Theme 6 - oral ulceration and mucocutaneous lesions

Question 1

What is oral lichen planus?

Answer 1

Common chronic inflammatory disorder affecting stratified squamous epithelia. Typically are small white papules that often coalesce and form lines (Whickham’s striae). Typically bilateral, F>M, 50-60years old.

Question 2

What are the clinical features of oral lichen planus?

Answer 2

• Bilateral but not always symmetrical
• Spontaneous exacerbations and improvements
• Striations and erythematous change
• Posterior buccal mucosa most frequent site followed by tongue, gingiva, labial mucosa and lower lip
• Palate, FoM and upper lip uncommon
• Lesions migrant and can spread

Question 3

In white OLP, describe the following types and their differential diagnosis:
a) Papular oral lichen planus
b) Reticular oral lichen planus
c) Plaque like oral lichen planus

Answer 3

a) Small white patches on buccal mucosa. DD = fordyce granules (these are more yellow)
b) Most common appearance, white striations, pt unaware of presence. DD = oral lichenoid lesions, geographic stomatitis
c) Long lasting striations that have coalesced to form patches. DD = leukoplakia, proliferative verrucous leukoplakia (PVL)

Question 4

What are 5 things that would suggest it is oral lichen planus?

Answer 4

1) Multifocal lesions
2) Papules and/or striae can be seen
3) Lesions involve dorsum of tongue
4) Typical cyclical course
5) Histology suggestive

Question 5

In Red OLP, describe the following:
a) Atrophic (erythematous) OLP
b) Erosive and bullous OLP

Answer 5

a) Erythematous lesions that affect the gingiva cause dequamative gingivitis. Frequently involve dorsum of tongue. Discomfort.

b)Erosions and straitions, unusual to see blistering. Intense pain and burning. Hardly remit spontaneously and may lead to confusion wuth other autoimmune mucosal blistering diseases e.g. PV

Question 6

What are the extra-oral manifestations of OLP?

Answer 6

Cutaneous lesions:
-can develop within several months after appearance of oral lesions
- Usually erythematous to violaceous papules that are flat-topped
- Most commonly volar wrists and forearms
- Self-limiting and cause itching

Genital LP:
- Genital mucosa affected in women with OLP = vulvovaginal-gingival syndrome
- Burning, pain, vaginal discharge, dyspareunia
- Penogingival syndrome less common, malignant change has been reported

Scalp:
- Planopilaris - causes scarring and alopecia

Nails:
- Nail plate damage, onycholysis, longitudinal striation

Question 7

What is the histopathology of OLP?

Answer 7

• Hyperkeratosis
• Basal cell liquification with apoptotic cells
• Band like inflammatory infiltrate (T-lymphocytes)
• Absence of epithelial dysplasia

Question 8

What is the pathogenesis of OLP?

Answer 8

T cell mediated autoimmune disease which CD8 and T cells trigger apoptosis of oral epithelial cells

Question 9

What is the classification of lichen planus and lichenoid lesions?

Answer 9

Oral lichen planus
Oral lichenoid lesions:
- Oral lichenoid contact lesion (OLCL)
- Oral lichenoid drug reaction (OLDR)
- Graft vs host disease - oral lichenoid lesion (GVHD-OLL)

Question 10

Why is biopsy and histological diagnosis of OLP/OLL recommended? What cannot be reliably distinguished?

Answer 10

To exclude dysplasia or maligancy
Histopathology cannot reliably distinguish between OLP and OLL

Question 11

What is the etiology of OLP/OLL?

Answer 11

• Genetic predisposition (cytokine polymorphisms)
• Dental materials (amalgam)
• Viruses (mainly HCV - liver disease associated with LP)

Question 12

How can you distinguish oral lichenoid contact lesion and oral lichen planus?

Answer 12

OLCL has close contact relationship with fillings.
Is not bilateral/symmetrical

Question 13

What is the evidence for dental materials causing OLCL?

Answer 13

Amalgam components, friction and plaque should be considered.
Replacement of amalgams can result in improvement but not all

Question 14

What is lichenoid dysplasia? What is its aetiology>

Answer 14

Oral lichen planus could be a premalignant condition - dysplasia with lichenoid features. OLP pts have increased risk of developing oral cancer so must be followed up.
Aetiology:
- Erosive variety
- Smoking, alcohol
- Viruses (HPV, HCV)
- OLP therapy
- Candida
- Diet

Question 15

In the oral mucosa, what is the
a) mucosa
b) submucosa
made up of?

Answer 15

a) Lining, epithelium, lamina propria (layer of connective tissue)
b) Fibrous and adipose tissue, minor salivary glands, neurovascular bundles

Question 16

What is the difference between
a) Masticatory mucosa
b) Lining mucosa
c) Specialised mucosa
and where are they each found?

Answer 16

a) Keratinised epithelium: Keratin layer, granular cell layer (loss of organelles), prickle cell layer (desmosomes anchor cells together), basal cell layer. Found gingivae and hard palate
b) Non keratinised epithelium. Found labial and buccal mucosa, ventrum of tongue, FOM, soft palate
c) Keratinised epithelium. Found dorsum of tongue, filiform, fungiform and circumvallate papillae

Question 17

Both clinically look like white patches, but what is the histology of
a) Hyperkeratosis
b) Hyperorthokeratosis

Answer 17

a) Retained nucleus in keratinised layer
b) No nuclei, excessive thickness

Question 18

What are the 3 white lesions due to ketatosis of the oral cavity that are hereditary?

Answer 18

1) White sponge naevus:
- Bilateral and symmetrical usually on BM
- Raised shaggy white plaque
- Autosomal dominant - mutation to keratin 4 and 13

2) Genodermatoses ie inherited skin conditions
- Dyskeratosis congenita (increased risk of SCC, seen in young when LP unusual! Skin hyperpigmentation, anaemia, thrombocytopenia etc)
- Tylosis (increased oesophageal cancer risk, hyperkeratosis palms and soles)
- Follicular keratosis (Darier’s disease)

3) Pachyonychia congenita
- Palmar plantar keratosis
- Gross thickening of nails
- Keratosis of oral cavity
- Similar to white sponge naevus but skin lesions
- Autosomal dominant

Question 19

What are the 3 white lesions due to keratosis that occur from trauma?

Answer 19

1) Frictional keratosis
- Cheek/lip chewing chronic e.g. linear alba

2) Chemical trauma
- Aspirin burn
- Oil of cloves
- Denture cleanser

3) Smokers keratosis
- chemical and thermal
- White plaques and melanosis (brown pigmentation)
- Inflamed salivary glands

Question 20

What are the 4 white lesions due to keratosis that occur from infections?

Answer 20

1) Syphilis (Treponema palidium)
- Sites: dorsum of tongue, syphilitic glossitis, syphilis leukoplakia
- Snail tracks and mucous patches = secondary, atrophic glossitis = tertiary
- Hutchinson incisor and mulberry molars
- Risk of malignant change

2) Chronic hyperplastic candidiasis
- White patches not rubbed off
- Sites: commissures, BM, lips tongue palate
- Antifungals don’t work
- Risk of malignant change (cellular atypia)
- Remember pseudomembranous candida are the white patches that wipe off

3) Hairy leukoplakia
- HIV patients/immunosuppression
- Site: lateral border of tongue
- Vertical white folds - corrugated
- Associated with EBV and candida
- No evidence of maliganant change

4) Squamous cell papilloma
- Lip, junction of hard/soft palate
- Common
- HPV
- Soft, painless lesion with fingerlike surface projections, pedunculated and asophytic (grow outwards)

Question 21

What are the 2 white lesions due to keratosis that occur from dermatological causes?

Answer 21

1) Lichen planus
- Type IV hypersensitivity - T cell triggered
- Reticular, plaque like or papular
- Erosive, atrophic, desquamative gingivitis
- Can have skin and genital lesions

2) Lupus erythematosus
- Discoid or systemic
- F>M
- Auto-immune disease type III hypersensitivity
- Multisystem: skin, joints, kidney, cardiac, pulmonary, neuro
- Butterfly rash

Question 22

What is a white lesion due to keratosis that occurs idiopathically?

Answer 22

Leukoplakia

Question 23

What is oral epithelial dysplasia?

Answer 23

Used to describe histological changes that suggest increased risk of malignant transformation in oral potentially malignant disorders. Recognises features of cytological and architectural atypia

Question 24

What is a) Homogenous leukoplakia and b) Non-homogenous leukoplakia

Answer 24

a) Asymptomatic uniform smooth surface, sharp demarcated, shallow fissures
b) Diverse appearance, speckled red/white, nodular, verrucous. Carries a higher risk of malignant transformation

Question 25

If a patient presents with leukoplakia what is important to find out from their social history and why? What other features are important when predicting risk of malignant progression?

Answer 25

If ever smoked. If a non-smoker may have more aggressive course Size, red specked, buccal mucosa tongue and FOM high risk sites

Question 26

What is erythroplakia?

Answer 26

Fiery red patch, sharply demarcated, flat or depressed. Erythematous mucosa with matt appearance. Unilateral isolated high suspicion of dysplasia

Question 27

What is the differential diagnoses for a red patch in the mouth?

Answer 27

- Erythroplakia - Denture induced stomatitis - Erosive LP - Pemphigus vulgaris - Erythema migrans/geographic tongue - Erythematous candida - Discoid lupus

Question 28

What is proliferative verrucous leukoplakia?

Answer 28

Multifocal oral leukoplakia with progressive course, starting as one patch then confluence. Frequently gingivae, alveolar process and palate. No erythema or ulceration. Histologically: verrucous, hyperkeratotic, lichenoid, dysplasia Has the highest risk of malignant change

Question 29

What is oral submucous fibrosis?

Answer 29

Blanching of mucosa, loss of tongue papillae, fibrous bands, tongue rigidity, shrunken uvula, trismus. Associated with betel nut chewing. Fibrosis of the lamina propria.

Question 30

Does OLP and lichenoid lesions have malignant potential?

Answer 30

Yes - if biopsy shows dysplasia then it should be called OLP with dysplasia

Question 31

What is oral lupus erythematous?

Answer 31

Chronic autoimmune disease: systemic, drug induced or discoid. Similar to OLP but may present with central erythema with surrounding straie whereas OLP more disorganised Malignant potential - carcinomas rare and most often on lips

Question 32

What is oral graft vs host disease (GvHD)?

Answer 32

Chronic form more likely to cause oral lesions. Result of allogenic haematopoetitic cell transplantation for malignant and non malignant conditions. Can manifest as scleroderma like changes, mucoceles, ulceration, OLP looking. Can progress to oral cancer

Question 33

What is reverse smoking and what lesions does it cause?

Answer 33

Palatal lesions as a result of holding the burning end of a cigarette in the mouth - malignancy on hard palate. thickened white plaque or erythroleukoplakia, erythema of salivary gland orifices

Question 34

What is dyskeratosis congenita?

Answer 34

Rare hereditary condition. Triad of oral leukoplakia, hyperpigmentation of skin, nail dystrophy. Prognosis poor: malignant change in oral lesions or bone marrow failure

Question 35

What is the pathway for mild epithelial dysplasia?

Answer 35

Routine monitoring for 5 years or if risk factors then oral epithelial dysplasia clinic for close monitoring, re biopsy or resection

Question 36

What is the treatment for oral graft vs host disease?

Answer 36

Like OLP - topical steroid and topical calcineurin inhibitors e.g. tacrolides. On top of immunosuppression

Question 37

What is the clinical presentation of recurrent aphthous stomatitis?

Answer 37

- Superficial oral mucosal ulceration - Recurrent - No obvious local cause - Self-limited - Otherwise healthy individual - Usually on non-keratinising oral epithelum

Question 38

What is the diagnostic criteria for minor RAS?

Answer 38

- Round or oval shaped - 2-10mm in diameter - Usually 1-5 per crop - Predominantly on non-keratinised mucosa - Heal without scarring - Last 7-14 days - Have peri-ulcer erythema in early stages

Question 39

What is the diagnostic criteria for major RAS?

Answer 39

- Round or oval shaped - 10+mm in diameter - Usually 1-2 per crop - Predominantly on non-keratinised mucosa - Heal with scarring - Last 14+ days - Variable peri-ulcer erythema

Question 40

What is the diagnostic criteria for herpetiform RAS?

Answer 40

- Round or oval shaped merging to form irreglar shapes - Initially less than 2mm in diameter - Usually 10+ per crop - Occurs on both keratinised and non-keratinised mucosa - Heal with or without scarring - Last 7-21 days - Have peri-ulcer erythema

Question 41

What is complex aphthosis?

Answer 41

Presence of almost constant multiple oral or oral and genital aphthae in the absence fo systemic manifestations

Question 42

What is the aetiology of RAS?

Answer 42

- Genetics (family history) - Haematinic deficiency e.g. iron, folic acid, vit B1, B2, B6 and B12 def - Gluten sensitive enteropathy - Food allergy e.g. chocolate, coffee, peanuts - Sex hormones e.g. occasional improvement with pregnancy - Drugs e.g. NSAIDs and B Blockers, nicorandil may cause major aphthae - Psychiatric illness - Immunodeficiency (humoral and cellular) - Infections e.g. bacteria, viruses - Local trauma (white halo)

Question 43

How is RAS diagnosed?

Answer 43

Exclusion of other causes FBC, ferritin, vit B12, red cell folate

Question 44

What are apthous-like ulcerations (ALU)?

Answer 44

Oral ulcerations that run a more complex clinical course requiring more extensive medical screening and management than RAS

Question 45

What diseases are apthous-like ulcerations (ALU) related to?

Answer 45

- Becets disease - MAGIC syndrome - Reiters syndrome - Cyclic neutropenia - PDAPA syndrome - Inflammatory bowel diseases

Question 46

What is cyclic neutropenia? What is its presentation?

Answer 46

Periodic decrease in the circulating neutrophil numbers from normal to very low. Autosomal dominant inherited. Usually recurs every 21 days and lasts 3-5 days Presentation - Recurrent infection of the sinuses, respiratory tract and skin - Inflammation of the throat and gums - Apthous like ulcers in mouth and colon - Recurrent fever - Abdominal pain

Question 47

What is periodic fever adenitis pharyngitis apthous ulcer syndrome (PFAPA)? What is its presentation?

Answer 47

Recurrent fever occuring every 3-6 weeks, lasting 3-5 days. Pharyngitis. Cervical adenitis. Aphthous stomatitis.

Question 48

How is PFAPA diagnosed?

Answer 48

1) Regularly recurring fevers with early age of onset 2) Aphthous stomatitis, cervical lymphadenitis, pharyngitis 3) Completely asymptomatic between episodes 4) Normal growth and development 5) Exclusion of cyclic neutropenia with blood test

Question 49

What is mouth and genital ulcers with inflamed cartilage (MAGIC syndrome)? How is it diagnosed?

Answer 49

An overlap of Becets disease and relapsing polychondritis Diagnosis at least 3 criteria: - Bilateral auricular chondritis - Serum negative non-erosive inflammatory polyarthritis - Nasal chondritis - Ocular inflammation - Respiratory tract chondritis - Audiovestibular lesion

Question 50

What is Behcets disease?

Answer 50

Multisystem inflammatory disorder characterised by recurrent apthous ulcers, occluar inflammation and involves joints, skin, CNS and GIT. Chronic condition and goes through phases

Question 51

What is the diagnostic criteria for Becets disease?

Answer 51

1) Recurrent oral ulceration (at least 3x year) plus one of the following: 2) Recurrent genital ulceration 3) Eye lesions 4) Skin lesions 5) Positive pathergy test Or point system, oral genital and ocular score 2, skin neuro and vascular score 1. 4+ = becets

Question 52

What is the epidemiology for Becets disease?

Answer 52

Common in Turkey and Middle East Onset 30-40 years Slightly higher M>F

Question 53

What is the difference between Becets disease and RAS?

Answer 53

In becets - More agressive ulcers - Major apthous like ulcers more common - Involvement of multiple sites more common - Involvement of soft palate and oropharynx more common

Question 54

How is Becets disease diagnosed?

Answer 54

Presence of aphthae FBC Erytrocyte sedimentation rate (ESR) C-reactive protein (CRP) Referral to dermatology, opthalmology, gastroenterology, neurology and reumathology when appropriate

Question 55

What is the management of Becets disease?

Answer 55

Topical corticosteroids for oral and genital ulcers. Colchicine orally for skin lesions

Question 56

What is the difference between pemphigus and pemphigoid?

Answer 56

Pemphigus = intra epithelial blister - altered adhesion between keratinocytes (acantholysis). Intercellular depositis of IgG and C3 in immunofluorescence. Pemphigoid = subepithelial blister - altered adhesion at BMZ

Question 57

What are the target antigens of a) Pemphigus vulgaris b) Pemphigus foliaceus c) Paraneoplastic pemphigus d) IgA Pemphigus?

Answer 57

a) DSG3 (desmoglein) b) DSG1 c) DSG 3 d) DSC1 (desmocollin)

Question 58

What is the epidemiology of pemphigus vulgaris?

Answer 58

- M=F - More common 30-60years - Meditarranean/Jewish ancestry (HLA-DR4 = ashkenazi Jews) - Potentially lethal - but prognosis involving oral cavity significantly better than that of mucotaneous PV

Question 59

What is the aetology of pemphigus vulgaris?

Answer 59

- Drugs containing sulfhydryl radical (thiol drugs) e.g. penicillamine and captopril - Non-thiol drugs e.g. rifampicin, NSAIDs, ACE-inhibitora, interferons - Physical agents e.g. heat, ionising rays - Viruses (herpesviridae) - Pesticides - Diet (garlic) - Stress

Question 60

What are the clinical features of pemhigus vulgaris?

Answer 60

- Onset usually involved oral cavity, lesions run chronic course. -Exclusive gingival lesions may appear at onset as isolated blisters and/or erosions of free gingivae -Red erosions/ulcerations mainly BM, palate and lips. -Minor insult to any uninvolved oral tissue may cause blister (Nikolsky's sign) -Lesions heal without scarring - Advanced = severe desquamative gingivitis - Ulcerations may affect other mucous membranes e.g. conjunctiva, nasal mucosa, pharynx, larync, skin, genitalia - Cutaneous lesions commonly affect skin of head and neck first, bleed easily and crust

Question 61

What is paraneoplastic pemphigus? What is it associated with?

Answer 61

Associated with neolplasia mainly B-cell lymohoproliferative disorders: - Non Hodgkin lymphoma - Chronic lymphocytic leukaemia - Castlemans disease - Thyoma - Sarcomas - Waldenstrom macroglobulinemia

Question 62

What are the clinical features of paraneoplastic pemphigus?

Answer 62

- Erosions of oral cavity more severe than in PV - Skin lesions - blistering and erosive, can have lichenoid features, can be targetoid, polymorphic, histology may show acantholosis - Involvement of bronchopulmonary tree - Final diagnosis based on circulating autoantibodies

Question 63

Which types of pemphigus have predominant skin involvement and predominant oral involvement?

Answer 63

Skin: - Pemphigus foliaceus Oral: - Pemphigus vulgaris - Paraneoplastic pemphigus

Question 64

What types of pemphigoid have predominant skin involvement and predominant oral involvement?

Answer 64

Skin: - Bullous pemphigoid - Herpes gestationis Oral: - Mucous membrane pemphigoid

Question 65

What is mucous membrane pemphigoid?

Answer 65

A group of autoimmune crhonic inflammatory sub-epithelial blistering diseases affecting mucous membranes characterised by linear depositon of IgG, IgA or C3 along epithelial BMZ

Question 66

What is the epidemiology of mucous membrane pemphigoid?

Answer 66

Av age 52-61years F>M Very rare in childhood

Question 67

What is the clinical hallmark of mucous membrane pemphigoid? What places have the highest frequency of involvement?

Answer 67

Scarring Oral - ocular - nasal - anogenital - skin - laryngeal - esophageal

Question 68

What are the clinical features of mucous membrane pemphigoid?

Answer 68

- Most pt have oral lesions, chronic course, gingiva, BM, palate, alveolar ridge, tongue as erythematous patches, blisters erosions - Nikolskys sign may be positive - Ocular involvement, sight theratening, begins as non-specific conjunctivitis, shortening of fornices then symblepharon (adhesion of eyelid to eyeball), entropion (inversion of eyelid), ankyloblepharon (fusion of eyelid), blindness - Skin can be eczematous like or recurrent blisters

Question 69

What are the differential diagnoses for bullous diseases?

Answer 69

Erythema multiforme Ulcerative lichen planus RAS Lupus erythematosus Theraml/chemical burns I/O herpes simplex Orofactial granulomatosis SCC Plasmacell stomatitis

Question 70

What is erythema multiforme?

Answer 70

Uncommon acute usually self-limiting inflammatory disorder affecting skin and mucous membranes

Question 71

What is the classification of erythema multiforme? a) Oral EM b) EM minor c) EM makor/steven johnson syndrome d) Toxic epidermal necrolysis

Answer 71

a) 1-3 weeks, oral erosions/ulcers, no skin, usually lip with crust b) 1-4 weeks, typical target lesions, at one site usually mouth, mild-sever erythema erosions and ulcers. >10% body SA c) 1-6 weeks. At least 2 mucosal sites. >30% body involved, target lesions, oral lesions widespread and severe d) 1-3 weeks - poorly defined erythematous macules and flat targets. Severe mucosal lesions, >30% body SA involved, systemic symptoms poor prognosis

Question 72

What is the epidemiology of erythema multiforme?

Answer 72

Adolescent or young males mainly affected. Must have HLA gene testing before giving drugs

Question 73

What are the clinical features of erythema multiforme?

Answer 73

- Oral involvement - early = erythematous spots which progress to blisters that break and for ulcers/erosions - Swollen cracked lips with erisons - Classic target lesion on skin, extremities usuall involved with symmetric distribution, unusual on face neck and trunk - In TEN skin lesions have irregular edges and lack oedematous ring

Question 74

How is erythema multiforme diagnosed?

Answer 74

- Detailed MH ( role of drugs, herpes) - Biopsy and DIF and IIF helpful to exclude other diseases. - Serology - HLA testing - Histologically can mimic pemphigoid/pemphigus

Question 75

What is the treatment for erythema multiforme?

Answer 75

-Withdraw cause -Oral EM = topical corticosteroids - Short course coricosteroids e.g. 50mg prednisolone x3 days then 25 mg next 3 days - If HSV associated the 6 moth trial antiviral therapy with acyclovir - If resistant to prednisolone reconsider diagnosis or add azathioprine, mycophenolate mofetil or dapsone

Question 76

What is Nikolsky's sign in bullous disease?

Answer 76

Blow air and detachment of gingivae

Question 77

What tissue test should be done with a patient that has suspected bullous disease? What should then be carried out on these?

Answer 77

Biopsy, DIF (differentiates PV from MMP Serum test, IIF - diagnoses PV standard, split skin to diagnose MMP ELISA Immunoprecipitation

Question 78

What is orofacial granulomatosis?

Answer 78

Chronic granulomatous inflammation predominant cell type is activated macrophage in oral and maxillofacial region

Question 79

What are the non-infectious types of orofacial granulomatosis?

Answer 79

Idiopathic OFG Crohns disease Melkerson-Rosenthal syndrome Cheilitis granulomatosa Sarcoidosis Wegeners granulomatosis Foreign body reaction

Question 80

What are the infectious types of orofacial granulomatosis?

Answer 80

Tuberculosis Tertiary syphilis Mycotic granulomatous infection Parasites

Question 81

What is crohns disease? What is its epidemiology?

Answer 81

Bowel inflammatory condition primarily affecting distal portion of small bowel and proximal colon. F>M, 20-40 years, 60-80 years

Question 82

What is the aetiology of crohns disease?

Answer 82

Genetic susceptibility Environmental/microbial factors e.g. smoking, diat, mycobacterium avium paratuberculosis, drugs e.g. oral contraceptive, NSAIDs Immune response - CD4 TH1 reaction induced

Question 83

What are the clinical features of Crohns disease?

Answer 83

GI signs - cramping diarrhoea pain fever weight loss anaemia Oral lesions - non specific = angular cheilitis, apthous like oral ulcers, chronic stomatitis. Specific = diffuse or nodular swelling, cobblestone mucosa, linear ulcers, mucosal tags, erythema/swelling on gingivae Peripheral atheritis Skin - erythema nodosum, pyoderma gangrenosum Eyes - conjunctivitis

Question 84

How is crohns disease diagnosed?

Answer 84

1) History 2) Physical exam 3) Elevated EST and C reactive protein 4) Radiographic MRI and endoscopic findings 5) Antibodies anti-saccharomyces cerevisiae serological markers 6) Biopsy

Question 85

What is Melkersson-Rosenthal Syndrome?

Answer 85

Clinical traid of lesions: 1) Persistent swelling of oro-facial tissues 2) Fissured tongue 3) Facial paralysis 2/3 signs accepted for Dx

Question 86

What is cheilitis granulomatosa?

Answer 86

Limited form of MR more common than complete triad. Generally painless enlargement of one or both lips, mainly young adults DD angioedema

Question 87

What is idiopathic orofacial granulomatous?

Answer 87

Cases that are restricted to oral cavity without any identifiable know granulomatous disease, usully involving lips. May progress to Crohns disease

Question 88

What is the aetiology of Melkersson-rosenthal and idiopathic OFG?

Answer 88

Most cases idiopathic Hypersensitivity to food substances Hypersensitivity to dental materios Infectious ages: mycobacteria, spirochetes, borrelia burgdorferi

Question 89

How is the diagnosis of Melkersson-rosenthal and idiopathic OFG made?

Answer 89

Biopsy - in early stages only oedema, indistinguishable from sarcoidosis or crohns. Stains for mycobacteria and fungal organisms recommended

Question 90

What is the management of Melkersson-rosenthal and idiopathic OFG?

Answer 90

Elimination diet Topical corticosteroid (little effect) Cheiloplastly (recurrence)

Question 91

What is Wegener's Granulomatosis?

Answer 91

Uncommon multi-system disorder, pathological triad: 1) Necrotising granulomatous lesions in upper or lower respiratory tract 2) Systemic vasculitis involving small arteries and vein 3) necrotising glomerulonephritis If untreated it is fatal

Question 92

What are the signs and symptoms of Wegener's Granulomatosis?

Answer 92

Pulmonary symptoms e.g. cough Hearing loss Renal involvement Oral lesions but rarely presenting feature - hyperplastic granular gingivitis (strawberry gingivitis) Tooth mobility, poor healing after extraction, cranial nerve palsy, parotid swelling

Question 93

How is Wegener's granulomatosis diagnosed?

Answer 93

2 of the following: 1) Oral ulcers or nasal discharge 2) Presence of nodules or cavities on a chest xray 3) Nephritic urinary sediment 4) Granulomatous inflammation on biopsy Serological markers and biopsy

Question 94

What is sarcoidosis?

Answer 94

Multisystem disorder, young and middle aged adults, more likely women

Question 95

What is the aetiology of sarcoidosis?

Answer 95

-Infectious agents: mycobacterium tuberculosis main culprit. Fungal and viral agents -Organic particles: wood burning particles -Inorganic agents: insecticides - Immune response

Question 96

What are the clinical features of sarcoidosis?

Answer 96

Acute forms: - Lofgrens syndrome = fever, erythema nodosim, arthralgia - Heerfordt-Waldenstrom syndrome =fever, parotid gland enlargement, anterior uveitis, facial nerve palsy Asymptomatic forms: - Lung invovement Eyes - acute uveitis, keratoconjuctivitis, sicca, retinal vascultitis Skin - erythema nodosum, lupus pernio, maculopapular eruption, subcutaneous nodules Oro-facial very rare - ST swelling, major salivary gland enlargement Hypercalcaemia Liver Neurosarcoidosis

Question 97

How is sarcoidosis diagnosed?

Answer 97

Diagnosis without biopsy with Lofgrens Syndrome Biopsy from involved organ e.g. skin, LN, SG, oral mucosa

Question 98

What can a foreign body reaction be caused by and what can they cause to the body?

Answer 98

Food particles of plant origin Dental materials - abrasives, polishing and dental restorative material Silicone mainly in liquid form Painful and erythematous lesions of gingiva and orofacial swelling. Evidence of granulomatous inflammation and presence of floreing materials

Question 99

If a biopsy is taken and there is a granulomatous reaction with ziehl-neelson negative, what would the follwoing suggest? a) Gastro-intestinal involvement b) history of foreign material c) No systemic symptoms or facial palsy d) granuloma and respiratory symptoms e) necrosis and vasculitis, respiratory symptoms and hearing loss

Answer 99

a) Crohns b) Foreign body granuloma c) Cheilitis granulomatosa/MRS/isiopathic OFG d) Sarcoidosis e) Wegener

Question 100

What could be the causes of localised pigmentation of the oral mucous membrane?

Answer 100

- Amalgam tattoo - Graphite tattoo - Freckles/ephelides - Melanotic macules - Naevi - Melanoma

Question 101

What could be the causes of diffuse pigmentation of the oral mucous membrane?

Answer 101

- Racial pigmentation - Somkers melanosis - Drug induced pigmentation - Peutz-Jeghers Syndrome - Laugier-Hunziker syndrome - Addison disease

Question 102

What is an amalgam tattoo? How do they occur and what are the clinical features?

Answer 102

Introduced during condensation of amalgam fillings, fragments fall away during tooth XTN and fall into open extraction sockets, leaching of retrofilled endo tooth Pigment greyish/blue, located on gingivae, alveolar mucosa and buccal mucosa

Question 103

What is a graphite tattoo?

Answer 103

Black or grey, usually on hard palate from pencil lead.

Question 104

What are melanotic macules?

Answer 104

Focal macular pigmentations on the vermillion border of lips, palate, gingivae and buccal mucosa, dont darken after sun exposure, usually brown, F>M. Commonly solitary lesion, no malignant potential. Histologically shows increased melanin pigmentation in basal cell layer.

Question 105

What are naevi? Explain their development

Answer 105

Proliferation of melanocytes a) Junctional naevi - originate as basal layer melanocytes that proliferate in lower strata of epithelium along the junction with connective tissue b) Compound naevi - later the melanocytes drop off into the connective tissue to form islands c) Intradermal or intramucosal naevi - eventual fate is to leave the surface epithelium entirely so all the clusters of naevus cells reside in the dermis or submucosa

Question 106

What are the clinical features of naevi?

Answer 106

- Primarily on hard palate, BM and vermillion border - Varying size and shades (can be blue) - Around 2/3 are raised - Malignant potential not determined, excision advised

Question 107

What is a melanoma? What is its epidemiology?

Answer 107

Agressive malignant tumour of melanocytes Rare, 1% of all melanomas arise in oral mucosa. 20-80 years M>F Prognosis poor

Question 108

What are the clinical features of a melanoma?

Answer 108

- 75% cases on hard palate or maxillary gingivae - Asymptomatic pigmented patches or plaques with assymmetrical irreguar borders - Superficial melanoma = aradial growth phase - Invasive melanoma = vertical growth phase - May represent metastatic rather than primary lesions - Can be rapidly enlarging nodules resulting in ulceration, bleeding, pain and loosening of teeth

Question 109

What are the clinical features of racial pigmentation?

Answer 109

Pigmented macules of various configurations , usually diffuse and bilaterally located Light to dark brown Gingivae most common then BM, lips, palate, fungiform papillae

Question 110

What is smokers melanosis?

Answer 110

Patchy brown macular pigmentation present BM in heavy smokers Macules 0.5-1cm, multiple and bilateral Basilar melanosis without melanocyte proliferation

Question 111

What is peutz-jeghers syndrome? Why is it important to diagnose?

Answer 111

Mucotaneous hyperpigmentation (pigmented macules, confluent, varying size and shades of brown, lips and BM) and GI hamartomatous polyposisis - inherited autosomal dominant Any adult pts with longstanding pigmented oral macules require gastroenterology investigation to look for polyps - these can undergo malignant transformation and metastasise to breast testicles and ovaries

Question 112

What is laugier-hunziker syndrome?

Question 113

What is Addisons disease?

Answer 113

Primary adrenocortical insufficiency resulting from deficit of cortisol and aldsterone production by the adrenal glands.

Question 114

What are the clinical manifestations of addisons disease?

Answer 114

- Malaise - Postural hypertension - Nausea - Anorexia - Pigmentation - darkening of skin and mucous membranes due to increased circulating adrenocorticotrophic hormone (ACTH)

Question 115

What are the likely drugs that cause drug induced pigmentation if the colour is a) Greyish-blue b) Brown or if the patient has c) HIV

Answer 115

a) Antimalarials, amiodarone, clofazimine b) ACTH, chemotherapeutic agents, oral contraceptives, phenothiazine c) Azidothymide and ketoconazole

Question 116

What does the drug imatinib mesylate that is used in the management of onco-haematological conditions such as myeloid leukaemia do to the oral cavity?

Answer 116

Pigmentation (usually blue)

Question 116

What does the drug imatinib mesylate that is used in the management of onco-haematological conditions such as myeloid leukaemia do to the oral cavity?

Answer 116

Pigmentation (usually blue)

Question 117

What is the differential diagnosis for pemphigus vulgaris oral lesions/ what else do they look like?

Answer 117

MMP Erythema multiforme Erosive LP Herpetic stomatitis Self injury/thermal burn