Tongue, Fissured

Tongue, Fissured

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Tongue, Fissured is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Furrowed Tongue
  • Lingua Fissurata
  • Lingua Plicata
  • Lingua Scrotalis
  • Plicated Tongue
  • Scrotal Tongue
  • Cerebriform Tongue
  • Grooved Tongue

Disorder Subdivisions

  • None

General Discussion

Fissured tongue is a benign condition that is sometimes referred to as scrotal or plicated tongue. It is characterized by numerous shallow or deep grooves or furrows (fissures) on the back (dorsal) surface of the tongue. The surface furrows may differ in size and depth, radiate outward, and cause the tongue to have a wrinkled appearance. The condition may be evident at birth (congenital) or become apparent during childhood or later. Reports suggest that the frequency and severity of fissured tongue appear to increase with age.



In some cases, fissured tongue may develop in association with infection or malnutrition. In other affected individuals, it may occur with certain underlying syndromes or may be a familial condition, suggesting autosomal dominant inheritance.

Symptoms

Fissured tongue is characterized by a division into lobules, convolutions, and ridges on the tongue that resemble the skin patterns of the scrotum. The markings on the back of the tongue are exaggerated, and knoblike projections (fungiform papillae) may be prominent. The grooves tend to radiate from the central depression of the tongue, resembling the ribs of a leaf. It is probably a secondary phenomenon, caused by the topography of the underlying muscle bundles. Pain in the tongue (glossodynia) sometimes occurs with this condition.

Causes

Fissured tongue may appear to occur as a primary isolated condition; as a familial disease entity; and/or in association with various underlying conditions or syndromes.



In some cases, fissured tongue is apparent at birth (congenital) due to incomplete fusion of the two halves of the tongue. In other instances, the condition may develop due to malnutrition, low levels of vitamin A, certain infections, or trauma.



Fissured tongue may also occur in association with certain underlying syndromes, including Melkersson-Rosenthal and Down syndromes.



Melkersson-Rosenthal syndrome is a rare disorder that often becomes apparent during childhood. It is characterized by paralysis of one or both sides of the face; chronic facial swelling, particularly of the lips; and fissured tongue. The syndrome usually appears to occur randomly for unknown reasons (sporadically); however, some familial cases have also been described, suggesting autosomal dominant inheritance. (For further information on this disorder, choose "Melkersson Rosenthal" as your search term in the Rare Disease Database.)



Down syndrome, also known as trisomy 21 syndrome, is the most common chromosomal abnormality syndrome. In those with Down syndrome, all or a portion of chromosome 21 is present three times rather than twice in all or some cells of the body. Associated symptoms and findings may vary greatly in range and severity from case to case. However, common features include low muscle tone (hypotonia) and a poor startle (Moro) reflex; short, broad hands; malformations of the skull and facial (craniofacial) region; mental retardation; congenital heart defects; increased susceptibility to certain infections; and/or short stature. Characteristic craniofacial abnormalities may include a short, small head (microbrachycephaly); upwardly slanting eyelid folds (palpebral fissures); vertical skin folds that may cover the eyes' inner corners (epicanthal folds); a small nose with a depressed nasal bridge; a relatively flat facial profile; and excessive skin on the back of the neck. In addition, affected individuals commonly have a fissured tongue that appears relatively large. (For further information on this disorder, use "Down" or "trisomy 21" as your search term in the Rare Disease Database.)



In addition, reports suggest that many individuals with fissured tongue also have geographic tongue. The latter is characterized by rapid loss and regrowth of the threadlike elevations (filiform papillae) of the tongue surface, leading to the development of smooth pinkish red patches with surrounding, thickened, whitish borders. (For more information on geographic tongue, please see the "Related Disorders" section of this report below.) Geographic tongue and/or fissured tongue have been observed in several members of certain multigenerational families. According to investigators, evidence suggests that, in such familial cases, the conditions may be due to changes (mutations) of a gene that is transmitted as an autosomal dominant trait with reduced penetrance.



Human traits, including the classic genetic diseases, are the product of the interaction of two genes for that condition, one received from the father and one from the mother. In autosomal dominant disorders, a single copy of the disease gene (received from either the mother or father) may be expressed "dominating" the other normal gene and resulting in the appearance of the disease. The risk of transmitting the disease gene from affected parent to offspring is 50 percent for each pregnancy regardless of the sex of the resulting child. In autosomal dominant disorders with reduced penetrance, fewer than 100 percent of those with the defective gene(s) for the disorder manifest associated symptoms and findings.



According to researchers, in such familial cases, fissured tongue appears to develop subsequent to geographic tongue. In addition, as noted above, the severity of fissured tongue appears to increase with age.



Investigators indicate that fissured tongue with normal appearing (i.e., rather than smooth surfaced) filiform papillae is not familial and not associated with geographic tongue. Rather, some researchers suggest that tongue fissuring with normal filiform papillae should be considered a variation of normal tongue anatomy, while fissured and geographic tongue should be seen as a distinct disease entity.

Affected Populations

Fissured tongue affects males and females in equal numbers, and all ethnic groups.

Standard Therapies

In fissured tongue, oral hygiene is very important to keep the ridges in the tongue free of foreign matter that might otherwise cause inflammation. The symptoms of fissured tongue may disappear spontaneously.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Behrman RE, et al., eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, Pa: W.B. Saunders Company; 2000:1119, 2027.



Beers MH, et al., eds. The Merck Manual. 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:754.



Fauci AS, et al., eds. Harrison's Principles of Internal Medicine. 14th ed. New York, NY: McGraw-Hill Companies, Inc.; 1998:90.



Gorlin RJ, et al., eds. Syndromes of the Head and Neck. 3rd ed. New York, NY: Oxford University Press; 1990:33-37, 611-12.



Buyse ML. Birth Defects Encyclopedia. Dover, Mass: Blackwell Scientific Publications, Inc; 1990:312, 391-93, 1687-88.



JOURNAL ARTICLES

Kullaa-Mikkonen A. Familial study of fissured tongue. Scand J Dent Res. 1988;96:366-75.



Kuramoto Y, et al. Geographic tongue in two siblings. Dermatologica. 1987;174:298-302.

Resources

Smell and Taste Center

Smell and Taste Center

University of Pennsylvania

5 Ravdin Building

3400 Spruce Street

Philadelphia, PA 19104

Tel: (215)662-6580

Fax: (215)349-5266

Email: Geraldine.Fischer@uphs.upenn.edu

Internet: http://www.med.upenn.edu/stc/index.html



NIH/National Institute of Dental and Craniofacial Research

Building 31, Room 2C39

31 Center Drive, MSC 2290

Bethesda, MD 20892

USA

Tel: (301)496-4261

Fax: (301)480-4098

Tel: (866)232-4528

Email: nidcrinfo@mail.nih.gov

Internet: http://www.nidcr.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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