; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

Author and Editor Disclosure

Synonyms and related keywords: scrotal tongue, lingua plicata, Melkersson-Rosenthal syndrome, Down syndrome, benign migratory glossitis, geographic tongue

Background

Fissured tongue is a condition frequently seen in the general population that is characterized by grooves that vary in depth and are noted along the dorsal and lateral aspects of the tongue. Although a definitive etiology is unknown, a polygenic mode of inheritance is suspected because the condition is seen clustering in families who are affected. Patients are usually asymptomatic, and the condition is initially noted on routine intraoral examination as an incidental finding. Fissured tongue is also seen in Melkersson-Rosenthal syndrome and Down syndrome and in frequent association with benign migratory glossitis (geographic tongue).

Melkersson-Rosenthal syndrome is a rare condition consisting of a triad of persistent or recurring lip or facial swelling, intermittent seventh (facial) nerve paralysis (Bell palsy), and a fissured tongue. The etiology of this condition is also unknown. The orofacial swelling usually manifests as pronounced lip enlargement. It may or may not affect both lips, and it may be tender or erythematous. Histologic examination of this tissue exhibits characteristic noncaseating granulomatous inflammation. Therapy for these lesions is often intralesional steroid injections. The facial paralysis is indistinguishable from Bell palsy, and it may be an inconsistent and intermittent finding with spontaneous resolution. The presence of fissured tongue in association with these other features is diagnostic of the condition.

Pathophysiology

This condition affects only the tongue and is a finding in Melkersson-Rosenthal syndrome, which consists of a triad of fissured tongue, granulomatous cheilitis, and cranial nerve VII paralysis (Bell palsy).

Frequency

United States

Overall, the prevalence within the United States has been reported to range from 2-5% of the population.

International

The prevalence worldwide varies by geographic location and has been reported to be as high as 21%.

Mortality/Morbidity

Fissured tongue is a totally benign condition and is considered by most to be a variant of normal tongue architecture. When seen in association with Melkersson-Rosenthal syndrome, the morbidity is due not to the fissured tongue but is secondary to the granulomatous inflammation of the lips/facial soft tissues and facial paralysis.

Race

No predilection for any particular race is apparent.

Sex

Some reports have shown a slight male predilection.

Age

Although fissured tongue may be diagnosed initially during childhood, it is diagnosed more frequently in adulthood. The prominence of the condition appears to increase with increasing age.



History

The lesions are usually asymptomatic unless debris is entrapped within the fissure or when it occurs in association with geographic tongue (a common finding).

Physical

  • On clinical examination, fissured tongue affects the dorsum and often extends to the lateral borders of the tongue.
  • The depth of the fissures varies but has been noted to be up to 6 mm in diameter.
  • When particularly prominent, the fissures or grooves may be interconnected, separating the tongue dorsum into what may appear to be several lobules.

Causes

Although a specific etiology has not been elicited, a polygenic or autosomal dominant mode of inheritance is suspected because this condition is seen with increased frequency in families with an affected proband.



Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome)
Geographic Tongue


Histologic Findings

A biopsy is rarely performed on a fissured tongue because of its characteristic diagnostic clinical appearance; however, histologic examination has shown an increase in the thickness of the lamina propria, loss of filiform papillae of the surface mucosa, hyperplasia of the rete pegs, neutrophilic microabscesses within the epithelium, and a mixed inflammatory infiltrate in the lamina propria.



Medical Care

No definitive therapy or medication is required. If symptomatic, patients are encouraged to brush the dorsum of the tongue to eliminate debris that may serve as an irritant.

Consultations

Additional consultation with an appropriate physician may be necessary if the patient exhibits manifestations of Melkersson-Rosenthal syndrome, particularly cranial nerve VII paralysis.



Complications

  • Complications are not associated with the fissured tongue per se but are noted in association with the manifestations of Melkersson-Rosenthal syndrome.

Patient Education

  • Inform patients that this is a frequent finding and no specific therapy is required, aside from that necessary to treat the manifestations of Melkersson-Rosenthal syndrome, if present.



Medical/Legal Pitfalls

  • Because this condition is common in the general population, the diagnosis is easily made, and no medical intervention is required, no medicolegal issues are identified.

Special Concerns

  • This condition is observed in patients of all ages and has no impact on the general systemic health of the patient, unless it is associated with Melkersson-Rosenthal syndrome.



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Fissured Tongue excerpt

Article Last Updated: Oct 5, 2006