Disorders of Oral Pigmentation Clinical Presentation

Updated: Nov 01, 2019
  • Author: Talib Najjar, DMD, MDS, PhD; Chief Editor: Jeff Burgess, DDS, MSD  more...
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Presentation

History

Peutz-Jeghers syndrome

A family history of Peutz-Jeghers syndrome may be present. Repeated bouts of abdominal pain in patients younger than 25 years may be noted. Unexplained intestinal bleeding in a young patient may be present. Tissue from the rectum may be prolapsed. In females, menstrual irregularities (due to hyperestrogenism from sex cord tumors with annular tubules) may be present. Precocious puberty may be noted.

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A history of dental restorations must be present.

Nevi and melanoma

Oral nevi could arise spontaneously anywhere in the oral cavity and lips. Oral melanomas arise silently, with few symptoms noted until progression has occurred. Most people do not inspect their oral cavity closely, and melanomas are allowed to progress until significant swelling, tooth mobility, or bleeding causes them to seek care. Pigmented lesions ranging from 1 mm to 1 cm or larger are found. Reports of previously existing pigmented lesions are common. These lesions may represent unrecognized melanomas in the radial growth phase.

Iatrogenic pigmented lesions

Iatrogenic pigmented lesions are found most commonly in persons who smoke heavily and in females taking contraceptive pills.

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Physical Examination

Peutz-Jeghers syndrome

Cutaneous pigmentation (1- to 5-mm macules) of the perioral region crosses the vermilion border (94%) and the perinasal and perioral areas. Lesions may be present on the fingers and the toes, on the dorsal and volar aspects of the hands and the feet, and around the anus and the genitalia. The pigmentation may fade after puberty. Mucous membrane pigmentation is primarily on the buccal mucosa (66%) and rarely on the intestinal mucosa. A rectal mass (rectal polyp) may be observed. Gynecomastia and growth acceleration (due to Sertoli cell tumor) may be found. In males, a testicular mass may be present.

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Dark macular lesions, which are often bluish, are seen.

Nevi and melanoma

Oral nevi are often seen by dentists during routine oral examinations. Malignant melanomas are often clinically silent, and they can be confused with a number of asymptomatic benign pigmented lesions. Oral melanomas are largely macular, but nodular and even pedunculated lesions can occur.

Pain, ulceration, and bleeding are rare in persons with oral melanoma until late in the disease course.

The pigmentation varies from dark brown to blue-black; however, mucosa-colored and white lesions are occasionally noted, and erythema is observed when the lesions are inflamed.

The palate and the maxillary gingiva are involved in approximately 80% of patients, but lesions are also identified on the buccal mucosa, the mandibular gingiva, and the tongue. The oral mucosa is primarily involved in less than 1% of melanomas. Metastatic melanoma most frequently affects the mandible, the tongue, and the buccal mucosa.

Features of long-standing lesions include elevation, color variegation, ulceration, and satellite lesions that may have the appearance of physiologic pigmentation.

A neck mass may be present, indicating regional metastasis; however, this is rare unless the primary tumor is extensive.

Amelanotic melanoma accounts for 5-35% of oral melanomas. This melanoma appears in the oral cavity as a white, mucosa-colored, or red mass. The lack of pigmentation contributes to clinical and histologic misdiagnosis.

Iatrogenic pigmented lesions

These appear as pigmented macules or papules that are smaller than 6 mm in greatest diameter.

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Complications

Peutz-Jeghers syndrome

In young patients, small intestinal obstruction and intussusception are the main complications. These complications result from the small intestinal location of the polyps.

Cancer is the main consequence as patients with Peutz-Jeghers syndrome age (93% cumulative risk by age 64 y). The major sites of cancer occurrence (in order of risk) are the small intestine, the stomach, the pancreas, the colon, the esophagus, the ovaries, the lungs, the uterus, and the breasts. In addition, other reproductive site cancers have been associated with Peutz-Jeghers syndrome, including adenoma malignum of the cervix, Sertoli cell tumors, and sex cord tumors with annular tubules.

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No major complications are reported.

Melanoma

Complications stem from the loss of anatomic structures as a result of the surgical procedure.

Interferon use is associated with malaise, flulike symptoms, fever, and myalgia.

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