Keypoints

Keypoints

Causes may include:

• Inflammatory causes:

 infection (the usual cause)

 local viral (e.g. herpes simplex infections) or bacterial (dental, scalp, ear, nose or throat) – including cat-scratch fever, staphylococcal lymphadenitis and cervicofacial actinomycosis (Figure 3.2)

 systemic: viral, e.g. infectious mononucleosis, cytomegalovirus, or HIV infection; bacterial, including syphilis, tuberculosis, brucellosis; fungal, rarely; parasitic, toxoplasmosis; Kawasaki disease (mucocutaneous lymph node syndrome)

 others: sarcoidosis, Crohn disease, orofacial granulomatosis, connective tissue diseases

• Malignant disease:

 local (oral, scalp, ear, nose or throat usually; rarely thyroid)

 systemic: leukaemias and lymphomas, carcinomas, Letterer–Siwe disease

• Drugs:

 particularly phenytoin.

Clinical features:

 lymphadenitis: discrete tender, mobile, enlarged firm nodes; rarely suppurate

 metastases: discrete or matted, fixed, enlarged, hard nodes (rubbery in lymphomas); may ulcerate.

Investigations:

 culture and sensitivity testing, if infection is suspected

 search for lesion in drainage area (imaging if necessary)

 blood picture; monospot test or toxoplasma serological profile (TSP)

 fine needle aspiration biopsy (FNAB) or biopsy if neoplasm suspected.

Diagnosis: see above.

Management:

 treat cause

 if oral disease is ruled out, then referral to appropriate health provider is needed

Acute bacterial (ascending) sialadenitis

Rare, except when following hyposalivation.

Typical orofacial symptoms and signs: painful swelling of one gland only, with red, shiny overlying skin, trismus, and purulent discharge from duct (Figures 3.3, 3.4).

Main oral sites affected: parotid gland.

Aetiopathogenesis: usually a bacterial infection ascends the duct of a reduced- or non-functioning salivary gland. Infectious agents include pneumococci, Staphylococcus aureus or viridans streptococci.

Gender predominance: male.

Age predominance: older adults.

Extraoral possible lesions: none.

Main associated conditions: dehydration; poor oral hygiene.

Differential diagnosis: other causes of sialadenitis: mainly mumps.

Investigations: purulent exudate for culture and sensitivities.

Main diagnostic criteria: clinical features.

Main treatments: antimicrobials (flucloxacillin if staphylococcal and not allergic to penicillin); sialogogues (saliva stimulants such as chewing gum or pilocarpine).

Mucocele

Common.

Typical orofacial symptoms and signs: dome-shaped, bluish, translucent, fluctuant, painless swelling, usually <10 mm diameter (Figure 3.5) which may rupture. Recur frequently. Deeper mucoceles are less common, more persistent and are often retention cysts (Figure 3.6).

Main oral sites affected: lower lip mainly. Superficial mucoceles are small intra-epithelial lesions (<5 mm diameter) sometimes simulating a vesiculobullous disorder but usually producing a small vesicle only; seen often in the soft palate. Ranula is the term used for the ‘frog belly’ appearance of a large retention mucocele in the floor of the mouth often arising from the sublingual gland and, rarely, burrowing through the mylohyoid muscle (plunging ranula).

Aetiopathogenesis: usually extravasation of mucus from a damaged minor salivary gland duct; rarely retention of mucus within a salivary gland or its duct.

Gender predominance: none.

Age predominance: young people.

Extraoral possible lesions: none except neck swelling and airway obstruction if plunging ranula.

Main associated conditions: superficial mucoceles may be seen in lichen planus or Graft versus host disease.

Differential diagnosis: diagnosis is clear-cut but neoplasm must be excluded, particularly in the upper lip.

Investigations: microscopic features.

Main diagnostic criteria: clinical history and features.

Main treatments: if asymptomatic and small, observe; otherwise, use cryosurgery, laser ablation, micro-marsupialization or excision. Some lesions spontaneously resolve. Systemic gamma linolenic acid (evening primrose oil) has also been used.

Mumps (acute viral sialadenitis)

This is more common in childhood if vaccination with MMR (mumps, measles and rubella vaccine) is not taken.

Typical orofacial symptoms and signs: incubation period 14–21 days. Infections are often subclinical. Malaise, fever, anorexia and sialadenitis – painful, diffuse swelling of one/both parotids and sometimes submandibulars may be seen (Figure 3.7). Saliva is non-purulent but the duct is inflamed. Trismus and occasionally dry mouth may be present.

Main oral sites affected: parotids bilaterally usually.

Aetiopathogenesis: mumps virus; rarely Coxsackie, ECHO, EBV or HIV infection.

Gender predominance: none.

Age predominance: typically in children.

Extraoral possible lesions: complications are uncommon but may include pancreatitis, encephalitis, orchitis, oophoritis and deafness.

Main associated conditions: as above.

Differential diagnosis: differentiate from obstructive and/or bacterial sialadenitis and recurrent juvenile parotitis mainly.

Investigations: mumps antibody titres (rarely needed); serum amylases or lipases (occasionally); ultrasound.

Main diagnostic criteria: clinical history and features.

Main treatments: symptomatic (reduce fever, pain and malaise with paracetamol, and maintain hydration).

Necrotizing sialometaplasia

(see page 289)

Salivary gland neoplasms

These are uncommon. Classification of the most common salivary neoplasms is:

• Adenomas:

 pleomorphic adenoma (PA: mixed tumour)

 Warthin tumour (adenolymphoma or papillary cystadenoma lymphomatosum)

 ‘monomorphic’: adenolymphoma/oncocytic adenoma/ (canalicular, basal cell, others)

• Others:

 polymorphous low-grade adenocarcinoma

 mucoepidermoid carcinoma

 acinic cell carcinoma

 adenoid cystic and other carcinomas.

Typical orofacial symptoms and signs: asymptomatic, firm and sometimes nodular swelling in one gland (usually parotid) and possible eversion of ear lobe (Figures 3.8). Warthin tumour may be bilateral. No hyposalivation. Malignant neoplasms classically may grow rapidly, may cause pain, may ulcerate and may involve nerves (e.g. facial palsy).

Main oral sites affected: most tumours involve the parotid (75%), where most are benign pleomorphic adenomas (60%). Most other salivary tumours are in the minor glands, where many are malignant (60%), often arising from palatal glands (Figures 3.9–3.11). Few tumours are in the submandibular or sublingual glands. Submandibular tumours can be benign or malignant. Most sublingual gland tumours are malignant.

Intraoral (minor) salivary gland neoplasms

• Intraoral salivary gland neoplasms are less common than neoplasms in major glands, but a higher proportion are malignant.

• Mucoepidermoid carcinoma is the most common intraoral salivary neoplasm, but adenoid cystic carcinoma and pleomorphic adenoma (PA) are relatively common in the mouth.

• Salivary gland tumours in the tongue are usually malignant – and are especially adenoid cystic carcinoma.

• Salivary gland tumours in the sublingual gland are usually malignant but are rare.

• Salivary gland tumours in the lips are usually seen in the upper lip but are typically benign (pleomorphic or other adenoma).

Typical orofacial symptoms and signs: Pleomorphic adenomas are typically rubbery and often lobulated (Figures 3.12, 3.13); benign neoplasms form painless swellings. Malignant tumours are not initially distinguishable clinically from benign tumours but, in their later stages, are often painful and ulcerate, invade perineurally and they metastasize to upper cervical lymph nodes.

Main oral sites affected: the palate is the intraoral site of predilection.

Aetiopathogenesis: unknown.

Gender predominance: females.

Age predominance: older persons.

Extraoral possible lesions: none.

Main associated conditions: none.

Differential diagnosis: from other causes of lumps or ulcers, particularly:

 lower lip salivary tumours – from mucocele, benign connective tissue tumours

 tongue salivary tumours – from carcinoma, benign connective tissue tumours

 palatal salivary tumours – from oral carcinoma, benign connective tissue tumours, lymphomas, antral carcinoma or necrotizing sialometaplasia.

Main diagnostic criteria: ultrasound, advanced imaging and microscopy.

Main treatments: wide excision; radiation treatment if perineural invasion, and microscopic examination.

Sarcoidosis

Uncommon granulomatous condition.

Typical orofacial symptoms and signs: salivary gland swelling usually painless, bilateral (Figure 3.14). Often hyposalivation is present. May be cervical lymphoadenopathy; mucosal nodules; gingival hyperplasia; or labial swelling.

Main oral sites affected: lips, palate, major salivary glands.

Aetiopathogenesis: unknown.

Gender predominance: female.

Age predominance: adult.

Extraoral possible lesions: can affect any organ, typically lungs and lymph nodes (e.g. hilar nodes); also ocular (e.g. uveitis) and skin (e.g. erythema nodosum) lesions. The rare combination of parotitis, anterior uveitis, VIIth cranial nerve paralysis and fever is termed uveoparotid fever (Heerfordt–Waldenstrom) syndrome.

Main associated conditions: sometimes autoimmune disorders.

Differential diagnosis: from other causes of salivary gland swelling (particularly sialosis, Sjögren syndrome and IgG₄ disease, hyposalivation, metastatic disease, lymphomas, Wegener granulomatosis, rheumatoid nodules, varicella–zoster infection and atypical infections such as Mycobacterium avium complex, cytomegalovirus, and cryptococcosis.

Investigations: lesional biopsy; chest radiograph; gallium scan, raised serum angiotensin-converting enzyme (SACE) and adenosine deaminase. Vitamin D and prolactin levels are often increased.

Main diagnostic criteria: microscopy and SACE.

Main treatments: corticosteroids, methotrexate or other immunosuppressive drugs if lungs or eyes are involved, or if there is hypercalcaemia.

Sialosis

Typical orofacial symptoms and signs: painless, usually bilateral salivary gland swelling (Figure 3.15).

Main oral sites affected: parotids.

Aetiopathogenesis: the common feature is autonomic dysfunction. Frequently idiopathic; known causes include:

 neurogenic: various drugs, such as isoprenaline

 dystrophic-metabolic: anorexia–bulimia, alcoholic cirrhosis, diabetes, malnutrition, thyroid disease, acromegaly and pregnancy.

Gender predominance: male but depends on cause.

Age predominance: older patients.

Extraoral possible lesions: dependent upon aetiopathogenesis.

Main associated conditions: see aetiopathogenesis.

Differential diagnosis: sarcoidosis, Sjögren syndrome, IgG₄ disease and Warthin tumour mainly.

Investigations: ultrasound, MRI; blood glucose, liver function tests, immunoglobulins; possibly growth hormone levels.

Main diagnostic criteria: clinical, ultrasound, MRI and exclusion of other diagnoses.

Main treatments: identify and treat predisposing cause.

Sjögren syndrome

Uncommon, the cause is unknown but it is immunological and possibly viral. The common features are dry mouth and dry eyes: joint and other problems may be associated.

Typical orofacial symptoms and signs: saliva is frothy and not pooling; the mucosa may be parchment-like with food debris (Figure 3.16), the lips often dry (Figure 3.17) and the tongue lobulated and depapillated (Figure 3.18). There may be complaints of dry mouth (hyposalivation): difficulty in eating dry foods, disturbed taste, and disturbed speech and swallowing. Rampant caries, candidosis and recurrent sialadenitis may be seen (Figures 3.19 and 3.20). Salivary and lachrymal glands may sometimes swell (Figures 3.21, 3.22). There may be conjunctival dryness and redness (termed injection) (Figure 3.23) and rheumatoid disease (Figure 3.24).

Aetiopathogenesis: autoimmune inflammatory exocrinopathy.

Gender predominance: women.

Age predominance: middle age or older.

Extraoral possible lesions: dry eyes (keratoconjunctivitis sicca): initially asymptomatic, later gritty sensation, itching, soreness or inability to cry. Swollen lachrymal glands. Extraglandular disease may precede exocrine problems by years, and includes fatiguability, fever and Raynaud phenomenon. Late complications include:

 glomerulonephritis

 lymphoma.

Main associated conditions: there is no connective tissue disease in primary (SS-1, sometimes termed sicca syndrome) but, in secondary Sjögren syndrome (SS-2) there is typically rheumatoid arthritis or less frequently primary biliary cirrhosis, and occasionally other autoimmune disorders. Sjögren syndrome predisposes especially to B cell non-Hodgkin lymphoma (NHL) – a mucosa-associated lymphoid tissue (MALT) malignancy. The risk is greatest in SS-1; affects up to 5% of patients and is seen mainly in mucosal extranodal sites (salivary glands – especially the parotids, mouth, skin, nodes, stomach, lung). Lymphoma is a high risk in those with persistent salivary gland swelling, splenomegaly or lymphadenopathy. Diagnosis of lymphoma is best by:

 ultrasonography

 high-resolution CT

 MRI

 MRI contrast sialography (gadolinium MRI with fat subtraction)

 histology.

Most of these lymphomas are relatively low-grade and require either no treatment apart from regular follow-up or low-dose chemotherapy.

Differential diagnosis: dry mouth can result from many other causes. Similar sicca syndromes may be seen in HCV or HIV disease, IgG₄ syndrome, or in graft-versus-host disease (GVHD).

Investigations: salivary flow rates (reduced); auto-antibodies, especially rheumatoid factor, Ro (SS-A) and La (SS-B) antibodies; serum IgG₄ levels, and ultrasound. Labial salivary gland biopsy is carried out in some centres and this and IgG₄ levels are most helpful in Ro/La negative patients. Sialography and/or scintigraphy are occasionally employed but are largely superseded by ultrasound.

Main diagnostic criteria: clinical features plus Ro (SS-A) and La (SS-B) antibodies, and ultrasound; occasionally labial salivary gland biopsy.

Main treatments: control underlying disease if possible (e.g. ciclosporin, azathioprine, hydroxychloroquine and anti-B cell monoclonals such as the anti-CD20 rituximab)

 dry eyes – methylcellulose eye drops or rarely ligation or cautery of nasolacrimal duct

 dry mouth:

– saliva substitutes (mouth-wetting agents)

– saliva stimulants (sialogogues); sugar-free chewing gum, or drugs (pilocarpine, cevimeline, anetholetrithione or bethanecol) may be used to stimulate salivation

– interferon-alpha

– preventive dental care (oral hygiene, limitation of sucrose and other sugar intake, use of fluorides, amorphous calcium phosphate, chlorhexidine)

– treat infections.

IgG₄ syndrome

IgG₄ syndrome (‘IgG₄ related systemic sclerosing disease’, ‘IgG₄-related autoimmune disease’, ‘IgG₄-related systemic disease’, ‘IgG₄-positive multiorgan lymphoproliferative syndrome’) often manifests with enlarged salivary glands (previously called Mikulicz disease or Kuttner tumour [sclerosing sialadenitis]) and one-third also suffer from dry eyes and dry mouth and arthralgias, so for many years this was considered a subgroup of Sjögren syndrome.

IgG₄ syndrome affects mainly middle-aged men. Many other organs and tissues are also involved, including pancreas (the most commonly affected tissue), gall bladder, bile duct, retroperitoneum, kidneys, lung, prostate, lymph nodes, breast, and thyroid and pituitary glands.

Patients have neither anti-Ro/SS-A nor anti-La/SS-B autoantibodies but they have low titres of rheumatoid factor (RF), antinuclear autoantibodies (ANA), and decreased serum complement. Treatment includes systemic corticosteroids or anti-CD20 therapy (rituximab).

Keypoints

Actinic burns and cheilitis

Mainly seen in fair-skinned individuals exposed in sunny climes or at high altitude.

Typical orofacial symptoms and signs: erythema, oedema, vesiculation and occasionally haemorrhage typify burns (Figures 3.25, 3.26); later whitish lesions or keratosis characterize actinic cheilitis – which is potentially malignant.

Main oral sites affected: lower lip.

Aetiopathogenesis: shortwave ultraviolet (UV) light (sunlight may also trigger herpes labialis and lupus erythematosus).

Gender predominance: male.

Age predominance: old age.

Extraoral possible lesions: cutaneous cancers on other areas of the face and exposed skin.

Differential diagnosis: from other causes of burns (e.g. friction and heat).

Investigations: biopsy.

Main diagnostic criteria: history and clinical features; biopsy persistent lesions.

Main treatments: prophylaxis – reduce sun exposure; bland or barrier creams (Uvistat); topical bleomycin, or lip shave.

Allergic angioedema

Uncommon: mainly seen in those with allergic (atopic) tendency.

Typical orofacial symptoms and signs: rapid development of oedematous swelling (Figure 3.27).

Main oral sites affected: lip(s) and tongue; oedema may spread to involve the neck and hazard the airway.

Aetiopathogenesis: type 1 allergic response.

Gender predominance: none.

Age predominance: none.

Extraoral possible lesions: atopy (allergic rhinitis, hayfever or eczema).

Main associated conditions: as above.

Differential diagnosis: from hereditary angioedema (C1 esterase inhibitor deficiency), and other causes of diffuse facial swelling including: oedema from trauma, infection or insect bite; surgical emphysema; Crohn disease, orofacial granulomatosis; sarcoidosis; cheilitis glandularis; lymphangioma; haemangioma.

Investigations: allergy testing, C1 esterase inhibitor levels.

Main diagnostic criteria: history of atopic disease and/or exposure to allergen; allergy testing (prick test).

Main treatments: mild angioedema – antihistamines; severe angioedema – intramuscular adrenaline, and intravenous corticosteroids.

Angioma (haemangioma)

These are fairly common oral lesions.

Typical orofacial symptoms and signs: a painless reddish, bluish or purplish soft vascular lesion which blanches on pressure (diascopy) (Figures 3.28, 3.29).

Main oral sites affected: most common on the lip, tongue, or buccal mucosa as painless reddish, or often bluish or purplish soft lesions which usually blanch on pressure, are fluctuant to palpation, are level with the mucosa or have a lobulated or raised surface. Haemangiomas are at risk from trauma and prone to excessive bleeding if damaged (e.g. during tooth extraction). Occasionally, oral haemangiomas calcify (phlebolithiasis).

Aetiopathogenesis: haemangiomas represent hamartomas or vascular anomalies. In children, they are usually congenital and developmental in origin. In adults, they may be acquired.

Gender predominance: female.

Age predominance: many haemangiomas appear in infancy, most by the age of 2 years, grow slowly and, by age 10, the majority have involuted (resolved). In adults, however, haemangiomas rarely involute spontaneously; rather they remain static or slowly enlarge.

Extraoral possible lesions: haemangiomas are typically seen in isolation but a few may be multiple and/or part of a wider syndrome such as:

 Sturge–Weber syndrome (angioma that extends deeply and rarely involves the ipsilateral meninges, producing a facial angioma and seizure disorder, sometimes with learning impairment)

 blue rubber bleb naevus syndrome (BRBNS) – multiple cutaneous venous malformations in association with visceral lesions, most commonly affecting the GI tract

 Maffucci syndrome – multiple angiomas with enchondromas

 Dandy–Walker syndrome – a congenital brain malformation involving the cerebellum, or other posterior cranial fossa malformations.

Main associated conditions: as above.

Differential diagnosis: varicosities, pyogenic granuloma, lymphangioma, Kaposi sarcoma and epithelioid angiomatosis.

Investigations: for large angiomas, angiography may be needed rather than biopsy. After intravenous administration of contrast medium, enhancement is observed in haemangiomas in areas corresponding to those with high signal on T2-weighted MRI.

Main diagnostic criteria: angiomas blanch on pressure (diascopy), or contain blood on aspiration with a needle and syringe.

Main treatments: most angiomas are small, of no consequence and need no treatment but if they do for aesthetic or functional reasons or because of previous episodes of significant bleeding, they respond well to cryosurgery, laser ablation or intralesional injections of corticosteroids, sclerosing agents, interferon alpha, or systemic corticosteroids or propranolol. Very large haemangiomas may need to be treated with ligation or embolization – mainly for cosmetic reasons or if bleeding is troublesome.

Carcinoma

Uncommon in England and Wales (600 cases/year) and declining: much more common nearer the Equator, especially in white-skinned people.

Typical orofacial symptoms and signs: thickening, induration, crusting or ulceration, usually at vermilion border of lower lip just to one side of midline (Figures 3.30, 3.31). There is late involvement of the submental and other lymph nodes.

Main oral sites affected: lower lip.

Aetiopathogenesis: predisposing factors include UV irradiation (sun), immunosuppression and tobacco.

Gender predominance: male.

Age predominance: old age.

Extraoral possible lesions: cutaneous cancers on face and other sun-exposed skin.

Main associated conditions: xeroderma pigmentosum and discoid lupus erythematosus predisposed.

Differential diagnosis: from herpes labialis, keratoacanthoma, and (rarely) basal cell carcinoma (on skin), molluscum contagiosum.

Investigations: biopsy. Fibreoptic pharyngolaryngoscopy with autofluorescence (symptom-directed bronchoscopy/oesophagoscopy); ultrasound-guided fine needle aspiration biopsy of any neck mass; staging – supported by MRI (or CT) from skull base to sternoclavicular joints (plus USgFNA and/or FDG-PET if anything equivocal); CT thorax.

Main diagnostic criteria: biopsy is confirmatory.

Main treatments: surgery (wedge resection or lip shave) or irradiation. The prognosis is good: 70% 5-year survival.

Discoid lupus erythematosus (DLE)

Rare. DLE may be categorized as:

• Localized DLE – the head and neck only are involved.

• Widespread DLE – other areas are affected, even if the head and neck are also involved. People with widespread disease may have abnormalities of blood or positive serology, and are more likely to develop SLE (systemic lupus erythematosus).

Typical orofacial symptoms and signs: lesions on vermilion border are scaly and crusting. Intraoral lesions have central atrophic and often indurated red area with border of radiating white striae, and peripheral telangiectasia (Figure 3.32). There is a small predisposition to carcinoma of the lip.

Main oral sites affected: buccal mucosa, palate/alveolar ridges and lips (almost always the lower lip).

Aetiopathogenesis: unclear: drugs, hormones and viruses may contribute in genetically predisposed persons.

Gender predominance: females.

Age predominance: adults.

Extraoral possible lesions: the discoid rash is usually on the face with red patches that are thick and often scaly, appearing red with a whitish, or at least, lighter-coloured, scaly rim. As the patches heal they tend to scar. If discoid lupus occurs on the scalp the hair will be lost, leaving permanent bald areas.

Main associated conditions: none; discoid lupus is a type of lupus that tends to be confined to the skin and mucosa and other organs in the body are not involved.

Differential diagnosis: systemic lupus erythematosus, lichen planus, leukoplakia (keratosis), lichenoid white lesions and carcinoma.

Investigations: biopsy; serology to exclude SLE.

Main diagnostic criteria: histology.

Main treatments: topical corticosteroids (e.g. betamethasone valerate 0.1% or clobetasol cream) or tacrolimus; cryosurgery or excision of localized lesions; follow-up because of increased risk for malignant transformation.

Erythema multiforme

Uncommon.

Typical orofacial symptoms and signs: serosanguinous exudate on ulcerated swollen lips, and ulcers (Figures 3.33, 3.34).

Main oral sites affected: lips and oral mucosa.

Aetiopathogenesis: putative reaction to microorganisms (herpes simplex, mycoplasma), to drugs (e.g. NSAIDs, antimicrobials, sulphonamides, beta blockers, dapsone, salicylates, tetracyclines) or to other factors.

Gender predominance: males.

Age predominance: young adult.

Extraoral possible lesions: may affect mouth alone, or skin and/or other mucosa. The minor form affects only one or two mucosal sites and/or the skin. The major form (Stevens–Johnson syndrome) affects more than two mucosal sites and is widespread, with skin rashes, fever and toxicity. Rashes are various but typically ‘target’ lesions or bullae. The most severe form is toxic epidermal necrolysis (TEN) when the lesions affect most of the body surface and the condition is life-threatening.

Main associated conditions: as above.

Differential diagnosis: from other lip lesions, and other causes of mouth ulcers – particularly primary herpes stomatitis and pemphigus.

Investigations: history of exposure to agents and biopsy.

Main diagnostic criteria: clinical picture; biopsy sometimes helpful.

Main treatments: minor form – symptomatic treatment and systemic corticosteroids; major form – systemic corticosteroids or other immunomodulatory drugs.

Exfoliative cheilitis (factitious cheilitis, le tic de lèvres)

An uncommon chronic superficial inflammatory disorder characterized by hyperkeratosis and desquamation of the vermilion epithelium.

Typical orofacial symptoms and signs: persistent scaling of the lips.

Main oral sites affected: often starts in the centre of the lower lip (Figures 3.35–3.38) and spreads to involve the whole of the lower or of both lips. The patient may complain of irritation or burning and can be observed frequently biting or sucking the lips. Lip scaling and crusting is more or less confined to the vermilion border, persisting in varying severity for months or years. There may be bizarre yellow hyperkeratotic or thick haemorrhagic crusts.

Aetiopathogenesis: most patients seem to have a personality disorder. A preoccupation with the lips is prevalent in some individuals. In some cases it appears to start with chapping or with atopic eczema, and develops into a habit tic. Many cases are thus thought to be factitious, caused by repeated self-induced trauma such as repetitive biting, picking, lip sucking, chewing or other manipulation of the lips. Exacerbations have been associated with stress.

Gender predominance: female.

Age predominance: children and young adults.

Extraoral possible lesions: none.

Main associated conditions: none.

Differential diagnosis: actinic cheilitis, contact cheilitis, glandular cheilitis, lupus erythematosus, Candida infections (where it is sometimes termed cheilo-candidosis), and HIV infection.

Investigations: biopsy is sometimes indicated and allergy testing for severe cases.

Main diagnostic criteria: diagnosis is restricted to those few patients whose cheilitis cannot be attributed to other causes, such as contact sensitization or UV light.

Main treatments: some cases resolve spontaneously or with improved oral hygiene. Reassurance and topical corticosteroids may be helpful but often exfoliative cheilitis is refractory to treatment. When a factitial cause is suspected, a psychiatric consultation and care may be beneficial; some improve with psychotherapy and antianxiolytic or antidepressant treatment.