Pigmented Lesions Of Oral Mucosa Short Notes
Question 1. Enumerate the port-wine stains or Sturge- Weber syndrome.
Answer:
- Sturge-Weber syndrome (SWS) is a nonfamilial neurocutaneous syndrome characterized by facial port-wine stains and occipital leptomeningeal angiomatosis with resulting neurological abnormalities.
- Other features associated with this condition include ocular and rarely internal organ involvement.
Sturge-Weber syndrome Etiology:
- It is a congenital malformation resulting from changes in the ectoderm, mesoderm, and neural crest derivatives.
- The persistence of a vascular plexus around the cephalic neural tube portion characterizes leptomeningeal angiomatosis and facial port-wine stains.
Sturge-Weber syndrome Classification:
- Type 1 (Classic) SWS: This is the most common form with both facial and leptomeningeal angiomas. Seizures usually occur in the first year of life, and ocular involvement (glaucoma) is likely to be present.
- Type 2 SWS: This type manifests with facial angioma and may be glaucoma but with no evidence of intracranial disease.
- Type 3 SWS: This type only involves leptomeningeal angioma, with no facial nevus and no ocular manifestation.
Read And Learn More: Oral Medicine and Radiology Question And Answers
Sturge-Weber syndrome Clinical Features:
- The port-wine stain (nevus flammeus) is the most characteristic finding and present since birth.
- It affects half of the face and may extend to the neck with a characteristic to darken with age, ranging from reddish to purple over time.
- Only persons manifesting the nevus flammeus along the ophthalmic branch develop the syndrome in its classical form.
Sturge-Weber syndrome Oral Manifestations:
- Oral findings include hemangiomatous lesions in the lip, oral mucosa, gum, tongue, and palatal region.
- The oral manifestations are generally unilateral and disappear abruptly in the middle line.
- Ipsilateral gingival hyperplasia and gin¬gival hemorrhage even for minimal traumatism may also present.
- Macroglossia and maxillary bone hypertrophy may present in some patients leading to facial asymmetry.
- Dilantin therapy for convulsive crises may exacerbate gingival enlargement.
Sturge-Weber syndrome Investigations:
- The intraoral radiographic findings reveal horizontal and vertical bone resorptions with loss of lamina dura of the teeth in the lesion side obviously confirming diagnosis with the clinical observation of other changes, such as gingival hyperplasia.
- Computer tomographic image reveals intracranial calcifications and unilateral cerebral atrophy.
- Magnetic resonance image shows cerebral vascular malformations.
Sturge-Weber syndrome Management:
- Usually, the port-wine nevus in the face may be improved by the use of new techniques of laser therapy.
- Periodic oral examination is necessary for patients presenting with oral changes.
- Oral infections should be eradicated since it may aggravate the vascular picture.
- Extreme care is essential during oral surgical procedures due to the possibility of intense hemorrhage.
Question 2. What is Peutz-Jeghers syndrome?
Answer:
- Peutz-Jeghers syndrome is a rare genetic disease associated with mutations in a few tumor suppressor genes. It is characterized by hamartomatous polyps in the gastrointestinal tract (mostly in the small bowel), and pigmented mucocutaneous lesions with an increased risk of carcinomas of the gastrointestinal tract, pancreas, breast, and thyroid.
- Black-to-brown spots of less than 1 mm in size are typically localized on the lower lip and in the perioral area. Intraoral, intranasal, and conjunctival pigmented lesions may also be present.
- The oral lesions are benign and histologically characterized by an increase in melanin in the basal layer without an obviously increased number of melanocytes.
- A fading or disappearance of the spots is usually observed in older age.
- Dental practitioners when come across the characteristic oral pigmentation should advise the patients for needful medical examination to reduce the potential later complications.
Question 3. Describe about drug-induced pigmen¬tation.
Answer: Certain drugs may cause an intraoral inflammatory reaction and subsequently induce postinflammatory hyperpigmentation which is the basis of pigmentation seen in fixed drug reactions.
- Clotrimazole was the most common drug-associated to oral pigmentation followed by tetracycline; however, many others have been implicated including colchicines, ketoconazole, pyrimethamine, and barbiturate.
- Arsenic drugs can directly induce pigmentation by combining with sulfhydryl groups in the epidermal cells causing increased action of tyrosinase.
- Phenothiazines and minocycline may be deposited in the skin or mucosa and directly react with melanin to form a drug-pigment complex.
- Fixed drug eruptions are more commonly seen in people with dark skin and often present as a slate brown color due to pigmentary incontinence of melanophages in the upper dermis. Pigmented macules of the tongue may also manifest as a result of a fixed drug eruption.
- A dark-pigmented macular area on the dorsum of the tongue is noted following inhalation of the heroin and methaqualone.
- Oral brownish pigmentation has also been reported in human immunodeficiency virus (HIV)—positive patients receiving treatment with zidovudine (AZT), ketoconazole, and clofazimine.
- Question 4. What is exogenous pigmentation or metallic pigmentation?
Answer: - Many metals have been implicated in the production of pigmentation in the oral region. These include:
- Lead, which produces plumbism— characteristic generalized cutaneous “lead hue” (Described as a combination of pallor and lividity), and “lead lines” (Burtonian line) on the gingiva as gray areas of discoloration below the gingival margins.
- The intake of lead is possibly from the cooking vessels, containers, and glass vessels. The ingested lead is secreted in saliva and deposited along the free gingival margin of teeth. Chelating agents are helpful in removing lead from the body.
- Mercury can cause slate-gray gingival pigmentation. The actual mercury poisoning is called pink disease or acrodynia characterized by excessive salivation and difficulty in masti-cation.
- Amalgam tattoo is caused by the presence of metallic restorative material in the oral tissues that are spilled and implanted during the treatment procedures. Amalgam tattoos are painless, gray-blue macules that range in size from a few millimeters to greater than 1 cm.
- The tattoo can be single or multiple. Most amalgam tattoos are located on the gingiva and edentulous mucosa, but can also be seen on the hard palate, buccal mucosa, and floor of the mouth. The radiographic evaluation may be positive.
- On histopathological exa¬mination, fine black granular or fibrillar material embedded in the connective tissue or in a perivascular location with little or no inflammatory response is seen.
Question 5. Describe the heme-associated lesions of the oral mucosa or ecchymosis and petechiae.
Answer:
- Ecchymosis and petechia are extravascular lesions of varying sizes.
- They occur due to the extravasation of red blood cells into the submucosa and appear as bright red macules. They do not blanch and typically resolve within a month.
Ecchymosis:
- Ecchymosis appears as multiple red-brown macules measuring 1 cm or larger in size. In case of trauma, ecchymosis will disappear within 2 weeks.
- Patients on anticoagulants may predispose to trauma on chewing and often present with ecchymosis on the tongue or cheek.
- Hereditary coagulation disorders and chronic liver failure also cause ecchymosis of the skin and mucosa. In such cases, prothrombin time (PT), partial PT (PPT), and clotting time should be evaluated.
Petechiae:
- Petechiae are pin-size flat hemorrhagic spots usually less than 2 mm in size and initially appear as red and within a few days turn brown due to lysis of extravasated erythrocytes.
- They occur on the soft palate due to excessive or aggressive self-inflicted suction of the soft palate against the posterior tongue. Usually occur in a cluster of 10-30 spots.
- They are also precipitated by viral or allergic pharyngitis which stimulates the action of clicking on the palate with the tongue to get relief from pruritus.
Petechiae may also be secondary to:
- Autoimmune or idiopathic thrombocytopenic purpura
- HIV-related thrombotic thrombocytopenic purpura
- Platelet aggregation disorders
- Aspirin toxicity
- Myelosuppressive chemotherapy.
Ecchymosis and petechia Diagnosis: Platelet count and platelet aggregation test.
Ecchymosis and Petechia Management:
- If trauma or suction is the cause, then the patient should be educated to stop the habit.
- For hemorrhagic conditions, referral to a hematologist is necessary.
Question 6. Describe the cafe au lait pigmentation.
Answer:
- Cafe au lait spots are present in neurofibroma¬tosis (type 1), an autosomal dominant disease characterized with nodular and pendulous neurofibromas of skin and oral cavity and concomitant cutaneous pigmentation.
- Cafe au lait spots or cafe au lait macules are hyperpigmented lesions that may vary in color from light brown to dark brown; this is reflected by the name of the condition which means “color of coffee with cream”.
- They appear in late childhood as multiple small macules or as broad diffuse lesions overlying the surface of neurofibromatosis swelling.
- This type of pigmentation is also associated with McCune-Albright syndrome, tuberous sclerosis, and Fanconi’s anemia.
- This pigmentation does not require any treatment and only monitoring of the underlying associated condition is sufficient.
Question 7. What is black hairy tongue or hairy tongue?
Answer:
- Black hairy tongue is a benign disorder characterized by hypertrophy and discoloration of the middle and posterior third of the dorsum of the tongue.
- This condition is characterized by abnormal hypertrophy and elongation of filiform papillae on the surface of the tongue due to defective desquamation of the tongue papillae resulting in excessive growth and thickening of the filiform papilla.
- The elongated papillae will subsequently collect debris, bacteria, fungi or other foreign materials that contribute to the discoloration.
- The proposed precipitating factors include smoking or chewing tobacco, drinking alcohol, poor oral hygiene using peroxide-containing mouthwash, radiation therapy, trigeminal neuralgia, using drugs that cause xerostomia like anticholinergics, antihypertensives and antidepressants, and antibiotics, such as tetra¬cyclines and penicillins.
- Hairy tongue is usually asymptomatic, but occasionally patients may complain of tickling or burning of the tongue, nausea, halitosis, dysgeusia, and an unattractive appearance of the tongue.
Black hairy tongue Diagnosis: Visual identification of discolored tongue with elongated papilla.
Black hairy tongue Management:
- Discontinuation of the offending agent alongside practicing good oral hygiene usually resolves the problem.
- Gentle tongue debridement can be accomplished by cleaning the tongue with a soft toothbrush and a solution of baking soda also help.
Question 8. What is diascopy?
Answer:
- Diascopy is a chairside investigation to check the blanching and changes in the color of a lesion. This test is applied to differentiate the vascular lesion from other pigmented, nonvascular lesions.
- A thin, transparent glass slide is used to apply pressure on the lesion by pressing over the surface.
- In hemangioma, there will be complete blanching of the lesion. The blood within the vessels is completely poured out when firm pressure is applied.
Question 9. What is blue nevus?
Answer:
- A blue nevus is a benign, acquired melanoma- cystic lesion that typically presents as an asymptomatic, slate-blue or blue-black smooth-surfaced macule or papule. Size is usually less than 6 mm in diameter. The melanotic cells are located deep in the connective tissue giving the lesion a bluish tint.
- Oral mucosa is rarely affected by blue nevus. The intraoral variant of blue nevus is typically identified between the third and fifth decades of life, females tend to be more commonly affected than males.
- Two-thirds of all intraoral blue nevi are found on the hard palate, and buccal mucosa is the second most common site of presentation.
- The blue nevus is characterized by a variety of histologic subtypes, although most are classified as either “common” or “cellular”. Common blue nevi are almost always innocuous lesions and rarely recur.
- Rare cases of malignant melanoma have been reported to arise in cellular blue nevi.
Question 10. Describe the malignant melanoma.
Answer:
- In the head and neck region, melanoma occurs on facial skin and oral mucosa. On facial skin, the malar region is the common site as it is more exposed to sunlight.
- Facial melanoma appears as a nodular or macular lesion with irregular margins and possesses varying shades from brown to black to blue with some areas of depigmentation.
- Mucosal melanoma is rare. It is characterized by the proliferation of malignant melanocytes along the junction between the epithelial and connective tissues, as well as within the
connective tissue. - The most common sites are the anterior labial gingival and the hard palate. Oral melanoma occurs between the fourth and seventh decades of life with a greater incidence in men than in women.
- Clinically, oral melanoma may present as an asymptomatic, slow-growing brown or black patch with asymmetric and irregular borders or as a rapidly enlarging mass associated with ulceration, bleeding, pain, and bone destruction.
Malignant melanomas are characterized by:
- A prolonged radial growth phase where it grows superficially and laterally followed by a vertical growth phase where there is deep connective tissue invasion takes place or
- A direct vertical growth phase.
The tumor, nodes, metastasis (TNM) clinical staging system for oral melanoma recognizes three stages:
- Stage 1: Primary tumor (T, any N0, M0)
- Stage 2: Metastatic tumor to regional lymph nodes (T, any N1, M0)
- Stage 3: Metastatic tumor to distant sites (T, any N, any M1).
Treatment involves radical surgical excision with clear margins. Distant metastases often affect lungs, brain, liver, or bones. The prognosis for patients with oral melanoma is much worse than for those with cutaneous lesions.
Question 11. Describe the hemochromatosis.
Answer:
- Hemochromatosis is a condition in which hemosiderin pigments are deposited on multiple organs and tissue.
- It is characterized by increased intestinal absorption of iron and subsequent iron deposition in the liver, pancreas, heart, and pituitary leading to organ damage, iron storage pigmentation, diabetes mellitus, liver and cardiac impairment, and arthropathy.
The liver is the first organ to be affected in 95% of cases and hepatocellular carcinoma is common. - Diabetes mellitus and skin pigmentation together are known as “Bronze diabetes”.
- Hemochromatosis
Hemochromatosis Oral Pigmentation: Diffuse brown to gray macules occur on the palate and gingival due to submucosal iron deposition.
Hemochromatosis Diagnosis:
- Blood tests for serum iron. Either total iron binding capacity or transferrin are done as screening tests. Serum ferritin test also indicated which is elevated in patients with hemochromatosis.
- Incision biopsy and Prussian blue staining of the pigmented tissue will color the iron.
Hemochromatosis Management: Medical referral is advisable. Parenteral administration of desferrioxamine is used to remove excess iron deposition.
Question 12. What is hemangioma?
Answer:
- The hamartomatous proliferation of vascular channels is known as hemangioma. It is a developmental disorder and usually regresses at puberty.
- Oral hemangiomas are present within the connective tissue of mucous membrane and appear reddish blue if the proliferating vessels are closer to the epithelium and deep blue if they are at the deeper level. They are raised and nodular or diffuse, flat and macular.
- The frequently affected organ is the tongue and appears as diffuse, raised, multinodular blue deep racemose. Labial mucosa is the second common site and appears as a localized, raised blue lesion.
They are asymptomatic and blanch under pressure. - When a microscopic glass slide is placed on the surface of the hemangioma and adding pressure will demonstrate blanching and relieving the pressure will cause refilling and original color.
Hemangioma Management:
- Many cases undergo spontaneous involution after puberty. In persisting cases, laser surgery or cryotherapy may be carried out.
- For larger lesions, sclerosing agents like 1% sodium tetradecyl sulfate can be administered by intralesional injection.
Question 13. Describe Kaposi’s sarcoma.
Answer:
- Kaposi’s sarcoma is a multicentric angio- a proliferative disorder of endothelial origin with slowly progressive growth.
- It predominantly affects mucocutaneous sites, but may also affect visceral organs. This is associated with human herpes virus type 8 (HHV-8).
Kaposi’s sarcoma can be primarily categorized into four types:
- Epidemic of AIDS-related
- Immunocompromised
- Classic or sporadic
- Endemic (African).
Oral lesions occur as blue, red, and purple at the hard palate, gingiva, oropharynx (upper and lower retromolar area, and soft palate, alveolar mucosa, and the dorsum of the tongue) with decreasing order of frequency. It does not have a tendency for metastasis and is not fatal.
Kaposi’s sarcoma is prevalent in HIV sero¬positive patients, most cases of HIV-related Kaposi’s sarcoma occur when patients have a CD4 cell count below 200 cells/mm³.
Kaposi’s Sarcoma Investigations:
- CD4 count and viral load.
- Punch biopsy and histopathological examination.
Management of HIV-related Kaposi’s Sarcoma
- Antiretroviral Therapy: Optimal control of HIV infection using highly active antiretroviral therapy (HAART) is an integral part of successful Kaposi’s sarcoma therapy.
- Local Therapy: Local therapies can be used for palliation of locally advanced symptomatic disease or in individuals who have cosmetically unacceptable lesions:
-
- Radiation therapy
- Cryotherapy
- Laser therapy
- Surgical excision
- Intralesional vinca alkaloid therapy.
- Multiple biweekly injection of vinblastine is administered for eradication of this tumor.
- Intralesional injection of 1% sodium tetradecyl sulfate causes tumor necrosis.
- Topical retinoids.
- Kaposi’s sarcoma Immunomodulation: Immunomodulation with interferon-alpha is effective through its antiangiogenic, antiviral, and immunomodulatory properties.
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