EPONYMOUS AND OTHER CONDITIONS

Question 1

Addison disease (hypoadrenocorticism)

Answer 1

Adrenocortical destruction, reduced cortisol, and subsequent increased release of pituitary adrenocorticotrophic hormone (ACTH).
A rare disease of young or middle-aged females, the usual cause is autoimmune hypoadrenalism, rarely, tuberculosis, histoplasmosis (sometimes HIV/AIDS) or carcinomatosis.
Weight loss, weakness, and hypotension, with brown hyperpigmentation, especially in sites usually pigmented (areolae and genitals) or traumatised, in flexures, on the gingivae, and at the occlusal line are seen.
Diagnosis is from low blood pressure, low plasma electrolyte and cortisol levels and impaired response to ACTH stimulation (synacthen test).
Management is fludrocortisone plus corticosteroids.

Question 2

Battle sign

Answer 2

Bruising over the mastoid - sign of basilar skull fracture

Question 3

Behcet syndrome

Answer 3

‘Adamantiades syndrome’
A systemic inflammatory vasculitis of unknown etiology, characterized by relapsing episode of oral aphthous ulcers, genital ulcers, cutaneous and ocular lesions and other manifestations, including vascular, neurological and gastrointestinal involvements

Question 4

Bell palsy

Answer 4

The common lower motor neurone facial palsy.

Question 5

Bell sign

Answer 5

Seen in lower motor neurone facial palsy, when the eye rolls upward on attempted closure.

Question 6

Bohn nodules

Answer 6

Keratin-filled cysts derived from palatal salivary gland structures scattered over the palate, especially at the junction of the hard and soft palate.

Question 7

Burkitt lymphoma

Answer 7

Caused by Epstein-Barr virus, most common in children in sub-Saharan African endemic malaria areas, especially Uganda and Kenya, characterised by lymphomatous deposits in many tissues, especially the jaws (in 50% of patients). Responds well to chemotherapy.

Question 8

Cannon disease

Answer 8

Congenital white sponge naevus

Question 9

Carabelli cusp

Answer 9

Congenital additional palatal cusp on upper molars

Question 10

Christmas disease

Answer 10

Blood clotting factor IX defect

Question 11

Coxsackie virus

Answer 11

Coxsackie viruses are many, and can cause herpangina, hand, foot and mouth disease, and other illnesses.

Question 12

Crohn disease

Answer 12

A chronic inflammatory idiopathic granulomatous disorder that may be caused by Mycobacterium avium subspecies paratuberculosis. Mutations in the CARD15 gene (NOD2 gene) are also implicated. About 20% have a blood relative with some form of inflammatory bowel disease.

Question 13

Cushing syndrome

Answer 13

Moon face with buffalo hump, hirsutism (excess hair) and hypertension due to an ACTH-producing pituitary adenoma.

Question 14

Down syndrome

Answer 14

Trisomy 21. The commonest recognisable congenital chromosomal anomaly. Patients are of short stature with characteristic brachycephaly, midface retrusion and upward sloping palpebral fissures (mongoloid slant - slanting eyes). Learning disability and dental anomalies and periodontitis are common.

Question 15

Ehlers-Danlos syndrome

Answer 15

A group of congenital collagen disorders (autosomal dominant, autosomal recessive, or X-linked), with altered mechanical properties of skin, joints, ligaments and blood vessels. Phenotypes vary depending upon which collagen type is affected.
EDS is characterised by hyperflexible joints, hyperextensible skin, bleeding and bruising, and mitral incompetence. Patients can bend the thumb right back and may be able to touch the top of their nose with their tongue.
Recurrent dislocation of the temporomandibular joint may be seen. Dental anomalies include deep-fissured premolars and molars, dentinal abnormalities, such as shortened deformed roots, and multiple large pulp stones.
Ten types were described: in types IV, VIII and IX there is severe early onset periodontal disease with loss of permanent teeth. Type III genotypes show resistance to local analgesia.

Question 16

Epstein-barr virus

Answer 16

A herpesvirus implicated in infectious mononucleosis (glandular fever/mono), hairy leukoplakia, nasopharyngeal carcinoma and some lymphomas.

Question 17

Epstein pearls

Answer 17

Cystic keratin-filled nodules derived from entrapped epithelial remnants along the line of fusion along the midpalatine raphe.

Question 18

Frey syndrome

Answer 18

Gustatory sweating and flushing after trauma to skin overlying a salivary gland due to crossover of sympathetic and parasympathetic innervation to the gland and skin.

Question 19

Gardner syndrome

Answer 19

Familial adenomatous polyposis (FAP), formerly termed familial polyposis coli (FPC).
An autosomal dominant condition caused by mutation in APC tumour suppressor gene on chromosome 5.
Intestinal polyps have a 100% risk of undergoing malignant transformation, so early identification of disease is critical.
Gardner described the occurrence of FAP as extracolonic manifestations of desmoids, osteomas and epidermoid cysts. Underupted and supernumerary teeth may be present. Multifocal pigmented lesions of the fundus of the eye are seen in 80%.

Question 20

Garre osteomyelitis

Answer 20

This is proliferative periostitis

Question 21

Goltz syndrome

Answer 21

Focal dermal hypoplasia
An x-linked disorder with multiple mesenchymal defects, skin lesions, and oral warts and dental defects.

Question 22

Gorlin-Goltz syndrome

Answer 22

(Gorlin syndrome; multiple basal cell naevi syndrome; naevoid basal cell carcinoma syndrome (NBCCS).
An autosomal-dominant trait related to chromosome 9q22.3-q31 and associated with patch gene mutations and deletions.
The syndrome consists of multiple basal cell carcinomas (BCC), keratocystic odontogenic tumours (KCOTs), vertebral and rib anomalies and temporoparietal bossing with broad nasal root, calcification of the falx cerebri and abnormal sella turcica.
Jaw cysts are indistinguishable from other KCOTs and are treated similarly.
Diagnosis is suggested by major criteria - positive family history; more than one BCC, KCOTS (first sign in 75%); palmar or plantar pits; or calcified falx cerebri. Minor criteria include congenital skeletal anomalies: bifid, fused, splayed or missing ribs; or bifid, wedged of fused vertebrae; occipitofrontal circumference over 97th percentile, with frontal bossing; cardiac or ovarian fibromas; medulloblastoma; lymphomesenteric cysts; and congenital malformations, such as cleft lip and/or palate, polydactlyl, congenita ocular anomaly (cataract, microphthalmos, coloboma)

Question 23

Graves disease

Answer 23

Hyperthyroidism with ophthalmopathy and exophthalmos

Question 24

Guillain-Barre syndrome

Answer 24

Acute infective polyneuritis; facial palsy may be seen.

Question 25

Heck disease (focal epithelial hyperplasia)

Answer 25

Papillomas caused by human papilloma viruses 13 or 32, seen especially in ethnic groups such as American Indians and Inuits.

Question 26

Hodgkin disease

Answer 26

Lymphoma that affects particularly males in middle age. Progressive lymphoid tissue involvement, often begins in the neck with enlarged, discrete and rubbery lymph nodes. Pain, fever, night sweats, weight loss, malaise, bone pain and pruritus (itching) are common. Treatment by chemotherapy and radiotherapy is remarkably successful.

Question 27

Horner syndrome

Answer 27

This is caused by interruption of sympathetic nerve fibres peripherally as a result, for example, of trauma to the neck or lung cancer infiltrating superior cervical sympathetic ganglion, and comprises, usually, unilateral: > miosis (pupil constriction) > ptosis (drooping of upper eyelid) > loss of sweating of the ipsilateral face > apparent enophthalmos (retruded eyeball).

Question 28

Hutchinson teeth

Answer 28

Screwdriver-shaped incisor teeth in congenital syphilis

Question 29

Kaposi sarcoma (KS)

Answer 29

A malignant neoplasm of endothelial cells caused by human herpesvirus 8, especially in HIV/AIDS or immunosuppressed patients.

Question 30

Kawasaki disease (mucocutaneous lymph node syndrome)

Answer 30

Fever, cheilitis, lymphadenopathy, desquamation of hands and feet, and cardiac lesions in periodic epidemics with geographic spread, suggest and infectious aetiology. Management includes gamma globulin and aspirin and long-term anticoagulation.

Question 31

Lemierre syndrome

Answer 31

Throat infection is followed by identified potentially lethal anaerobic septicaemia - postanginal septicaemia with metastatic abscesses - most commonly in the lung, due to anaerobe Fusobacterium necrophorum.

Question 32

Ludwig angina

Answer 32

Infection of sublingual and submandibular fascial spaces

Question 33

Marfan syndrome

Answer 33

An autosomal-dominant condition linked to chromosome 15 in FBN1 gene, which codes for fibrillin-1, essential for the formation of elastic fibres. Reduced levels of fibrillin-1 allow transforming growth factor (TGFB) to damage heart and lungs. Prevalent among basketball and volleyball players, patients are tall, thin and with arachnodactyly (long, thing spider-like hands). There may be spontaneous pneumothorax, lens dislocation, aortic regurgitation or dissecting aneurysms, mitral valve prolapse, and palate is occasionally cleft or with bifid uvula. Joint laxity is common and TMJ may dislocate. Multiple dental cysts are less common oral complications. Can be confused with Loeys-Dietz syndrome.

Question 34

Melkersson-Rosenthal syndrome

Answer 34

The rare association of facial swelling (usually granulomatous cheilitis), with facial palsy and fissured tongue.

Question 35

Moon molars

Answer 35

Hypoplastic molar from congenital syphilis

Question 36

Nikolsky sign

Answer 36

A term meaning blistering, or the extension of a blister, on gentle pressure (seen mainly in pemphigus and pemphigoid)

Question 37

Non-Hodgkin lymphoma (NHL)

Answer 37

More common than Hodgkin disease and generally with a poorer prognosis, and a predilection for the gastrointestinal tract and central nervous system. Enlargement of cervical lymph nodes is often the first sign, but NHL may occur in the gingivae or faucial region; a recognised complication of HIV/AIDS and may be Epstein-Barr virus related.

Question 38

Osler-Rendu-Weber disease (hereditary haemorrhagic telangiectasia; HHT)

Answer 38

An autosomal-dominant disorder where telangiectases are present orally, periorally and in the nose, gastrointestinal tract and occasionally on the palms. Telangietases may bleed, and cryosurgery or laser treatment may be needed.

Question 39

Paget disease of bone

Answer 39

A bone disorder characterised by the total disorganisation of bone remodelling

Question 40

Papillon-Lefevre syndrome

Answer 40

A congenital condition mapped to chromosome 11q with a defect in the polymorphonuclear leukocyte lysosomal enzyme cathepsin c, causing palmoplantar hyperkeratosis and juvenile periodontitis, which affects both dentitions, and immunodeficiency. Acitretin may correct the defective CD3-induced T lymphocyte activation.

Question 41

Peutz-Jeghers syndrome

Answer 41

An autosomal-dominant condition due to a chromosome 19p STK11/LKB1 often associated with gene mutation mutations in LKB1. It consists of circumoral melanosis and intestinal polyposis. Oral brown or black macules appear in infancy and affect especially the lips and buccal mucosa and may be seen on extremities and abdomen. The intestinal polyposis may cause intussusception or other types of obstruction. Almost 50% of patients develop and die from some type of cancer by age 57 years - often extra-intestinal cancers.

Question 42

Ramsay-Hunt syndrome

Answer 42

A lower motor neurone facial palsy due to herpes zoster of the geniculate ganglion of the seventh nerve, facial nerve. Presents with vesicles in the ipsilateral pharynx, external auditory canal and on the face.

Question 43

Riga-Fede disease

Answer 43

TUGSE (traumatic ulcerative granuloma with stromal eosinophilia)

Question 44

Sjogren syndrome

Answer 44

Chronic autoimmune disease that causes the body's immune system to attack the glands that produce tears and saliva. This leads to a reduction in the amount of tears and saliva, resulting in dry mouth and eyes.

Question 45

Stafne bone cavity

Answer 45

An ectopic inclusion of salivary tissue in the mandible, often from the submandibular gland. Radiography shows a cystic radiolucency. No treatment is indicated, but if a diagnosis cannot be confirmed by sialography, or if serial radiographs show an increase in cavity size, exploratory surgery is indicated.

Question 46

Stevens-Johnson sydrome

Answer 46

Stevens-Johnson syndrome is a rare condition arising from ‘over-reaction’ of the immune system to a trigger such as a mild infection or a medicine, leading to blistering and peeling of the skin and surfaces of the eyes, mouth and throat. The most common triggers for Stevens-Johnson syndrome in children are infections, usually viral. Common infectious triggers include herpes, mumps, flu and the Epstein Barr virus. In adults, reactions to medicines, such as pain killers and antibiotics, are more common. In many cases, the trigger cannot be identified. The main feature of Stevens-Johnson syndrome is a skin rash. The affected patches may look like a ‘target’ with a purple or dark area of skin surrounded by a lighter area. Initially, the skin may be discoloured but then blisters develop which burst and leave sore areas. The rash is not usually itchy. Crops of discoloured areas may develop over a period of several weeks. The mucous membranes – moist areas of tissue such as the eye, inside of the mouth and throat – are also affected with blisters and ulceration. Ulceration of the mucous membranes has the potential to cause the most serious problems such as dehydration because swallowing is so painful.

Question 47

Sturge-Weber syndrome (encephalotrigeminal angiomatosis)

Answer 47

A congenital hamartomatous angioma of the upper face (naevus flammeus), oral mucosa and underlying bone (with hemihypertrophy of bone and accelerated eruption of associated teeth), exetnding intracranially to cause convulsions, and contralateral hemiplegia and intracerebral calcifications, and sometimes learning disability.

Question 48

Turner tooth

Answer 48

Hypoplastic tooth due to damage to the developing tooth germ.

Question 49

Von Willebrand disease

Answer 49

Common bleeding disorder caused by defective blood clotting factors VIII and platelet dysfunction

Question 50

Warthin tumour

Answer 50

Benign salivary gland neoplasm- adenolymphoma or papillary cystadenoma lymphomatosum.

Question 51

Wegner granulomatosis

Answer 51

Idiopathic disseminated malignant granulomatosis. A rare, idiopathic disorder of necrotizing granulomatosis. Initially affecting the respiratory tract, arteritis affects small vessels in lungs, kidneys, eyes, skin, muscles, joints and mouth and is potentially lethal. A painless progressive swelling of the gingivae in a previously healthy mouth, particularly associated with swollen inflamed papillae with a fairly characteristic 'strawberry-like' appearance, should arouse suspicion. Diagnosis is supported by biopsy, imaging and anti-neutrophic cytoplasmic antigens (ANCA), anaemia, elevated ESR, CRP or PV and white blood cell and platelet counts. Management is antimicrobials, cytotoxic agents or radiotherapy.

Question 52

Acanthosis nigricans

Answer 52

A rare paraneoplastic conditions where papillomatous oral lesions are seen often in patients with internal malignancy.

Question 53

Acatalasia

Answer 53

Autosomal recessive defect in the enzyme catalase, which normally removes reactive oxygen species, such as hydrogen peroxide, from the tissues. Severe periodontal destruction and oral ulceration can result.

Question 54

Achondroplasia (chondrodystrophia fetalis)

Answer 54

An autosomal dominant condition in which endochondral ossification is reduced. The most common form of short-limbed dwarfism - patients have disproportionately short limbs, bowed legs, kyphosis, prominent buttocks and abdomen, and trident hands with short fingers. The skull is relatively large, having prominent frontal, occipital and parietal bones. The middle third of the face can be hypoplastic with nasal bridge depressed and a class III type malocclusion. The foramen magnum is often narrow and spinal cord compression can occur. Joints can be of limited mobility and the pelvic inlet narrow.

Question 55

Actinic prurigo (AP)

Answer 55

A chronic, pruritic skin condition caused by an abnormal reaction to sunlight.

Question 56

Actinomycosis

Answer 56

A rare infection with Actinomyces israelii, below the mandibular angle (not lymph nodes) that may follow jaw fracture or tooth extraction. Prolonged therapy, usually with penicillin, is indicated.

Question 57

Acute necrotizing ulcerative gingivitis

Answer 57

A non-contageous anaerobic gingival infection associated with overwhelming proliferation of Treponema vincentii (spirochaete) and fusiform bacteria. Predisposing factors include smoking, viral respiratory infections and immune defects, such as HIV/AIDS. Uncommon, except in resource-poor groups, this typically affects adolescents and young adults, especially in institutions, armed forces, etc., or people with HIV/AIDS. Characteristic features include severe gingival soreness, profuse bleeding, halitosis and a bad taste. Inderdental papillae are ulcerated with necrotic slough. Malaise, fever and/or cervical lymph node enlargement (unlike herpetic stomatitis) are rare. Cancrum oris (noma) is a very rare complication, usually in debilitated children. Diagnosis is usually clinical. Smear for fusospirochaetal bacteria and leukocytes: blood picture occasionally. Differentiate from acute leukaemia or herpetic stomatitis. Manage by oral debridement, metronidazole (penicillin if pregnant) and oral hygiene.

Question 58

Amelogenesis imperfecta

Answer 58

A rare genetic defect of enamel formation due to mutations in AMLEX, ENAM and MMP20 genes, with a wide variety of patterns. All teeth are affected, as are other family members. Diagnosis is from clinical features. Fluorosis, tetracycline staining, dentinogenesis imperfecta and oculodentodigital dysplasia may need to be differentiated. Management requires restorative dental care. Type 1 = hypoplastic. > defect: matrix > enamel is thin, hard, pitted or grooved. > radiographically: normal enamel density > inheritance: AD, AR or x Type 2 = hypomaturation > defect: maturation > enamel is normal thickness, softer and liable to attrition. Snow capped > inheritance: AD, AR or x Type 3 = hypocalcified > defect: calcification > enamel has normal thickness, softer and liable to attrition. Discoloured white to brownish-yellow and darkening with age. radiographically: enamel similar to dentine > inheritance: AD or AR Type 4 = hypomaturation with taurodontism > defect: mixed - between types 1 and 2 > variable enamel appearance > inheritance: AD

Question 59

Amyloidosis

Answer 59

The deposition in tissues of amyloid - an eosinophilic hyaline material, with a fibrillar structure on ultramicroscopy. Amyloid can be identified via Congo red stain under fluorescent or polarised light, thioflavine T stain under fluorescent light, or immunoreactivity with antibodies for immunoglobulin light chains. Primary (including myeloma-associated) amyloidosis: associated with deposits of immunoglobulin light chains. Manifestations include macroglossia and oral petechiae or blood-filled bullae (secondary purpura). Secondary amyloidosis: seen mainly in rheumatoid arthritis and ulcerative colitis, rarely affects the mouth, and is associated with deposits of AA proteins. Diagnose from biopsy, blood picture: raised ESR, CRP or PV and marrow biopsy; serum proteins and electrophoresis; urinalysis; skeletal survey for myeloma. Manage by chemotherapy (melphalan, corticosteroids or fluoxymesterone).

Question 60

Angina bullosa haemorrhagica (localised oral purpura)

Answer 60

Blood blisters in the mouth or pharynx, mainly on soft palate, seen in presence of any immunological or platelet-associated cause. Seen mainly in older people, the aetiology is unclear, though there are occasional associations with use of steroid inhalers. There is rapid onset, with breakdown of the blister in a day or two to an ulcer. Diagnose from clinical features, although it may be necessary to confirm haemostasis is normal; and rarely to biopsy to exclude pemphigoid. Manage by reassurance. Topical analgesics may provide symptomatic relief.

Question 61

Ankyloglossia (tongue-tie)

Answer 61

An uncommon genetic condition which results in the lingual fraenum anchoring the tongue tip, restricting tongue protrusion and lateral movements. Oral cleansing (but not speech) is impaired. Differentiate from tethering by scarring. Management is surgery (fraenectomy) if severe.

Question 62

Branchial cyst

Answer 62

A lymphoepithelial cyst that may arise from enclavement of salivary tissue in a lymph node, or from the cervical sinus. It usually becomes apparent in the third decade. It requires excision.

Question 63

Bullous pemphigoid

Answer 63

An autoimmune disease usually with widespread crops of tense, fluid-filled blisters on the skin. The diagnosis is confirmed by biopsy. Bullous pemphigoid is treated with prednisolone (enteric-coated).

Question 64

Caries

Answer 64

Dental caries can occur on any tooth surface exposed to the oral environment, but not surfaces retained within bone. There are four factors in the aetiology: > a tooth surface (enamel or dentine) > cariogenic (or potentially caries-causing) bacteria in dental plaque > fermentable carbohydrates > time The specific bacterial species believed to cause caries include Streptococcus mutans and Lactobacilli. Particularly for root caries, the most closely associated bacteria are Lactobacillus acidophilus, Actinomyces viscosus, Nocardia spp., and Streptococcus mutans. Sugars (sucrose, glucose, fructose) are metabolised to acetic, lactic, formic and succinic acids. Different individuals are susceptible to different degrees depending on tooth shape, oral hygiene, and the flow and buffering capacity of their saliva.

Question 65

Cheek biting (morsicatio buccarum)

Answer 65

common and seen in adults mainly, especially anxious patients, and those with other psychologically related disorders, e.g. temporomandibular pain-dysfunction syndrome, it is also rarely caused by self-mutilation, seen in psychiatric disorders, learning disability, Lesch-Nyhan syndrome and some rare syndromes with insensitivity to pain. Abrasion of the superficial epithelium leaves whitish fragments on reddish background. The lesions are invariable restricted to the lower labial mucosa and/or buccal mucosa near the occlusal line on one or both sides.

Question 66

Chemical burns

Answer 66

Can be caused by various chemicals or drugs; notably aspirin put in the sulcus to try and relieve toothache, or cocaine. A white lesions with sloughing mucosa is seen. Diagnosis is by history and clinical features.

Question 67

Chondroma

Answer 67

Benign cartilage forming tumour. A benign tumour, rare in the jaws. The anterior maxilla, mandibular symphysis and the coronoid and condylar processes are the most common sites. Chondromas are typically found in the older patient and form slow-growing, painless masses. Provided surgical excision of these masses is complete, recurrence is unlikely.

Question 68

Chondrosarcoma

Answer 68

Malignant cartilage forming tumour These tend to affect the older patient and, although isolated cases arise from chondromas, most arise de novo. The behaviour is very unpredictable, but many grow rapidly and produce extensive local destruction. Radical surgery, the only means of eradicating the tumour, can be difficult as the edges may not be apparent clinically or radiographically. Multiple local recurrence is common and the prognosis is worse than for osteosarcoma. Chondrosarcoma is a bone sarcoma, a primary cancer composed of cells derived from transformed cells that produce cartilage. (cartilage is formed by tumour cells) A painless swelling that may cause teeth to loosen and the periodontal ligament space to widen

Question 69

Chronic ulcerative stomatitis

Answer 69

This is similar to erosive lichen planus, with ulcers and erosiions affecting mainly the buccal or lingual mucosae, but sometimes the gingivae. The lesions are clinically normal mucosa have a distinct pattern of particulate stratified squamous epithelium-specific (SES) multinuclear antibody (ANA) on direct immunofluorescent examination of perilesional tissue, and patients can have circulating ANA. Hydroxychloroquine is effective therapy.

Question 70

Cleidocranial dysplasia (dysostosis)

Answer 70

A rare autosomal dominant condition, or mutation. Clinical features include exaggerated transverse diameter of cranium, delayed fontanelle closure, multiple wormian bones, frontal and parietal bossing, depressed nasal bridge, maxillary hypoplasia, underdeveloped paranasal sinuses, high arched palate (? cleft), delayed or failed tooth eruption, multiple supernumerary teeth, dentigerous cysts, aplastic of hypoplastic clavicles (shoulders can be approximated), short stature and other skeletal anomalies. Diagnosis is from the family history and ability to bring together the shoulders; jaw skull and skeletal radiography. Management is to leave unerupted teeth alone unless there are complications. Eruptions of permanent teeth is retarded and dentigerous cysts are frequently found. Supernumerary teeth are common in cleidocranial dysplasia, especially in the anterior mandible. The crowns of the teeth are normal, but the roots can be short, thin and lack acellular cementum. Surgery is often necessary.

Question 71

Coeliac disease

Answer 71

Coeliac disease is a reaction to gluten, ingestion of which activates immune cells in the small intestine, which trigger inflammation and local damage, disruption food absorption. Untreated coeliac patients lose weight, develop deficiency syndromes such as anaemia, and experience symptoms such as diarrhoea. Dental hypoplasia and oral ulceration may result. Diagnosis is confirmed by malabsorption, blood tissue transglutaminase antibodies and villous atrophy on jejunal biopsy. Gluten is found in wheat, barely and rye, which means that many dietary staples, such as bread, many breakfast cereals and food like pizza and pasta can no longer be eaten.

Question 72

Congenital epulis

Answer 72

A rare reactive process seen on the alveolus of a neonate.

Question 73

Crystal deposition diseases (gout)

Answer 73

A term used to encompass diseases that produce crystals in joints (e.g. gout, chondrocalcinosis, secondary gout caused by cytotoxic therapy) including the temporomandibular joint. Gout itself is an uncommon disorder of metabolism in which excessive levels of uric acid in the blood and other body fluids crystallize out in synovial fluid causing joint inflammation. The patient complains of painful, swollen joint and 'pan-meniscal crepitation' is elicited on movement. Acute attacks are often preceded by a 'binge' of dietary or alchol excess or 'starvation', trauma or unusual physical exercise, surgery of systemic illness. In chronic gout, trophi are frequently felt in the cartilage of the pinna of the ear. Diagnosis is clinical, but usually confirmed by a leukocytosis, raised ESR, CRP or PV raised serum urate level and aspiration biopsy for examination under polarising light for crystals or urate. Imaging is not particularly helpful since the appearance resembles osteoarthritis. Treatment must not be restricted just to the local treatment of the TMJ. NSAIDs should be started. Colchicine is effective. Gout is usually treated prophylactically with allopurinol. Dietary advice and weight control are also important.

Question 74

Cyst

Answer 74

A pathological cavity having liquid, semi-liquid or gaseous contents. It is frequently, but not always, lined with epithelium. Cysts of the jaws mostly arise from odontogenic epithelium, and are relatively common lesions. Non-odontogenic developmental cysts are uncommon. The lining of these cysts is either stratified squamous epithelium or pseudostratified ciliated columnar (respiratory) epithelium. Non-odontogenic cysts include the following: - Nasopalatine duct cyst
Nasolabial cyst
Thyroglossal tract cyst
Branchial cyst
Epidermoid cyst
Epidermal inclusion cyst
Tricholemmal cyst
Dermoid cyst
Teratoid cyst
Heterotopic gastrointestinal cyst

Question 75

Nasopalatine or incisive canal cyst

Answer 75

Derived from the vestigial oronasal ducts. They may occur either within the nasopalatine canal, or in the soft tissues of the palate at the opening of the canal, grow slowly, and may discharge into the mouth giving a salty taste. Radiological examination shows a well-defined rounded ovoid or occasionally heart-shaped defect in the anterior maxilla. Nasopalatine cysts must be distinguished from a normal large anterior palatine fossa which may be up to 7mm diameter, and from radicular cysts associated with the maxillary incisors. These cysts should be enucleated and seldom recur.

Question 76

Nasolabial cyst

Answer 76

Soft tissue cysts found within the nasolabial fold. They are lined by respiratory epithelium, and if allowed to grow may distord the upper lip and alar base. Treatment is simple excision.

Question 77

Dentigerous imperfecta

Answer 77

A rare autosomal-dominant disorder in which the dentine is abnormal in structure and hence, translucent, and poorly attached to enamel. All teeth are affected, but primary teeth are more severely affected than permanent teeth. In the permanent dentition the teeth that develop first are generally more severely affected than those that develop later. The teeth are: > translucent > may vary in colour from grey to blue to brown > enamel is poorly adherent to the abnormal underlying dentine and easily chips and wears > crowns are bullous with pronounced cervical constriction and the roots are short > fracture easily (there is progressive obliteration of pulp chambers and root canals with secondary dentine) > periapical radiolucencies are not uncommon. There are three types of dentinogenesis imperfecta: Type I = associated with osteogenesis imperfecta: most severe in deciduous dentition; bone fractures; blue sclera; progressive deafness; caused by mutations in one of several genes. Type II = hereditary opalescent dentine: defect equal in both dentitions; caused by mutations in the DSPP gene. A few families with type II have progressive hearing loss in addition to dental abnormalities. Type III = brandywine type: associated with shell teeth and multiple pulpal exposures; caused by mutations in the DSPP gene. Differentiate mainly from amelogenesis imperfecta, tetracycline staining and dentine dysplasia. Management is by restorative dental care.

Question 78

Dermatitis herpetiformis

Answer 78

An uncommon chronic skin disorder associated often with gluten-sensitive enteropathy, affecting mainly middle-aged males. Symmetrical papulovesicular eruptions on extensor surfaces. Oral lesions of vesicles and/or desquamative gingivitis, similar to pemphigoid typically follow skin lesions. Biopsy of perilesional tissue, with histological and immunostaining examination are essential to diagnosis, showing IgA deposits at the papillae. Jejunal biopsy often indicated. Dapsone is the main therapy - sulfamethoxyridazine and sulfasalazine are alternatives. A gluten-free diet can minimise disease activity and can reduce or avoid the need for drugs.

Question 79

Dermoid cyst

Answer 79

A rare developmental cyst that presents as a doughy painless swelling in the midline floor of mouth and needs to be differentiated from ranula and cystic hygroma. Diagnosis is by aspiration, but there is risk of infection. Management is by surgical removal.

Question 80

Desquamative gingivitis

Answer 80

A fairly common problem in which the gingivae show chronic desquamation and is a term that denotes a particular clinical picture and not a diagnosis in itself. Many of the patients are middle-aged women. Desquamative gingivitis is mainly a manifestation of: - mucocutaneous disorders, usually. Most gingival involvement in the vesiculobullous or skin diseases (dermatoses) is related to lichen planus or pemphigoid, but pemphigus, dermatitis herpetiformis, linear IgA disease, chronic ulcerative stomatitis and other conditions may need to be excluded. Most of these conditions are acquired, but a few are congenital with a strong hereditary predisposition, such as epidermolysis bullosa. - chemical damage, such as reactions to sodium lauryl sulphate in toothpastes. - allergic responses - drugs - psoriasis - pyostomatitis vegetans Some patients make no complaint, but others complain of persistent gingival soreness, worse when eating spices, or acidic foods, such as tomatoes or citrus fruits. Most patients are seen only when vesicles and bullae have broken down to leave desquamation, and the clinical appearance is thus of erythematous gingivae, mainly labially, the erythema and loss of stippling extending apically from the gingival margins to the alveolar mucosae. The desquamation may vary from mild almost insignificant small patches to widespread erythema with a glazed appearance. In addition to a full history and examination, biopsy examination and histopathological and immunological investigations are frequently indicated. Conditions which should be excluded include: > reactions to mouthwashes, chewing gum, medications and dental materials. > candidosis > lupus erythematosus > plasma cell gingivitis > Crohns disease, sarcoidosis and orofacial granulomatosis > leukaemias > factitial (self-induced) lesions The treatment of desquamative gingivitis consists of: - improving the oral hygiene - minimising irritation of the lesions - specific therapies for the underlying disease where available - local or systemic immunosuppressive or dapsone therapy, notably corticosteroids. Corticosteroid creams used overnight in a soft polythene splint may help.

Question 81

Drug addiction (illegal drug use)

Answer 81

Addicts in need of a 'fix' will often falsely complain of severe pain or injury. Disturbed behaviour in a drug addict may be caused by withdrawal symptoms and the patient may need compulsory detention. If an addict is admitted to the ward, contact a licensed psychiatrist for heroin or cocaine to be given for addicts. The most serious problems in the management of addicts include: > overdose > withdrawal effects > behavioural problems (including theft) > violence > hepatitis > HIV infection > other infections > oral lesions typically are those of these conditions or neglect of hygiene

Question 82

Dysarthria (disordered speech)

Answer 82

Deranged speech can be due commonly to drugs (including alcohol), CNS disorders as in learning disabilites, cerebral palsy, parkinsonism, delirium or dementia; loss of voice due to laryngeal disease or paralysis (dysphonia); or defects in articulation becuase of paralysis, rigidity, tissue loss or scarring, or involuntary movements of tongue or palate. The most common oral cause of dysarthria is immobility of the tongue after a lingual block local analgesia injection, trauma to the tongue, scarring, diseases affecting the tongue (such as carcinoma) or foreign bodies (such as oral piercing). Spastic dysarthria is usually caused by cerebrovascular disease affecting the motor cortex such as parkinsonism, or drugs, such as phenothiazines; basal ganglia disease such as choreic dysarthria; cerebellar diseases causes ataxic dysarthria.

Question 83

Dyskinesias

Answer 83

Abnormal movements of the tongue or facial muscles, sometimes with abnormal jaw movements, bruxism or dysphagia. Involuntary tongue protrusion and retraction, and facial grimacing are common dyskinesias. Most dyskinesias resolve within 4 weeks of stopping any causal medication. If not, treat with anti-parkinsonian drugs (e.g. benzhexol), baclofen, benzodiazepines, dopamine depletors, calcium channel blockers, clozapine, buspirone, a-adrenergic agonists, vitamin E or botulinum toxoid.

Question 84

Dysphasias

Answer 84

Disturbance of langue use (or aphasias), and my be caused by brain disease, such as stroke or after head injury, in which there is loss of production and comprehension of speech and language.

Question 85

Epidermolysis bullosa

Answer 85

A rare genetically-determined disorder related to a defect in the epidermolysis bullosa antigen in the epithelial basal lamina, leading to blistering after trauma or scarring, which may cause severe disability, such as limb deformities, microstomia, ankyloglossia and trismus. Enamel hypoplasia may be seen. Diagnosis is from the family history and a biopsy to exclude other blistering diseases. Management includes careful attention to oral hygiene. Trauma should be minimised, and drugs such as phenytoin may help reduce the blistering.

Question 86

Epidermolysis bullosa acquisita

Answer 86

A rare non-inherited chronic mechanobulluous disease characterized by autoantibodies directed against type VII collagen. Clinically bullae are frequently induced after mechanical irritation. The diagnosis should be made on the history, clinical features, histopathological, direct and indirect immunofluorescent examination. Biopsy of perilesional tissue, with histological and immunostaining examination are essential to the diagnosis. Topical corticosteroids effectively control gingival lesions. Systemic corticosteroids alone or in association with immunosuppressive agents and dapsone are suggested treatments.

Question 87

Fibro-osseous lesions

Answer 87

A group of disorders of unknown aetiology, composed of fibrous and ossified tissue. The main disorders include Paget disease of bone, fibrous dysplasia and cherubism and the ossifying fibroma.

Question 88

Fissured lip

Answer 88

Lip fissures may appear especially where there is exposure to adverse environments, or where the lip swells as in Down syndrome or cheilitis granulomatosa.

Question 89

Fissured tongue

Answer 89

An extremely common genetic condition, the dorsum has deep irregular fissures, but is normally papillaed. A fissured tongue may also be seen in Down syndrome or Melkersson-Rosenthal syndrome. There are associations with erythema migrans in particular. The diagnosis is usually clear cut. The lobulated tongue of Sjogren sydrome must be differentiated.

Question 90

Fluorosis

Answer 90

The condition of enamel defects caused by high levels of fluoride. High levels in drinking water are uncommon the the developed world, but are particularly common in parts of the Middle East, India and Africa. Fluorosis affects may teeth: - mildest form: white flecks or spotting or diffuse cloudiness - more severe form: yellow-brown or darker patches - most severe form: yellow-brown or darker patches, sometimes with pitting Diagnosis is from history, clinical appearance and data about fluoride content of drinking water. It is necessary to differentiate from amelogenesis imperfecta and tetracycline staining. Management of severe forms is by the use of veneers or crowns.

Question 91

Furred tongue

Answer 91

Coating of the tongue is quite commonly seen in healthy adults, particularly in edentulous patient, those who are on a soft, non-abrasive diet, those with poor oral hygiene, or those who are fasting. The coating in these instances appears to be of epithelial, food and microbial debris, which collects since it is not mechanically removed. Indeed, the tongue is the main reservoir of some microorganisms, such as candida albicans and some streptococci. The tongue may be coated with off-white debris in many illnesses, particularly febrile diseases, hyposalivation and ill patients - especially those with poor oral hygiene or who are dehydrated. The history is important to exclude a congenital or hereditary cause of white lesion or candidosis. The clinical appearances may strongly suggest the diagnosis, but investigations are often required if the white lesion does not scrape away from the mucosa with a gauze. Biopsy may then be rarely indicated. Treatment is of the underlying cause.

Question 92

Gingival bleeding

Answer 92

This is usually explained by the presence of gingivitis. However, unexplained bleeding can be due to thrombocytopenia (e.g. in leukaemia, HIV disease, idiopathic thrombocytopenic purpura, myelodysplastic syndromes) or occasionally warfarin, other drugs or blood clotting defects.

Question 93

Glossitis

Answer 93

The term given when the tongue is sore or more appropriately, when the dorsum of the tongue become depapillated and red. Causes include anaemia, vitamin or iron (haematinic) deficiency, chemotherapy or radiation therapy, or infection, such as candidosis. Glossitis in haematinic deficiency states, is seen mainly in: > malabsorption states > pernicious anaemia > chronic bleeding from gastrointestinal or genitourinary tract > vegans > dietary faddists > resource poor circumstances (deficiencies of vitamins of the B group other than B12 are occasional cause of glossitis, mainly seen in chronic alcoholics or in those with malabsorption or starvation). The tongue may appear completely normal or there may be: - linear or patchy red lesions: especially in Vit B12 deficiency. - depapillation with erythema: in deficiencies of iron, folic acid or B vitamins lingual depapillation begins at the tip and margins of the dorsum, but later involves the whole dorsum (blad tongue). Various other patterns are described, in deficiencies of: - riboflavin: papillae enlarge initially, but are later lost - niacin: red, swollen, enlarged 'beefy' tongue - pyridoxine: swollen, purplish tonuge There may also be: - pallor - burning mouth syndrome - ulceration - angular stomatitis Differentiate from erythema migrans, lichen planus and acute candidosis. As sore tongue can be the initial symptom or iron, folate or vit B12 deficiency and can precede any haemoglobin fall, a full blood picture with assays of iron (serum ferritin), vitamin B and folate are essential. Biopsy is rarely indicated. Replacement therapy should be instituted after the underlying cause is established and rectified.

Question 94

Glossopharyngeal neuralgia

Answer 94

A rare condition in which pain with all the characteristics of trigeminal neuralgia is experienced in the posterior tongue, fauces, pharynx and sometimes beneath the angle of the mandible. Treatment is with carbamazepine or, in intractable cases, posterior fossa neurosurgery.

Question 95

Gonorrhoea

Answer 95

Infection with Neisseria gonorrhea in the mouth is uncommon or rarely recognised. Pharyngitis may occur after orogenital or oroanal contact. Pharyngitis may occur after orogenital or oroanal contact. Swab for culture and sensitivity. Treat with amoxicillin or ciprofloxacin.

Question 96

Granular cell myoblastoma (granular cell tumour)

Answer 96

A fairly common hamartoma, the origin of which is unclear. Presents as pedunculated or occasionally sessile swelling on the gingivae, tongue, buccal mucosa or lip. It should be excised. Histology shows a pseudo-epitheliamatous appearance, but it is benign.

Question 97

Haemophilia

Answer 97

Haemophilia A: deficiency of blood clotting factor VIII Haemophilia B: Christmas disease, factor XI deficience. Haemophilia C: Factor XI deficiency Von willebrand disease: deficiency in Von Willebrand factors

Question 98

Hairy leukoplakia

Answer 98

HIV-infected and other severely immunocompromised patients may develop white oral lesions with a vertically corrugated or 'hairy' surface which usually affects the lateral margins or dorsum of the tongue. Epstein-Barr virus may be implicated.

Question 99

Hand, foot and mouth disease

Answer 99

A common Coxsackie virus infection (usually A16; rarely A5 or A10), with an incubation of 2-6 days occurring in small epidemics, in children. Clinical features include oral ulcers resembling herpetic stomatitis, but affecting labial and buccal mucosa, no gingivitis, mild fever, malaise, anorexia, rash (red papules that evolve to superficial vesicles in a few days, found mainly on palma and soles). Diagnose from the clinical features. Serology is confirmatory, but rarely required. Differentiate from herpetic stomatitis, chickenpox. Management is symptomatic.

Question 100

Herpangina

Answer 100

A Coxsackie virus infection (A7, A9, A16; B1, B2, B3, B4 or B5) or echovirus (9 or 17) with an incubation period of 2-6 days. Clinical features include oropharyngeal ulcers, no gingivitis, cervical lymphadenitis (moderate), fever, malaise, irritability, anorexia, vomiting, but no rash. Diagnose from clinical features. Serology (theoretically) is confirmatory. Differentiate from herpetic stomatitis and chickenpox. Manage symptomatically.

Question 101

Hypercementosis

Answer 101

This may be idiopathic or arise in: > occlusal trauma > an overerupted non-opposed tooth > periapical infection > Paget disease > acromegaly

Question 102

Hyperparathyroidism

Answer 102

Primary hyperparathyroidism - usually due to a parathyroid adenoma, carcinoma or hyperplasia of parathyroid tissue or ectopic production of parathyroid hormone (PTH) (e.g. by tumours of lung or kidney). May also rarely occur in association with adenomas of other endocrine glands (the multiple endocrine adenoma syndromes). Serum calcium levels are raised, as usually is the alkaline phosphatase. Phosphate levels are reduced. The clinical features are the direct result of either excess PTH or calcium, and particularly include renal stones, bone lesions, polyuria and abdominal pain ('stones, bones and abdominal groans'). Anorexia and psychoses are not uncommon. Secondary hyperparathyroidism: usually the consequence of renal disease in which low serum calcium as a consequence of impaired renal production of dihydroxycholecalciferol induces increased parathyroid activity, and eventually hyperplasia of the parathyroid glands. Secondary hyperparathyroidism may also follow malabsorption syndromes. Calcium levels are usually normal or low. Tertiary hyperparathyroidism: A consequence of chronic overstimulation of the gland in secondary hyperparathyroidism, which rarely leads to neoplastic change in the parathyroids, which then escape the normal control of serum calcium. Oral manifestations of hyperparathyroidism include reduced bone density (the outlines of the maxillary antrum, inferior dental canal and inferior border of the mandible can become less distinct), loss of the lamina dura, root resorption and radiolucencies (particularly of the mandible). The radiolucent lesions are either areas of high osteoclastic activity or giant cell lesions. The giant cell lesions can present intraorally as epulides. Hyperparathyroidism is managed by excision of the neoplastic of hyperplastic parathyroid tissue. In the secondary and tertiary disorders, treatment of the underlying disorder is also required.

Question 103

Hypodontia

Answer 103

A reduction in the number of teeth. The tooth most commonly missing are third molars and maxillary lateral incisors, followed by mandibular and maxillary second premolars. It may be associated with microdontia. If >6 teeth missing, the term oligodontia is sometimes used. Anodontia is total absence of the dentition. A genetic trait, ectodermal dysplasia should be considered.

Question 104

Hypoglossal nerve lesions

Answer 104

LMN lesions of the twelfth cranial nerve lead to unilateral tongue weakness, wasting and fasciculation. When protruded the tongue deviates towards the weaker side. Bilateral supranuclear (UMN) twelfth nerve lesions produce slow, limited tongue movements; the tongue is still and cannot be protruded far, and fasciculation is absent. Causes of lesions may be within the brainstem (infarction, syrinobulbia, motor neurone disease or poliomyelitis; at the skull base (jugular and anterior condylar foramina) causes include trauma and tumours (nasopharyngeal carcinoma, glomus tumour or neurofibroma) and within the neck and nasopharynx trauma of tumours (nasopharygneal carcinoma, metastases) or polyneuropathy.

Question 105

Hypoparathyroidism

Answer 105

Usually caused by damage to, or loss of, parathyroid tissue during neck surgery (e.g. thyroidectomy). It may also arise rarely as a familial disorder (idiopathic hypoparathyroidism) or very rarely as a consequence of in utero damage when it is associated with cellular immunodeficiency. The major clinical features are related to the low serum calcium levels. Tetany (hyperexcitability of muscles) is the most obvious and disturbing feature and can lead to laryngeal spasm. Hypoparathyroidism frequently causes paraesthesia round the mouth. Tapping the skin over the facial nerve can elicit involuntary twitching of the muscles of the upper lip or ipsilateral side of face. Hypoparathyroidism can cause enamel hypoplasia of developing teeth and delayed tooth eruption. Treat with hydroxycholecalciferol.

Question 106

Hypophosphatasia

Answer 106

A rare genetic defect in alkaline phosphate, causing cemental aplasia or hypopolasia and tooth mobility and early loss. Serum alkaline phosphatase is decreased with increased vitamin B6 and increased urinary phosphoethanolamine.

Question 107

Idiopathic bone necrosis

Answer 107

An unusual disorder in which as small portion of bone, typically of the mylohyoid ridge, undergoes spontaneous necrosis and sequestration with pain and ulceration.

Question 108

Impetigo contagiosa

Answer 108

A highly contagious skin infection with streptococci (group A). Uncommon, and seen mainly in underprivileged children (aged 2-6 years), lesions spread by touch to several areas. Clinical features include papules which change into vesicles surrounded by erythema then multiple pustules with golden crusts. Regional lymphadenopathy is sometimes seen, but systemic symptoms are rare. Diagnosis is clinical together with culture of pus. On lips, herpes labialis is the prime differential diagnosis, but other vesiculobullous diseases should also be excluded. Management is with antibiotics. If there is no symptoms, oral flucloxacillin should be used. Staphylococcus aureus phage type 71 causes bullous impetigo, a severe form with fever.

Question 109

Leukaemia

Answer 109

A malignant proliferation of leukocytes. Common oral manifestations of leukaemia include lymphadenopathy, bleeding, petechiae, gingival swelling, ulceration. Other findings include sensory changes (particularly of the lower lip), extrusion of teeth, painful swellings over the mandible, parotid swelling, fungal infections, and predisposition to herpesvirus lesions. Diagnose by blood film, white cell count (raised), differential count (show blasts), platelet count (reduced) and bone marrow biopsy. Treat mainly with chemotherapy. Oral hygiene should be carefully maintained with chlorhexidine mouth rinses and a soft toothbrush. Prophylactic antifungal and antiviral therapy is also indicated. Many chemotherapeutic agents can cause oral ulceration. Methotrexate is a major offender, but ulceration may be prevented or ameliorated by concomitant intraveous administration of folinic acid or topical folinic acid. There are several types of leukaemia: - acute lymphoblastic leukaemia (ALL) - acute myeloblastic leukaemia (AML) - chronic lymphocytic leukaemia (CLL) - chronic myeloid leukaemia (CML).

Question 110

Acute lymphoblastic leukaemia (ALL)

Answer 110

The most common leukaemia of childhood, it has a peak incidence at 3-5 years of age. Malignant lymphoblasts proliferate and infiltrate the viscera, skin and central nervous system. Marrow infiltration causes granulocytopenia (predisposing to infection), thrombocytopenia (causes a bleeding tendency), and anaemia ('there is no leukaemia without anaemia).

Question 111

Acute myeloblastic leukaemia (AML)

Answer 111

The most common acute leukaemia of adults. Features are similar to acute lymphoblastic leukaemia, but central nervous system involvement is rare.

Question 112

Chronic lymphocytic leukaemia (CLL)

Answer 112

The most common chronic leukaemia. It mainly affects the older patient. Some patients are asymptomatic, while in other there is fever, weight loss, anorexia, haemorrhage and infections. Lymph node enlargement is early and may be detected in the neck. CLL may need to treatment.

Question 113

Chronic myeloid leukaemia (CML)

Answer 113

This is characterised by proliferation of myeloid cells in the bone marrow, peripheral blood and other tissues, and mainly affects those over 40 years of age. Splenomegaly and hepatomegaly are common. Anaemia, weight loss and joint pains are not uncommon. The prognosis is variable, but sooner or later there is transformation to an acute phase similar to AML (blast crisis).