Developmental Disturbances of the Oral

Mucosa, Gingiva and Tongue

GROUP 3:Joyce Ng Siew Siew

Kavinder KaurHarvinder Singh

Kartick Arumugam

Developmental Disturbances of the Oral Mucosa

Fordyce’s GranulesFocal Epithelial Hyperplasia

White Sponge NevusLeukoedema

Caliber-Persistent Artery

FORDYCE’S GRANULES ectopic collections of sebaceous glands upper lip, buccal mucosa, gingiva, anterior pillars of fauces bilaterally symmetrical distribution 60-70% of adult population granules in upper lip increases during puberty; granules in buccal mucosa increases in later stages of life multiple, small, discrete, milia-like, yellowish stuctures; 1-2mm diameter occasionally form slightly raised confluent plaques

HISTOLOGY identical to normal sebaceous glands

found in skin but not associated with hair empty into a duct, which opens directly

onto mucosal surface peripheral cells are flat and darkly stained inner cells are lipid rich and pale

rarely develop into sebaceous cysts or adenomas

FOCAL EPITHELIAL HYPERPLASIA “Heck’s disease” multiple papillary or sessile areas of epithelial

hyperplasia Native Americans, Indians, Northern native people

and other ethnic groups in Europe and Africa caused by Human Papilloma Virus (HIV) type 13

and 32 (?) similar lesions in HIV-seropositive homosexual

males children, 3-18 years old white or pink multiple, small pedunculated,

polypoid or nodular soft tissue growths; 1-5mm diameter

buccal and labial mucosa most common most lesions regress after 4-6months, occasionally

recur

HISTOLOGY hyperparakeratosis of the covering epithelium with extensive acanthosis epithelial cells of the upper spinus layer show enlarged nuclei and vacuolated clear cytoplasms (koilocytes) deeper epithelial layer shows thickening, elongation and fusion of rete-pegs basal cell layer exhibit increased mitotic activity occasional focal areas of liquefaction degeneration of basal layer loose underlying connective tissue, well-vascularized and variable infiltrates of lymphocytes

WHITE SPONGE NEVUS autosomal dominant hereditary disorder mutations in the genes coding for keratins 4 and 13 and cells heap up on epithelial surface some congenital, some adolescence intraoral lesions almost always bilateral buccal mucosa and tongue mostly, sometimes vestibular mucosa asymptomatic, deeply folded white or grey lesion surface is soft, uneven, spongy sometimes can be peeled off by gentle rubbing without bleeding diffused or patchy, translucent opalescence similar to leukoedema

HISTOLOGY epithelial thickening, mild to moderate

hyperparakeratinization from surface until deep into spinus, acanthosis and spongiosis, intra cellular edema of spinus

cells with intracellular edema show vacuolated cytoplasms and shrunken nuclei

“basket weave” appearance – only cell walls and nuclei visible

individual cell keratinization in spinus cell layer, basal cells intact, lamina propria no inflammation

ELECTRON MICROSCOPYSome cells of spinus layer differentiate early and become enriched with tonofilaments

LEUKODEMA more in blacks than whites possibly due to mucosal pigmentation in blacks making edematous changes more noticeable variation of normal rather than disease more common and severe in smokers (?) diffused, gray-white, milky, opalescent mucosa folded surface, wrinkles or whitish streaks lesions do not rub off bilateral, may extend onto labial mucosa easy to diagnose: white appearance diminishes when cheek is stretched

HISTOLOGY increased thickness of epithelium with striking intracellular edema of spinus layer vacuolated cells appear large and have stretched nuclei parakeratinized epithelial surface broad and elongated rete ridges benign condition

CALIBER-PERSISTENT ARTERY “retained caliber labial artery”, “caliber-persistent labial artery” inferior alveolar artery retains its large size and thickened walls even after leaving the bone supplies mucosal aspects of lower lip artery becomes superficial towards midline usually few mm inferior to vermilion border palpable due to size 80% on lower lip, few on upper lip and hard palate 40-88 years old artery sessile, elongated nodule, may be pulsatile may be tender or ulcerated due to recurrent trauma or irritation from teeth

HISTOLOGY large artery with thick smooth muscle walls separated from overlying stratified squamous

epithelium by fibrovascular connective tissue “retained caliber” is obvious vessel somewhat parallel to surface of lesion excess keratin on surface and scattered chronic

inflammatory cells in stroma indicate chronic trauma

TREATMENTNone required unless it becomes tender or excessively enlarged from recurring trauma - simple surgical removal.

Developmental Disturbances of the Gingiva

Fibromatosis Gingivae

• Definition :Benign diffuse infiltrative proliferations of

fibroblast and mature collagen fibers within gingival tissues

Hereditary condition – transmitted as an autosomal dominant trait

Result of developmental abnormality of fibroblast function

• Clinical features : Localized / generalized

fibrous enlargement of gingiva

Mostly appears in young children but maybe present at birth / even may not be noticed until adult life

Both sexes equally effected Multinodular enlargements

especially in the inter dental papilla regions

Hyperplastic tissue is firm, painless, and gingiva is coral pink

Sometimes markedly enlarged gingiva may cover entire crown of erupted teeth (Eruption normal)

Associated with hypertrichosis, epilepsy, and mental retardation

Associated with syndromes, e.g. Cowden’s Syndrome and Rutherfords Syndrome

Can be a feature of “ Laband Syndrome”

• Histopathology : Covering epithelium is

hyperplastic and rete-pegs elongated

Fibrous connective tissue consists bundles of collagen fibers with scattered mature spindle shaped few multinucleated fibroblasts

Mucoid changes in gingival connective tissue

Significant number of mast cells

• Differential Diagnosis : Phenytonin sodium

induced gingival hyperplasia

Generalized hyperplastic gingivitis

Leukemic infiltration of gingiva

• Treatment : Periodic gingivectomy

with placement of gingival acrylic splints for cosmetic and functional reasons

• Slightly raised area of mandibular alveolar mucosa

• Commonly located lingual to the cuspids• Structure measures abt 2-4mm • Often present bilaterally between marginal

gingiva and mucogingival junctions• Common among children• Structural resemblance to incisive papilla• Histological – papilla represents a focus of fibro-

vascular tissue with an orthokeratinized / parakeratinized surface and usually covers an osseous foramen of nutrient blood vessel.

Retrocuspid Papilla

Retrocuspid papilla; here, a reddish, slightly-raised sessile small nodule behind or lingual to the lower cuspid tooth.

Retrocuspid papillae in two patients. They are bilateral, green arrows. They may be mistaken for disease.  

Developmental Disturbances of the Tongue

AGLOSIA What is it?Its a rare birth defect where the tongue is missing or underdeveloped

Symptoms ? •Missing tongue, •Feeding problems •Breathing, Speech & Swallowing •problems•Missing teeth• Tooth enamel defects •Missing or abnormally small tongue(two thirds of the tongue is missing)

Treatment ?•Orthodontics.•Breathing assistance may be needed •early in life if airway is compromised•Speech Therapy

MICROGLOSSIA

This is a condition where the size of the tongue is abnormally small. Cases of complete absence of the tongue have been reported.

Difficulties in SpeechDifficulties in Swallowing

NO TREATMENTaffected person will have to train their tongue to the best of their abilities.

MACROGLOSSIAWhat is it? • The presence of an excessively large

tongue, which may be congenital or may develop as a result of a tumor or edema due to obstruction of lymphatic vessels,or it may occur in association with hyperpituitarism or acromegaly. It also may be associated with malocclusion because of pressure of the tongue on the teeth.

Causes ?• Cystic hygroma • I-cell disease • Alpha-mannosidase deficiency • Opitz-Frias syndrome • Wiedemann-Beckwith syndrome • Pituitary tumour (growth hormone

secreting) • Amyloidosis • Tongue cancer • Gangliosidosis GM3 • Generalized gangliosidosis GM1 • Alpha-L-iduronidase deficiency • Hypothyroidism, congenital • Down syndrome

ComplicationsFeeding difficulty Speech problems Jaw development abnormalities Dental abnormalities Ulcerated tongue tip Necrotic tongue tip

Treatment•Get the correct diagnosis 1st !

•Tongue reduction surgery protect the airway & allow normal mastication it may also help optimize dental & oral cavity development.

•Beckwith-Wiedemann: Intractable neonatal hypoglycemia requires immediate recognition •and ICU management.

•Down syndrome: patients should be followed at a comprehensive Down syndrome care center; ( genetics referral )

•Hypothyroidism: Thyroxine replacement with careful tracking to keep levels therapeutic as patient grows .

ANKYLOGLOSSIAWhat is ?

term for tongue tied which is an anomaly present from birth. The severity of the anomaly is variable with the tongue

completed joined to the floor of the mouth in severe cases.

Symptoms•Reduced mobility of tip of tongue •Short frenulum•Impaired feeding ability •Impaired speech •Impaired oral hygiene

Usually seen in patients with ?Chromosome 9 trisomy syndrome

Treatment No treatment is usually required but

Surgery can be done in problematic cases

BIFID TONGUEA tongue with a groove or split

running lengthwise along the tip of the tongue. Also called cleft tongue.

A congenital structural defect of the tongue in which its anterior

part is divided longitudinally for a greater or lesser distance

SURGERY for treatment

FISSURED TONGUE Characterized by grooves that vary in depth and are noted along the

dorsal and lateral aspects of the tongue. The lesions are usually asymptomatic unless debris is entrapped within the fissure .

PhysicalUpon 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.

Fissured tongue is seen in Melkersson-Rosenthal syndrome, in most patients with Down syndrome, and in association with geographic tongue

MEDIAN RHOMBOID GLOSSITIS

Smooth-surfaced red area in posterior midline (large arrow) is slightly nodular. Patient also has midline fissure of anterior dorsum of tongue (small arrow).

Previously median rhomboid glossitis was thought to represent DEVELOPMENTAL DEFECT OF THE TONGUE due to tuberculum impar on the surface of the dorsum of the tongue

during normal embryogenesis however the tuberculum impar should retrude and is overgrown by the lateral lingual swellings.

now investigators believe median rhomboid glossitis is due to chronic infection of candida albicans

ETIOPATHOGENESIS

The embryonic tongue is formed by two lateral processes (lingual tubercles) meeting in the midline and fusing above a central structure from the first and second branchial arches, the tuberculum impar

The posterior dorsal point of fusion is occasionally defective, leaving a rhomboid-shaped, smooth erythematous mucosa lacking in papillae or taste buds

This median rhomboid glossitis (central papillary atrophy, posterior lingual papillary atrophy) is a focal area of susceptibility to recurring or chronic atrophic candidiasis, prompting a recent movement toward the use of posterior midline atrophic candidiasis as a more appropriate diagnostic term.

ETIOPATHOGENESIS

CLINICAL FEATURES

Median rhomboid glossitis presents in the posterior midline of the dorsum of the tongue, just anterior to the V-shaped grouping of the circumvalate papillae. The long axis of the rhomboid or oval area of red depapillation is in the anterior-posterior direction. Most cases are not diagnosed until the middle age of the affected patient, but the entity is, of course, present in childhood. There appears to be a 3:1 male predilection.

Those lesions with atrophic candidiasis are usually more erythematous but some respond with excess keratin production and, therefore, show a white surface change. Infected cases may also demonstrate a midline soft palate erythema in the area of routine contact with the underlying tongue involvement; this is euphemistically referred to as a kissing lesion.

Lesions are typically less than 2 cm. in greatest dimension and most demonstrate a smooth, flat surface, although it is not unusual for the surface to be lobulated. Occasional lesions have surface mamillations raised more than 5 mm. above the tongue surface, and occasional lesions are located somewhat anterior to the usual location. None have been reported posterior to the circumvallate papillae.

Prior to biopsy, the clinician should be certain that the midline lesion does not represent a lingual thyroid, as it may be the only thyroid tissue present in the patient's body. Additional clinical look-alike lesions include the gumma of tertiary syphilis, the granuloma of tuberculosis, deep fungal infections, and granular cell tumor.

Starts as a narrow,mildly erythematous area located along the median fissure of tongue

Lession is asymptomatic and enlarges for years

Developped lession appears as a diamond or lozenge shaped area devoid papilla

Colour of lesion varies from pale pink to bright red , ocasionally presence of white halo

Some patient even develop a similar lesion on the midline of the palate just opposite the tongue lesion

Asymptomatic but cause slight soreness or burning sensation

shows a smooth or nodular surface covered by atrophic stratified squamous epithelium overlying a moderately fibrosed stroma with somewhat dilated capillaries.

PATHOLOGY AND DIFFERENTIAL DIAGNOSIS

Chronic candida infection may result in excess surface keratin or extreme elongation of rete processes and premature keratin production with individual cells or as epithelial pearls (dyskeratosis) deep in the processes. Silver staining for fungus will often reveal candida hyphae and spores in the superficial layers of the epithelium. This pseudoepitheliomatous hyperplasia may be quite pronounced, and the tangential cutting of such a specimen may result in the artifactual appearance of cut rete processes as unconnected islands of squamous epithelium, leading to a mistaken diagnosis of well differentiated squamous cell carcinoma. Because of this difficulty, it is recommended that the patient be treated with topical antifungals prior to biopsy of a suspected median rhomboid glossitis.

GEOGRAPHIC TONGUEIs the multifocal, patchy irregular area of depapillation of tongue characterized by frequent remission and recurrences.

ETIOLOGY

EXACT ETIOLOGY NOT KNOWN

POSITIVE FAMILY HISTORY

EMOTIONAL PROBLEM

Signs and symptoms of geographic tongue include:

Map-like, or geographic, appearance of your tongue

Smooth, bright red patches of various shapes and sizes on the top surface of your tongue

Tongue discomfort

Soreness or a burning sensation that may worsen with hot, spicy or acidic foods

Factors that may be associated with or that may increase the risk of geographic tongue include:

Having relatives with geographic tongue Having a fissured tongue, a condition in which deep grooves form on your tongue's surface Psoriasis Excessive stress Hormonal changes Allergies Systemic steroid use Atopic dermatitis

However, research studies about the risk factors for geographic tongue have been contradictory, and the connection between geographic tongue and other conditions is uncertain. Geographic tongue can occur at any age. Geographic tongue affects about 1 to 3 percent of people. Some evidence suggests that it's more common in women than in men.

Irregular areas of dekeratinized and desquamated filiform papillae (red in color) are surrounded by elevated whitish or yellow margins due to acantholysis and hyperkeratosis. Neutrophils migrate into the epithelial layer, creating what are termed Munro's abscesses.

HISTOPATHOLOGY

TREATMENTNO SPECIFIC TREATMENT , BUT HEAVY DOSES OF VITAMIN MAY PRODUCE SOME RESULT , Eg: VITAMIN B

LINGUAL VARICES

A varix is a dilated,tortuous vein, which is often subjected to increased hydrostatic pressure but is poorly supported by the surrounding tissue

CLINICAL FEATURES

Varicosities can be observed in many oral location: Ventral surface of tongueFloor of the mouthLipsBuccal mucosa Commissures

Ventral surface of the tongue and floor of the mouth are most common sites for oral varices.

Clinically lingual varices appears small roundPurplish nodules lateral to the sublingual vein

Lingual Varices are common among people > 50 and become more conspicuous with advancing age

Presence of lingual varices < 50 indicates premature ageing

Lingual Varices may occur with leg varicosities, however there is no relationship between cardiopulmonary disorder and lingual varices

Lingual varices can be indistinguishable from hereditary hemorrhagic telangiectasia, however the absence of skin and mucosal lession and absence of bleeding tendency in lingual varices usually helps to establish the proper diagnosis.

Hairy Tongue• Definition :Hypertrophy of the filliform papilla of tongue

along with loss of normal desquamation process which eventually leads to formation of pigmented, thick, matted layer on the tongue surface heavily coated with bacteria and fungi

Filiform papillae

• Clinical Features : Commonly affects mid dorsum of

tongue Hypertrophy of filliform papilla

produces thick matted layer on dorsal surface

In extreme case may produce a thick, leathery coating on the tongue surface, known as earthy or encrusted tongue

Brushes soft palate and produce gagging sensations

Produces halitosis Can be irritation due to

accumulation of food debris and microorganisms

Often associated with various systemic diseases

Extension of the tongue with a gauze, showing the dorsal surface with elongated filiform papillae (hairy tongue).

• Treatment : Cleaning and scrapping

of tongueApplication of topical

keratolytic agentsConsumption of yogurtThe affected tongue

papilla often rapidly returns to normal when long-term antibiotics or other drugs are discontinued

• Definition : Accessory accumulation of functional thyroid gland

tissues within the body of the tongue.

• Clinical Features : seen in females during puberty or adolescence In the tongue, thyroid tissue appears as nodular exophytic

mass, measuring abt 2-3mm in diameter and located posterior to foramen caecum

Can also be present as smooth cystic swelling Symptoms – change of voice(dysphonia) , bleeding , pain ,

difficulty in swallowing (dysphagia), respiratory obstruction (dyspnea) and feeling of tightness in the throat

Lingual Thyroid Nodule

• Histopathology : Normal mature thyroid

tissues, although embryonic or fetal thyroid gland tissues may also be seen

Occasionally, thyroid nodules may exhibit colloid degeneration or goiter

• Differential Diagnosis : Thyroglossal tract cyst Neoplasms

• Diagnosis : Diagnostic procedures

include1. Iodine-131 and

technetium scans2. Preoperative biopsy

from the thyroid nodule• Treatment : Surgical excision

Thyroglossal Tract Cyst

• Definition :An uncommon developmental cystic lesion

arising frm the embryonic remnants of the thyroglossal tract and it develops in the midline of the neck, anywhere between the base of the tongue above and the thyroid gland below

The End…