Oral Cavity

ORAL CAVITY GENETIC DISORDERS AFFECTING THE ORAL MUCOSA AND LIPS: 1. WHITE LESIONS: conditions FORDYCE SPOT

Bohn’s and Epstein’s pearls (gingival cyst of the newborn) White sponge nevus

Dyskeratosis congenita

Features They are sebaceous glands containing neutral lipids and are seen after puberty. They are extremely common and 80% of the populations have them. They are usually seen in buccal mucosa inside the commisures and sometimes in the retromolar regions and upper lip. They appear as YELLOWISH SMALL GRAINS seen beneath the buccal and labial mucosa and are totally benign. No treatment is required. Discrete, pearly white or yellow, freely movable elevations, 2-3 mm in diameter at the gingival margins or midline of the hard palate are seen in upto 85% of newborn infants. They are superficial white lesions containing cysts and are shed in a few weeks. It is a rare familial disorder(FAMILY HISTORY IS POSITIVE) seen in CHILDHOOD and is a AD disorder. They appear as defects in keratin 4 and 13 with abnormal tonofilament aggregation. There is hyperplastic acanthotic epithelium in which gross edema causes a basket weave appearance. The oral mucosa is almost invariably affected ( affects the buccal mucosa bilaterally). It may also affect the upper respiratory tract, anus and genitalia. IT IS IN THE FORM OF PAINLESS SHAGGY OR FOLDED WHITE LESIONS. It is a benign condition and reassurance is all that is required. See notes.

Pachyonychia congenita

It is a benign disorder characterized by mutations in keratin. 60% of patients have oral keratoses, 16% have natal or neonatal teeth and 10% have angular stomatitis.

Tylosis and focal PPK Hereditary benign intraepithelial dyskeratosis Darier’s disease

Associated with oral white lesions

Warty dyskeratoma Chronic mucocutaneous candidiasis KID syndrome

It is a autosomal dominant condition .There is oral milky white smooth and transluscent plaques appear by childhood and become more obvious by adolescence. They appear in the buccal mucosa, lips and ventrum of the tongue. Seen in 50% cases of Darier’s disease. They are most marked in the patients with the most severe skin changes and typically flattish, coalescing red plaques that eventually turn white and affect the keratinized epithelia of the dorsum of the tongue, palate and gingival. They resemble nicotinic stomatitis clinically.salivary duct abnormalities including dilations and periodic stricturea and indentations may affect the main ducts. It typically presents as a nodule or papule on the gingival, palate or alveolar ridge. There is suprabasal epithelial clefts and corps ronds. Persistant adherent white lesions are seen in the mouth often with angular stomatitis. There is dental dysplasia, chronic mucocutaneous candidiasis

2. PIGMENTED LESIONS: condition Melanotic macule

feature It is an acquired small (< 2 cm) flat brown to brown black asymptomatic macule usually solitary, benign lesion unchanging in character. It is similar to ephelides and lentigo. They are seen on the vermillon border of the lips, gingival, buccal mucosa and palate. On the lips they are most on the midline of lower lip in the vermillon border. There are no nevus cells and there is only increased pigmentation at the tip of rete ridges.

Peutz- Jeghers syndrome Psuedoxanthoma elasticum Lentiginosis

notes

Centrofacial lentiginosis syndrome Carney complex

There is centrofacial lentigens with bone abnormalities, malformations due to dysraphia, endocrine dysfunctions and neurological diseases

Naevi

They include Peutz- Jeghers syndrome and the LEOPARD syndrome.

It is a autosomal dominant condition. It is a complex of myxomas, spotty pigmentation and endocrine overreactivity. There is cardiac and cutaneous myxomas, mammary myxoid fibroadenomas, spotty cutaneous pigmentation, primary pigmented nodular Adrenocortical disease, testicular Sertoli cell tumours and growth hormone secreting pituitary adenomas. The hyperpigmentation in Carney complex is FACIAL and occurs in the vermillon borders of the lips in 35%, oral mucosa in 8%. 2% have oral myxomas on the tongue and palate. It differs from the Peutz-Jegher’s syndrome in that hyperpigmentation is less intraorally but more common on the conjunctiva and other manifestations are also present. Cases previously desribed as NAME(nevi, atrial myxomas, myxoid nerofibroma, ephelides) syndrome and LAMB(lentigens, atrial myxomas, mucocutaneous myxomas, blue nevi) syndrome may represent this complex. They are much less common than the skin. Majority(60%) are Intradermal and 25% are blue nevi. The other types are also seen. They are seen particularly on the vermillon border of the lips and on the palate or buccal mucosa. They are generally brown macular. There is no evidence that most nevi except junctional nevi progress to melanomas. But excisional biopsy should be done for ruling out melanoma in every case more so if the lesions are raised.

CAUSES OF MUCOSAL PIGMENTATION: localised generalised Amalgam tattoo Racial Ephelides Smoking Nevus Drugs --- phenothiazines, antimalarial, Melanotic macule minocycline, contraceptive, mephenytoin Carney complex Addisons disease PJ syndrome Nelson’s syndrome Malignant melanoma Heavy metal Kaposi sarcoma Albright syndrome

Malignant acanthosis nigricans Hemochomatosis Generalised neurofibromatosis Incontitentia pigmenti Ectopic adrenocorticotropic hormone e.g. bronchogenic carcinoma. 3. RED LESIONS: condition Hereditary hemorrhagic telangiectasia(o sler rendu weber syndrome)

feature There are multiple telangiectasia of the lips, oral and nasal mucosa and perioral areas as well as GIT. Occasionally there are colomic or hepatic complications.

hemangiomas

Most are solitary but a few may be multiple and or part of a wider syndrome such as Maffucci syndrome. Also oral hemangiomas are seen in Sturge weber syndrome, Klippel Trenauney weber syndrome, blue rubber bleb syndrome or Dandy walker syndrome or other posterior cranial fossa malformation.

Hereditary mucoepithelial dysplasia

It is an autosomal dominant dyskeratotic epithelial syndrome affecting oral, conjunctival, nasal, vaginal, urethral, anal and bladder mucosa with cataract, follicular keartosis, nonscarring alopecia anf terminal lung disease. It is probably a pan epithelial cell defect of desmosomal and gap junction structure with a lack of keratinisation and cornification. Histologically the mucosal epithelium shows dyshesion, thinning of the epithelial layer and dyskeratosis. There is red periorificial mucosal lesions are typically noted during infancy and persist throughout life. The oral lesions are painless red macules or maculopapules and are seen predominantly on the palate and gingiva. Severe photohobia, tearing and nystagmus herald the development of keratitis, corneal vascularisation and lens cataract. In addition there are various cardiorespiratory complications especially potentially lethal bullous lung disease ---spontaneous pneumothorax, bullous emphysema terminating in cor pulmonale.

4. VESICULOEROSIVE LESIONS: condition feature Epidermolys Oral lesions are common in the dystrophic and lethal forms of EB. Overall oral is bullosa lesions are found in the 30% cases of EB. There is also a predisposition for SCC mainly in the Hallopeau-Siemens type. Dental hypoplasia, pitting, hypomineralisation and delayed eruption of teeth esp. in junctional variety. There is difficulty in maintaining oral hygiene leading to caries formation. Patients with recessive dystrophic EB suffer from severe growth retardation due to severe orophartngeal and esophageal blistering and scarring. Acrodermati It is a inborn error of metabolism resulting in zinc malabsorption and severe tis zinc deficiency. See notes. enteropathi ca Felty’s syndrome Immune Mouth ulcers (and early onset periodonditis) feature in congenital immune defects defects including Chediak-Higashi Syndrome, Papillon Lefevre syndrome, familial neutropenia, cyclic neutropenia, Job’s syndrome. Chronic granulomatous disease and glycogen storage disease type 1b. 5. LUMPS AND SWELLINg

conditions Hereditary angioedema

Acanthosis nigricans lymphangioma Dermoid cyst Lingual tonsil

Lingual thyroid

Multiple mucosal neuroma syndrome

feature Occurs due to deficiency or dysfunctional C1 esterase levels. It produces a more severe reaction with edema affecting the lips, mouth, face and neck region, the extremities and gastrointestinal tract. Blunt injury is the most consistent precipitating event. The trauma of dental treatment is a potent trigger and some attacks even follow emotional stress. The edema may persist for many hours and even upto 4 days. Airway involvement is a constant threat. There is low C4 levels but C3 levels are normal. Oral papilliferous lesions may be a feature of both familial and malignant acanthosis. Between 30-50% of patients with acanthosis nigricans secondary to neoplasia( malignant acanthosis nigricans ) have oral lesions which involve the tongue and lips predominantly. Frog spawn appearance , usually in the tongue and solitary. It is a hamartoma arising in the midline of the neck above the mylohyoid muscle, occasionally elsewhere, even in tongue and antrum. It usually becomes evident in the second decade. It causes elevation of the tongue. It is a mass of lymphoid tissue in the posterior surface of the tongue between the epiglottis posteriorly and the circumvallate papilla anteriorly. If it enlarges , it can cause globus sensation, alteration of voice, obstructive sleep apnoea or airways obstruction. It tends to involute with increasing age. There may be tonsillitis. It is a smooth surfaced lump in the midline of the base of the tongue at the site of foramen caecum. Occasionally it may produce dysphagia, cough, pain or rarely airways obstruction. The thyroid tissue may be functional or non functional. Malignant change is rare. The syndrome of multiple endocrine neoplasia (MEN) type 2b is inherited as an autosomal dominant trait , some sporadic. It is characterized by medullary carcinoma of the thyroid and pheochromocytoma in association with multiple mucosal neuroma and an abnormal phenotype ---- a striking facial appearance with thick everted lips that usually have a bumpy surface due to multiple neuromas. These are actually mucosal and submucosal hamartomatous proliferations of nerve axons, Schwann cells and ganglon cells. Lesions may also involve the tongue and commisures but are less frequent on the buccal mucosa, gingiva, palate, pharynx or larynx. Ganglioneuromatosis may also occur throughout the GIT . ocular changes include yellowish masses on the conjunctiva, thickened corneal nerves and keratitis due to decreased tear production. Most patients have asthetic marfanoid habitus, high arched palate, pectus excavatum. Ararchnodactyly and kyphoscoliosis.

6. OROCUTANEOUS DISORDERS conditions Cleft lip/palate

Cowden’s syndrome (multiple hamartoma syndrome) Gorlin’s syndrome (naevoid BCC)

Features Are the most common congenital craniofacial abnormalities. They are often accompanied by impaired facial growth, dental anomalies, speech disorder, poor healing and psychosocial problems. A cleft may involve only the upper lip or may extend to involve the nostril and the hard and soft palate. Isolated cleft lip may be unilateral ( mostly on the left) or bilateral. In combination with the cleft palate, they are mostly bilateral. Oral lesions are typically smooth, pink or whitish benign fibromas found especially on the palatal, gingival and labial mucosa. Other manifestations are -----Odontogenic keratocysts of the jaw’

Xeroderma pigmentosa NF-1 Garder’s syndrome Erythropoeti c protoporphy ria Down’s syndrome Tuberous sclerosis

SCC of the lips Intraoral neurofibromas of oral mucosa Multiple jaw osteomas are a feature of garder’s syndrome of familial polyposis coli. Some also have dental anomalies such as supernumery or impacted teeth or odontomas. Psuedoraghades are found.

Angular cheilitis, lip fissures. Pit shaped enamel defects and both dental and gingival fibromatosis

ACQUIRED DISORDERS OF THE ORAL MUCOSA AND LIPS MOUTH ULCERS: The causes of mouth ulcer are ----1. Local Factors  Accidental cheek bite or facial trauma ----- history, single ulcer of short duration (5-10 days)  Orthodontic appliance ---- chronic trauma may cause a well defined ulcer with keratotic halo.  Child abuse  Self mutilation  Cunnilingus or fellatio  Thermal burns  Chemical burns  Irradiation mucositis 2. Recurrent apthous stomatitis  It is characterized by recurring episodes of ulcers, typically from childhood or adolescence each lasting from 1-4 weeks before healing.  Aphthae typically are multiple, round or ovoid ulcers with circumscribed margins, erythematous halo and a yellow or grey floor.  The etiology is unclear. A positive family history is found with about one third and there is increased frequency of HLA-A2,A11, B12, DR2. There are identifiable predisposing factors in some patients ----- low serum iron or ferritin, folate and vit B12 deficiency, celiac disease, stress, trauma, cessation of tobacco smoking.  Ulcers like aphthae are also seen in Behcet’s syndrome, Sweet’s syndrome, HIV infection, cyclic neutropenia and other immunodeficiencies.  There is no evidence that RAS is an autoimmune disease. There is no association with systemic autoimmune disorders, none of the common autoantigens are found and RAS tends to resolve spontaneously with age. The serum immunoglobulins are normal.  Attempts to implicate a variety of viruses or bacteria in the etiology of RAS has largely been unsuccessful but there are cross reacting antigens between the oral mucosa and microorganisms such as Strep. sanguis or its L form, or HSP.  CMI mechanisms appear to be involved in the pathogenesis of RAS. IN THE LESIONS HELPER T CELLS PREDOMINATE EARLY ON WITH SOME NK CELLS. CYTOTOXIC CELLS THEN APPEAR AND THERE IS EVIDENCE FOR AN ADCC.  Clinical features: it is of 3 types ------ the most common are minor apthous ulcers(80%), major aphthous ulcers (10%) herpetiform type of ulceration (10%).  Minor aphthous ulcers or Miculitz ulcers occur mainly in the age group of 10-40 years. They cause minimal symptoms. They are usually 2-4 mm in diameter and are found mainly on the nonkeratinized mobile mucosa. They are uncommon on

the GINGIVA, DORSUM OF THE TONGUE AND PALATE. Only a few (1-6) appear at a time, they heal in 7-10 days and recur at variable intervals. Heal without scar. They are usually round to ovoid.  Major aphthous ulcers or Sutton’s ulcers are larger(even more than 1 cm), recur more frequently, last longer(10-40 days) and are more painful. They can occur anywhere in the oral mucosa including the dorsum of the tongue and palate. They heal with scarring.  Herpetiform ulcers: it is found in older age group and commoner in female. It is extremely painful an drecur so frequently that they seem almost continous. It begins with vesiculation which proceeds to multiple minute ulcers at any oral site. They then coalesce into large ragged ulcers that heal in 10 days or longer.  Treatment: predisposing factors should be corrected.oral hygiene. Steroids, pentoxyphylline, colchicines, dapsone, thalidomide, sucralfate, topical tacrolimus, levamisole. 3. Neoplasms 4. Systemic conditions (a) Hematologic conditions Deficiency states Leucopenia and agranulocytosi s leukemias

Myelodysplasti c syndrome lymphoma Mycosis fungoides psuedolympho ma histiocytosis

Features Low iron, folate or vitamin B12 levels may predispose to aphthae. There may also be other oral features like glossitis, angular stomatitis. Oral ulceration---- painful, deep, irregular ulcers often with only a minimal inflammatory halo involve the mouth and/or pharynx and tend to extend and penetrate slowly. Severe periodontitis is often also a feature. Oral ulceration is a prominent feature especially in acute leukemia. Other manifestations include mucosal pallor, gingival hemorrhage, gingival swelling, petechiae and ecchymoses. Oral infections with Candida and gram negative organisms are common. Ulceration, paresthesia, petechia, burning mouth, gingival swelling, xerostomia and herpes labialis Usually on the palate and pharynx. Can occur elsewhere. They may appear as oral swelling with or without ulceration. Herpes virus infection is common. There is increased incidence of oral lymphomas in HIV. Red or white lesions on the tongue. Tumor like infiltrates are seen in the oral cavity mostly in the palate. Produce lytic bone lesions, gingival swelling, periodontal destruction with loosening of the teeth, mouth ulcers. 111In- pentetreotide imaging may be useful in the diagnosis of Langerhans’ cell histiocytosis.

(b) Gastrointestinal condition Pyostomatitis vegetans Orofacial granulomatosis(O FG)

Feature They are mostly seen in inflammatory bowel disease and the course of the disease follows the associated bowel disease. The oral lesions are deep fissures, pustules and papillary projections. Ulcers classically involve the buccal sulcus where they appear as linear ulcers often with granulomatous masses flanking them. There is also thickening and folding of the mucosa to produce cobblestone type appearance and mucosal tags. Purple granulomatous enlargements may appear on the gingival. The lips and face may swell. There may be splitting of the lips and angular stomatitis. In HPE: non caseating granulomas and lymphomas may be seen located very deep, close to muscle. They occur in

Crohn’s disease

Crohn’s, as an ADR to various food additives such as cinnamaldehyde or benzoates or to menthol or cobalt. Clofazimine 100mg BD for 10 days then twice weekly for 4 months appears to help a majority of cases. It is the most effective drug during the early stages and works by clearing the granulomas. Others include diet excluding additives, IL steroids. Lesions are indistinguishable from OFG.

(c) Dermatological condition Lichen planus

pemphigus

Feature It is a T cell mediated autoimmune disease in which autocytotoxic CD8+ T cells trigger apoptosis of the epithelial cells. OLP occurs in 50% cases of LP and is probably 8 times more common than cutaneous LP. It mainly affects adults > 40 years. It may be involved alone or in association with diseases in the skin and other mucosa. The oral lesions may precede, follow or accompany lesions elsewhere. The association of oral LP with gingival and vulvovaginal lesions is called vulvovaginal- gingival syndrome. Most cases are idiopathic . some lichenoid lesions are related to dental materials --- chromate. Gold and thimerosal. Other lichenoid eruptions may be related to GVHD, drug intake (NSAIDS, sulfones, antimalarials, beta blockers and ACE inhibitor), diabetes or liver disease. Chronic liver disease esp, chronic active hepatitis and hep.C are associated with erosive LP. Pathology is similar to that of skin. They may present as ---- Reticular pattern : an interlacing reticulated pattern of white streaks is the most frequent form. Occurs B/L in the buccal and lingual mucosa.  Papular pattern : it is sually seen with lesions of the reticular pattern  Plaque form: it resembles leucoplakia though reticular pattern can be ssen in the periphery.  Atrophic pattern: red atrophic areas with a peripheral reticular pattern.  Ulcerative or erosive pattern: may develop from an atrophic area or bullous area. Usually affect the dorsum of the tongue or buccal mucosa. The erosions are often large, irregular and surrounded by an area of erythema and glazed surface (due to loss of papilla). Reticular lesions can be seen in the periphery.  Bullous pattern: it is rare. The most common area of involvement is the buccal mucosa. Some patients present with desquamative gingivitis. The atrophic and ulcerative types may be associated with pain and burning sensation. Malignant transformation occurs in less than 1% or so over 5 years particularly with the chronic erosive or atrophic forms. SCC may develop. Therefore follow up all patients at 6-12 monthly intervals. Tobacco and alcohol should be minimized. Treatment includes removal of the triggers, oral hygiene. 1. Mild OLP: Topical steroids(aerosols/pastes) or tacrolimus. Antifungals for candidial superinfection. 2. moderate OLP: Topical ciclosporin or tacrolimus with super potent steroids 3. severe LP: systemic steroids, azoran, endoxan, HCQS, acitretin, thalidomide or ciclosporin. 4. Other therapies ----- include retinoids, dapsone, LMW heparin. There are 2 overlap syndromes --- LP pemphigoides  LP/Lichen sclerosus overlap. Oral lesions are a rule in PV but are rare in the superficial forms of pemphigus. The PV cases which have only oral lesions have antibodies to desmoglein 3. usually the patients present with large painful irregular persistent red lesions in any part of the oral mucosa. Intact blisters are rare. Rarely in PV there may be acquired macroglossia or desquamative gingivitis. Treatment is largely based on systemic immunesuppression . oral lesions are recalcitrant. Try topical

Subepithelia l immune bullous disease

Lichen sclerosus

Lupus erythematos us

steroids/prostaglandin E2/ topical tacrolimus. Apart from PV, the other important pemphigus variant affecting the mouth is paraneoplastic pemphigus with painful extensive stomatitis, painful paronychia and lichenoid papules. Histology and DIF are characteristic.Treatment is immunosuppression. Recent therapeutic advances include the use of anti-CD20 monoclonal antibody( rituximab) and mycophenolate. Oral lesions may be seen in less common pemphigus variants especially in most cases of IgA pemphigus and in some cases pf pemphigus associated with IBD. 1. mucous membrane pemphigoid: Here, autoantibodies are present against different molecules of BMZ. Sera of oral pemphigoid patients selectively and specifically bind to human α6 integrin. Mucous membrane pemphigoid involves the oral mucosa in more than one third of the cases commonly causing gingival lesions. The usual lesion, desquamative gingivitis, is characterized by erythematous, glazed, sore gingival. Bulla are less common and are seen particularly in the soft palate. They rupture to form erosions. The bulla persist longer and may scar. They are typically filled with serous fluid. Treatment: topical steroids, azathioprine, systemic corticosteroids, tetracycline with or without nicotimanide. DAPSONE may be useful especially in the treatment of desquamative gingivitis. 2. Epidermolysis bullosa acquisita, DH, Linear IgA disease and CBDC are other conditions associated with oral erosions. 3. erythema multiforme ----notes 4. TEN --- notes Oral LSEA is uncommon but since it presents with whitish plaques, papules or a reticular pattern or erosions, all features of LP, it may be underdiagnosed. Histologically there is epithelial atrophy with hyperkeratosis, oedema of the papillary dermis and the lymphocytic infiltrate is less close to the epithelium than in LP. It has been suggested that mucosal lichen sclerosus is more common than formerly thought and may even case dysplasia. Almost 50% of LE patients have oral lesions. Which begin as red patches that break down into irregular slit like ulcers and often heal with scarring. Palate is mostly affected. Sjogren’s syndrome may occur with SLE. Oral petechia and herpetic infectios are also common. Similar erosions with a white border are seen in DLE. It may predispose to oral carcinoma. Oral ulcers are also described in drug induced lupus Dermatomyositis and MCTD may be associated with non specific mucosal erosions. Oral lesions in Reiter’s syndrome may include red patches or superficial painless mucosal erosions which may resemble erythema migrans(geographical tongue) both clinically and histologically.

(d) Infective viral Herpes simplex Varicella Herpes zoster HERPENGINA Hand, foot and mouth disease HIV

bacterial Acute necrotizing (ulcerative) gingivitis and noma (syn. Vincent’s angina) Syphilis Tuberculosis

fungal Candidiasis Actinomycosis Aspergillosis Cryptococcosis(indolent oral ulcers) Mucormycosis(black necrotic ulcer in palate) Leishmaniasis Histoplasmosis(ulcerogranulom atous disease) Blastomycosis(do) Coccidiodomycosis(do)

(e) Vasculitis -----polyarteritis nodosa(oral nodules may occur singly or in crops

along the path of vessels and esp in the tongue. Other lesions --- erythema, papule, hemorrhage, ulceration and necrosis) and giant cell arteritis (ischemic pain during mastication, intermittent claudication, ulceration and necrosis of tongue or occasionally lips). (f) GVHD ---- oral manifestation of GVHD consist of painful mucosal desquamation and ulceration and /or cheilitis, and the presence of lichenoid papules or striae. 5. Drugs ---- caustics, cytotoxic drugs, drugs causing erythema multiforme/SJS/TEN. 6. Irradiation of the oral mucosa ORAL SORENESS Causes: 1. Burning mouth syndrome(syn: glossodynia, oral dysaesthesia) It mainly affects the middle aged and elderly females. BMS with a tongue of normal clinical appearance may be seen in deficiency states and with psychogenic causes (a monosymptomatic hypochondriasis or an underlying anxiety about cancer or venereal disease appear to be the basis of the disease in most cases), drugs (e.g. ACEI, cytotoxic agents, protease inhibitors) and diabetes mellitus. Although the tongue is mostly affected, patient may also complain of occasional burning in the lips, gums and palate. THE BURNING SENSATION IS OFTEN BILATERAL AND IS RELIEVED BY EATING OR DRINKING. Diagnosis:

Causes of burning mouth --------LOCAL ---- Candidiasis  Other infections  Geographical tongue  LP  Oral submucosal fibrosis  Dentures SYSTEMIC ---- Psychogenic --- cancerophobia --- depression --- anxiety states ---hypochondriasis  Deficiency states ---- Pernicious anemia ---- vitamin B deficiency ---- folate deficiency ---- iron deficiency.  Diabetes  Drugs(captopril)

Management of BMS includes reassurance, treatment of underlying abnormality, psychological treatment like antidepressants for 2-3 weeks. 50% resolve spontaneously over 6-7 years. Others include topical benzydamine 0.01% rinse or spray, topical capsaicin cream 0.025%, clonazepam. WHITE LESIONS condition

feature

Cheek bite burns

Whitish shredded appearance usually of the buccal or lower labial mucosa at the occlusional line. Seen in tense and anxious individuals. Due to holding mouth washes, drugs against the buccal mucosa. They cause white sloughing lesions in the mucosa. They typically heal in 1-3 weeks.

LP Candidias notes is leukoplak It is defined as a white patch or plaque on the mucosa that cannot be rubbed off ia and that is not recognized as a specific disease entity. The term is also used irrespective of the presence or absence of epithelial dysplasia. It is common in adults, around 1% is affected. Most cases are seen between 50-70 years age group. It can be totally benign or sometimes can be precancerous or a marker of cancer elsewhere in the upper aero digestive tract. Oral See notes below. keratoses Hairy It is seen in severe immune defects especially HIV infection. Occasionally in leukoplak immunocompetant. It is caused by EBV. HL is a white patch usually seen in the ia parakeratinized mucosa of the tongue frequently bilaterally. The lesions are hyperplastic whitish plaques with a corrugated hairy appearance. They are mostly symptomless. Have no premalignant potential. Histologically there is hyperparakeratosis. HL needs no treatment and in HIV resolves with antiretroviral medications. psoriasis Oral white lesions, lesions like geographical tongue can occur in the buccal mucosa ( annulus migrans) particularly in pustular psoriasis Kopliks White specks may be seen in the buccal mucosa opp the first upper molar tooth spots in measles.

condition Black hairy tongue

Pigmentary incontinence Tattoos Food habits

PIGMENTED LESIONS Features The coating in most cases appears to be of epithelial food and microbial debris; indeed, the tongue is the main oral reservoir of some microorganisms such as Candida albicans and viridans streptococci. The filiform papilla are excessively long and stained by accumulation of squames and chromogenic microorganisms. This mostly occurs in the adults who are edentulous, on soft non abrasive diet, have poor oral hygiene or are fasting. The coating appears more obvious in xerostomia. Habits such as tobacco and betel use and various medicaments such as chlorhexidine or iron can cause black or brown superficial staining of the tongue. Occasionally a brown hairy tongue may be caused by drugs that induce xerostomia, lansoprazole or antimicrobial therapy. IT MAINLY AFFECTS THE POSTERIOR PART OF THE DORSUM OF THE TONGUE, ESPECIALLY CENTRALLY. Treatment: oral hygiene, avoid drugs, brush tongue with hard tooth brush. Topical tretinoin may be effective. In LP. Amalgam tattoos are common causes of blue black pigmentation of the gingival. Causes include --- Foods and beverages ( beetroot, red wine, coffee and tea) cause superficial staining  Confectionary such as liquorice causes superficial staining  Smoking tobacco is now a fairly common cause and cause extrinsic discoloration as well as intrinsic pigmentary incontinence. This is more in the case of reverse smoking.  Chewing betel may cause superficial brownish red discoloration mainly in the buccal mucosa with an irregular epithelial surface that has tendency to desquamate. The epithelium in betel chewer’s

mucosa is often hyperplastic and brownish amorphous material from the betel quid may be ssen intracellularly and intercellularly with ballooning of epithelial cells. Betel also predisposes to submucous fibrosis and cancer.  Drugs such as chlorhexidine, iron salts, griseofulvin, crack cocaine, minocycline, lansoprazole and HRT. The first two cause superficial staining. Drugs that cause intrinsic staining are ------- Antimalarials --- produce a variety of colors ranging from yellow(mepacrine) to blue black(amodaquine).  Minocycline ----- black discoloration of teeth, gingival and bone, skin, sclera and even breast milk. In a minority it produces a blue grey gingival pigmentation caused by staining of the underlying bone an dsome intrinsic faint bluish grey staining of the anterior teeth.  Busulphan, OCP, phenothiazines, anticonvulsants may occasionally produce brown pigmentation.  ACTH, zidovudine and clofazimine may also produce brown pigmentation.  Gold produces purplish gingival discoloration . ACTH induced hyperpigmentat ion HIV infection Oral mucosal melanotic macule

The brown or black pigmentation is variable in distribution but is seen typically on the soft palate, buccal mucosa and at sites of trauma.

Malignant melanoma Kaposi’s sarcoma

Rare and occurs most in the palate and maxillary alveolus.

Oral pigmentation is seen due to drug or adrenal hypofunction It occurs suddenly as a reactive lesions following trauma mostly in black people. It appears in a course of days to weeks and resolves spontaneously within 6 months. Melanin content is increased but not the number of melanocytes.

Occur mostly in HIV infected patients over the palate initially as redpurple macule that progresses to nodule that may be extensive and ulcerative. Multiple lesions are common. They are often asymptomatic but some are painful and bleed. Treatment is local radiotherapy/ laser removal/ systemic vinca alkaloids/ IL vinblastin. Occasionally it may regress spontaneously or with HAART, zidovudine or systemic vinca alkaloids. RED LESIONS

Condition Goegraphic al tongue ( benign migratory glossitis)

Larva migrans Strawberry tongue Telangiectas ia

Feature It is characterized by map like areas of erythema . the pattern changes from day to day and even within a few hours. They may be asymptomatic or cause sore tongue. There is increased thickness of the intervening filiform papilla. There may be family history. There is association with HLA B15 AND DR7. some have atopy and some relate it to specific food items. Similar lesions also seen in Reiter’s syndrome, generalized pustular psoriasis and acrodermatitis continua of Hallopeau. Rarely other sites like the labial and palatal mucosa may be affected. Pathologically there is epithelial thinning at the centre of the lesion with an inflammatory infiltrate mainly of PMNL. Irregular linear lesions with inflammatory border. Prominence of the lingual papilla may be seen in scarlet fever, Kawasaki disease and Riley Day syndrome. In Osler rendu syndrome, CREST, chronic liver disease, pregnancy and after irradiation.

Venous lake, pyogenic granuloma, hemangioma, angiosarcoma. candidiasis erythroplasi It is a red velvety lesion in level with or depressed below the surrounding a mucosa. Uncommon. It occurs in 6-7th decade. 75-90% of cases of erythroplasia prove to be carcinoma or carcinoma in situ or show severe dysplasia. The incidence of malignant change in erythroplakia is 17 times higher than leukoplakia. Therefore these areas should be excised and sent for HPE. Glossitis, desquamati ve gingivitis LOSS OF ELASTICITY OF ORAL LESIONS Condition Oral submucos al fibrosis

Systemic sclerosis

Feature It is a chronic disease of the oral mucosa that appears to be caused by the constituents of areca nut. There is a subepithelial chronic inflammatory reaction with fibrosis extending to the submucosa and muscle. Epithelial changes range from atrophy to keratosis and dysplasia. It develops insidiously often presenting with dysaesthesia or vesicular stomatitis. Later there may be symmetric fibrosis of the cheeks, lips and palate and noted as bands. It can become very severe that the affected site becomes white and firm with restricted mouth opening. It can predispose to oral carcinoma. Diagnosis by biopsy. Changes are --- restricted mouth opening with radiating fissures, telangiectasia, xerostomia, mandibular erosions, increased width of the periodontal ligament space of all teeth on radiography. Caries and periodontal disease. LUMPS AND SWELLING

Conditions mucocele

Oral papilloma

warts Focal epithelial hyperplasia (Heck’s disease) Denture granuloma epulis Giant cell epulis

Parotid duct cyst Fibroma

Features It is usually seen in lower labial mucosa usually resulting from the escape of mucous into the lamina propria from damaged minor salivary gland duct. They appear as painless, transluscent dome shaped whitish blue papules or nodules These are caused by HPV. They are most common at the junction of the hard an soft palate. It is a white or pink cauliflower like lesion that may resemble a wart. They remain benign. Excision must be total deep and wide enough to include any abnormal cells beyond the zone of the pedicle. Common wart and Condyloma acuminata are rare in the mouth but are mosr common in HIV. It is a rare benign familial disorder characterized by multiple soft circumscribed sessile nodular elevations of the oral mucosa. It is mostly caused by HPV-13 and HPV-32. it usually affects the lower lip and tongue. This growth appears on the labioalveolar fold as a localized firm, whitish, fissured. Fibrous granuloma. Any benign gingival tumor is called epulis. The most common is the fibrous epulis which appears as a hard, broad based nodule. It is a fibroma It is a bluish red gingival tumor dur to reactional hyperplasia of the mucoperiosteum and excess production of granulation tissue due to chronic irritation. Pregnancy epulis is painless though unsightly and may ulcerate and bleed. Develops opp upper second molar tooth on the buccal mucosa in musicians who play wind instruments. Pedunculated or sessile nodule that may occasionally get ulcerated in an area of the oral cavity but seems to have predilection for sites of

lipoma Leiomyoma neurofibromas Rhabdomyoma Rhabdomyosarco ma Neurilemmoma (schwannoma) Salivary gland adenoma Torus palatinus

trauma. Soft, compressible yellowish nodules mostly on the buccal mucosa or floor of the mouth. It is situated on the palate or tongue as bluish or red circumscribed firm tumors Oral lesions are not unusual and involvement of the tongue leads to macroglossia. Most extracardiac rhabdomyomas present in the mouth typically as lumps in the floor of the mouth, tongue or soft palate. Most are seen in the 6th decade. The most common oral presentation is a progressively enlarging mass; some 20% have enlarged regional lymph nodes. In advanced disease, there may be pain, paraesthesia, trismus or loosening of the teeth. The sites commonly involved in this tumor are the tongue and floor of the mouth. The lesions appear as sessile nodules softer than a fibroma. Excision is the treatment of choice. They appear mainly on the palate but may occur as slow growing tumors anywhere in the mouth These are bone excesses found in the midline of the hard palate.

SCROTAL TONGUE SYN: Fissured tongue, lingua plicata, lingua fissurata Congenital fissuring of the tongue is a developmental disorder.  The tongue is larger than normal. Its dorsal aspect exhibits numerous plicate and superficial or deep grooves or fissures usually arranged in an arborized pattern connected to longitudinal furrow along the median raphe.  The malformation, which has a familial tendency, may affect 3-5 % of the population.  It is frequently associated with Down’s syndrome and is a component of Melkersson Rosenthal syndrome.  It has a frequent association with geographic tongue  It is usually asymptomatic  Food deposits may get embedded in the tongue and cause chronic inflammation. Does not usually require any treatment except for mouth washes. MACROGLOSSIA Causes: 1. Congenital -------------- lymphangioma, Hemangioma. 2. Metabolic disorders ---------- primary amyloidosis, mucopolysaccharidosis, and glycogen storage diseases. 3. Endocrine disorders ------------ acromegaly, hypothyroidism 4. neoplastic (benign) --------- neurofibroma, granular cell myoblastoma, rhabdomyoma, glomus tumors 5. malignant ------------- metastatic carcinoma, SCC, adenocarcinoma, sarcoma, multiple myeloma. 6. miscellaneous ------ angioneurotic edema, nutritional disorder (iron deficiency, pellagra, pernicious anemia), trauma, infections (TB, syphilis and actinomycosis). INFECTIONS OF THE ORAL CAVITY 1. Acute necrotizing(ulcerating) gingivitis (ANUG) ----- Syn. Vincent’s infection  It is a mixed, mainly anaerobic flora consisting mainly of fusobacterium nucleatum and Borrelia vincentii is associated with this infection.  Immune depression may be a predisposing cause  It may occur in an epidemic form in institutions or in military camps.

 The mouth ulcer is restricted to the interdental papilla of the gingival which appear blunted. Acute onset of gingival soreness and bleeding and halitosis is characteristic. ANUG occurs in the anterior part of the mouth. Fever, cervical lymphadenopathy are present. Failure to adequately treat ANUG may lead to noma (cancrum oris).  In noma, the infection extends onto the skin and bones to result in destructive lesions with a fatal outcome. The condition occurs in malnourished and debilitated children.  Similar lesions of gangrenous stomatitis are increasingly reported in HIV disease.  Treatment consists of cleaning with hydrogen peroxide, soft tooth brush, oral metronidazole 200 mg TDS for 3-7 days. 2. Tuberculosis: Tuberculosis of the oral mucosa can be classified as ----(a) Primary ------ inoculation ( tuberculous chancre) ------ hematogenous ( miliary tuberculosis) (b) Secondary ----- ulceration or granuloma ----- tuberculosis cutis orificialis ----- mucosal extension of tuberculosis from osteomyelitis (c) Mycobacterial oral ulcers by MAI in AIDS. (d) M. chelonei may occasionally cause cervicofacial infection in the form of lymph node abscess or occasionally intraoral swellings. 3. gonorrhoea: Although rare, gonococcal stomatitis has been reported in oral sex with an infected partner. The oral mucosa appears intensely inflamed with multiple superficial erosions and ulcers which are covered by a yellowish psuedomembrane. 4. syphilis: notes 5. herpes simplex infection: notes 6. varicella 7. herpes zoster:  the pain in trigeminal zoster may simulate toothache  In mandibular zoster, there is ulceration of one side of the tongue, floor of the mouth and lower labial and buccal mucosa.  In maxillary zoster, one side of the palate, the upper gingival and buccal sulcus.  Rarely mandibular or maxillary zoster may disturb the formation of developing teeth or cause jaw necrosis.  If geniculate ganglion of the facial nerve is affected, there may be unilateral facial palsy with vesicles in the ipsilateral ear and ulcers in the soft palate. 8. herpengina  it is caused by Coxsakie virus in children.  The incubation period is 3-7 days  It begins with fever followed by the appearance of minute vescicles and erosions scattered over the pharynx, soft palate(mainly) and tonsils. There is enlarged and tender anterior cervical lymphadenopathy. There is sore throat.  The condition is self limiting. 9. hand foot and mouth disease  it is mainly caused by Coxsakie A virus but sometimes by Coxsakie B virus or enterovirus.  The I.P. is 3-10 days  Young children are particularly affected.  Many infections are subclinical but features of the clinical syndrome include the following -----------

General features like malaise, anorexia, irritability and fever. Anterior cervical lymphadenopathy ------- enlarged and tender Mouth ulcers are round or ovoid, usually sparse and may affect any site Rash ----- painful sometimes deep seated vesicles may appear usually on the hand and/ or feet particularly on the digits or at he base of the phalanges.  It is self limiting. Occasional encephalitis.  It is severe in adults.

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ORAL KERATOSIS Etiology: 1. Idiopathic 2. tobacco chewing ----- M 3. reverse smoking ------M 4. cigarette induced keratoses -------- U 5. pipe smoking ------ U 6. cigar smoking ------ U 7. Sniff dipper’s keratoses and other smokeless tobacco lesions -------- R 8. HPV induced proliferative verrucous leukoplakia --------- U 9. Candidial leukoplakia ------- U 10.Syphilitic leukoplakia -------U 11.Hairy leukoplakia -------- not recorded. CLINICAL FEATURES:  Leukoplakias vary in size, some are small and focal and others more widespread  They may be homogenous white plaques that can be faintly white or very thick and opaque. Or they can be nodular white lesions or lesions admixed with red lesions.  The malignant potential depends on the following ---------(a) Appearance  Homogenous leukoplakia has little malignant potential  Nonhomogenous or heterogenous leukoplakia are nodular, speckled with red patches, ulceration ------- they have high risk of malignancy. (b) Site  soft palate complex, ventrolateral tongue and floor of the mouth ------ high malignancy risk. (c) Etiological factors ---- see chart. Biopsy is mandatory in those leukoplakia which exhibit the following -----------

 Found in patients with previous or concurrent head and neck cancer.  Are non homogenous ----- have red areas, ulcerated, verrucous, idurated.  In high risk areas like the floor of the mouth and tongue  Focal  With symptoms  Without obvious etiological factors. Pathology:  They show, to a varying degree, increased keratin production, change in epithelial thickness and disordered epithelial maturation.  Mild dysplasia is not usually regarded as of serious significance  Severe epithelial dysplasia is thought to indicate a risk of malignancy.  Pagetoid dyskeratosis is considered a selective keratinocyte response in which part of the normal population of keratinocytes is induced to proliferate in response to friction.  Pagetoid cells are more common in suprabasal location and in the labial mucosa.

 These cells show positivity for high molecular weight cytokeratin and negative reaction for low molecular weight cytokeratin, epithelial membrane antigen, CEA and HPV.  The Immunohistochemical profile and morphology is also different from surrounding keratinocytes. D/D:      

White sponge nevus. Leukodema. Oral koilocytosis. Hairy leukoplakia. Pagetoid SCC. Extramammary Paget’s disease of the oral mucosa.

Prognosis:

1. Leukoplakia: 2-5% of leukoplakias become malignant in 10 years and 5-20% of leukoplakia is dysplastic.

2. 15-30% regress spontaneously. 3. At present, it is not possible to predict which dysplastic lesions will progress to carcinoma and which will regress. Management: 1. removal of risk factors (tobacco, alcohol and trauma). 2. surgery is an obvious option for the management of leukoplakia with high predisposition for malignant predisposition to malignant transformation such as leukoplakia that are ---- speckled  verrucous  from high risk sites (e.g. floor of the mouth/ventrum of the tongue or soft palate/ fauces)  in a patient with previous cancer of the upper aerodigestive tract  dysplastic  polysomic (aneuploidy or tetraploidy)  positive for genetic markers such as mutated tumor suppressor factor p53 or for loss of heterozygosity on chromosomes 3p and 9p. 3. Chemotherapy and chemoprevention:  Topical 0.5% bleomycin  Topical or systemic vitamin A derivatives  Calcipotriol. GINGIVAL SWELLING Causes are ------1. LOCAL  Chronic gingivitis  Chronic periodontitis  Acute necrotizing gingivitis 2. SYSTEMIC  Any condition causing exacerbation of gingivitis (e.g. pregnancy)  Leukemia  HIV infection  Other causes of purpura  Clotting defects  Drugs ------- e.g.anticoagulants  Scurvy.

Causes of gingival bleeding ------ GENERALIZED SWELLING  Chronic gingivitis  Hyperplastic gingivitis due to mouth breathing  Hereditary gingival fibromatosis  Drugs  Pregnancy  Sarcoidosis  Crohn’s disease  Leukemia  Wegener’s granulomatosis  Scurvy  Amyloidosis  Mucopolysaccharidosis  Mucolipidosis  Lipoid proteinosis  Juvenile hyaline fibromatosis  LOCALIZED SWELLING  Abscess > pregnancy  Cysts > sarcoidosis  Pyogenic granuloma > orofacial granulomatosis  Neoplasm > Crohn’s disease, WG  Wart > amyloidosis