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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.
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.
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.
Fissured tongue affects males and females in equal numbers, and all ethnic groups.
Geographic tongue is a relatively common, benign, inflammatory condition of the tongue (glossitis) of unknown cause. It is characterized by continually changing, localized areas of shedding and regrowth of the threadlike elevations (filiform papillae) of the tongue surface. Such changes lead to the development of one or more sharply defined, smooth patches on the tongue that are a bright pinkish red, with elevated white, yellow, or grayish borders. The condition is named for the characteristic geographic or maplike appearance of the tongue surface that results from the changing shape and outline of the lesions. Although such changes often do not cause symptoms (asymptomatic), the lesions may occasionally result in mild irritation or burning. (For further information, use "geographic tongue" as your search term in the Rare Disease Database.)
Hairy tongue is a benign condition that is also called black hairy tongue due to its characteristic appearance. It is characterized by abnormal elongation and blackish or dark brownish discoloration of the filiform papillae. Such changes typically begin at the back region of the body of the tongue and extend toward the front of the tongue's surface. The specific underlying cause of hairy tongue is unknown. However, possible predisposing factors may include poor oral hygiene and overgrowth of pigment-producing bacteria or fungi in the mouth, treatment with certain antibiotic medications, smoking, chewing tobacco, and/or mouthwash use. (For further information, use "hairy tongue" as your search term in the Rare Disease Database.)
There are additional conditions that may be associated with abnormal changes of the tongue. Such conditions are typically characterized by symptoms and physical findings that differ from those associated with fissured tongue. (For further information, choose the specific condition name in question as your search term in the Rare Disease Database.)
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.
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:
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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.
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.
Report last updated: 2008/04/25 00:00:00 GMT+0