Lec. 12 ORAL MEDICINE Dr.

Ali Al-Ibrahemy

NON INFECTIVE STOMATITIS

B. Ulcerations without preceding vesiculation
1. Behçet’s Syndrome
Behçet's syndrome was originally defined as a triad of oral aphthae, genital
ulceration and uveitis. However, it is a multisystem disorder with varied
manifestations and termed Behçet's disease. It is rare in the UK and USA but is
relatively common in countries bordering the Mediterranean (Behçet was a
Turk) and very common in Japan. The etiology is unknown but is thought to be
either an infection or an immunological reaction to an infectious agent. Patients
are usually young adult males between 20 and 40 years old. Patients suffer one
of four patterns of disease, namely:
 Mucocutaneous (oral and genital ulceration)
 Arthritic (joint involvement with or without mucocutaneous involvement)
 Neurological (with or without other features)
 Ocular (with or without other features)
The oral aphthae of Behçet's disease are not distinguishable from common
aphthae. They are the most consistently found feature and frequently the first
manifestation. Behçet's disease should therefore be considered in the differential
diagnosis of aphthous stomatitis particularly in patients in at risk racial group,
and the medical history should be checked for the features shown in the
frequency of other manifestations is highly variable. Special tests are not helpful
in diagnosis, apart from the pathergy test. The test is positive if there is an
exaggerated response to a sterile needle puncture of the skin. However, the test
must be interpreted by an experienced clinician and tends to be positive only in
Mediterranean patients. Moreover, a positive pathergy test does not correlate
with the presence of oral lesions or with the overall severity of the disease and is
rarely positive in UK patients. It is also not entirely specific for Behçet's disease.
The importance of making the diagnosis is indicated by the life-threatening
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nature of thrombosis and the risk of blindness or brain damage. The main
pathological finding is vasculitis and antiendothelial cell antibodies are
reportedly found in 50% of those with active disease, but their role remains
uncertain. The etiology is largely speculative but lesions may result from
immune-complex mediated vasculities of small vessels which is said to be present
in mucocutaneous and all types of lesion.
Diagnostic criteria for Behcet's disease
Major criteria Minor criteria
 Recurrent oral aphthae • Arthralgia or arthritis
 Genital ulceration • Gastrointestinal lesions
 Eye lesions • Central nervous system
 Skin lesions involvement
(Erythema nodosum, • Vascular lesions (thrombosis)
Subcutaneous thrombophlebitis,
hyperirritability of skin)
Treatment is difficult and requires a multiple approach. The oral ulceration may
be treated in the same way as the common form. When major aphthae are
severe or if there is frequent recurrence, thalidomide or colchicine, or both, are
often used and are highly effective.

2. Lichen Planus
Lichen planus is a common chronic inflammatory disease of skin and
mucous membranes. It mainly affects patients of middle age or over. Oral
lesions have characteristic appearances and distribution. The etiology of lichen
planus remains problematical. Cell-mediated immunological damage to the
epithelium and a plethora of immunological abnormalities has been reported.
Oral lichen planus is also a virtually invariable feature and an early sign of graft
versus-host disease but this does not clarify any immunological mechanisms.
Typical features of lichen planus
• Females account for at least 65% of patients
• Patients usually over 40 years
• Untreated disease can persist for 10 or more years
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• Lesions in combination or isolation comprise:
 Striae are most common and typically form sharply-defined snowy-white,
lacy, starry, or annular patterns. They may occasionally be interspersed
with minute, white papules. Striae may not be palpable or may be firmer
than the surrounding mucosa.
 Atrophic areas are red areas of mucosal thinning and often combined with
striae, so if the gingivae is involve, it appear shiny, inflamed and smooth
('desquamative gingivitis'). Soreness caused by atrophic lesions makes
toothbrushing difficult.
 Erosions are shallow irregular areas of epithelial destruction. These also
can be very persistent and may be covered by a smooth, slightly raised
yellowish layer of fibrin. The margins may be slightly depressed due to
fibrosis and gradual healing at the periphery. Striae may radiate from the
margins of these erosions.
 Plaques are occasionally seen in the early stages particularly on the
dorsum of the tongue. Otherwise they may result from persistent disease
and mainly affect the buccal mucosa.
• Common sites are:
 Buccal mucosa
 Dorsum of tongue
 Gingiva (infrequently)
• Lesions usually bilateral and very often symmetrical
• Lichen planus is a common skin disease but skin lesions are uncommon in
those who complain of oral symptoms. Skin lesions typically form purplish
papules; typical sites are the flexor surface of the forearms and especially the
wrists. Skin lesions help but are not essential to confirm the diagnosis of oral
lichen planus.
The diagnosis

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 The diagnosis of lichen planus can usually be made on the history, the
appearance of the lesions and their distribution. However, dysplastic
leukoplakias occasionally have a streaky whitish appearance. A biopsy should be
taken, particularly when striae are ill-defined, plaques are present or the lesions
are in any other ways unusual.
Treatment
No treatment is needed in asymptomatic lesions. Topical steroids (ointment
in orabase, intralesional injection), may be helpful. Systemic steroids in low
doses can be used in severe and extensive cases. The topical use of antiseptic
mouthwashes should be avoided.
3. Lichenoid drug reactions
This term is given to lichen planus-like lesions caused by either systemic
drug treatment or those where the histological picture is not completely
diagnostic.
A very wide range of drugs can cause lichenoid reactions of the skin, mucous
membranes or both. The clinical features are often indistinguishable from 'true'
lichen planus and usually consist only of white striae. When there is severe
atrophy or ulceration, detecting a possible causative drug may aid management
and a complete drug history is mandatory in all patients thought to suffer from
lichen planus. Some features which suggest a drug reaction is shown blew:-
 Onset associated with starting a drug
 Unilateral lesions or unusual distributions
 Unusual severity
 Widespread skin lesions
 Localised lesion in contact with restoration

In practice it can be difficult or impossible to differentiate lichenoid reactions

from lichen planus. Many patients are taking potentially causative drugs,
sometimes more than one.

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These are only the more common causes:
 Colloidal gold
 Beta-blockers
 Oral hypoglycaemics
 Allopurinol
 Non-steroidal anti-inflammatory drugs
 Methyldopa
 Penicillamine
 Some tricyclic antidepressants
 Thiazide diuretics
 Captopril

Biopsy can sometimes distinguish lichenoid reactions but the features are
relatively subtle and not completely specific. While biopsy is of value to exclude
other conditions it cannot usually distinguish lichen planus from a lichenoid
reaction. Lichenoid reactions are treated in exactly the same way as lichen
planus with withdrawal of drug(s) if possible. Thus, the absolute distinction
between lichen planus and lichenoid reaction is not always necessary for
treatment.
4. Lupus Erythematosus
Lupus erythematosus is a connective tissue disease which has two main
forms, namely systemic and discoid. Either can give rise to oral lesions which
may appear similar to those of oral lichen planus. Systemic lupus erythematosus
has varied effects, arthralgias and rashes are most common, but virtually any
organ system can be affected. A great variety of autoantibodies, particularly
antinuclear, is produced. Discoid lupus is essentially a skin disease with
mucocutaneous lesions indistinguishable clinically from those of systemic lupus.
These may be associated with arthralgias but rarely, significant autoantibody
production.
Clinically, oral lesions appear in about 20% of cases of systemic lupus, and
can rarely be the presenting sign. Typical lesions are white, often striate, areas
with irregular atrophic areas or shallow erosions, but the patterns, particularly
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those of the striae, are typically far less sharply defined than in lichen planus.
They are often patchy and unilateral and may be in the vault of the palate which
lichen planus typically spares. Lesions can form variable patterns of white and
red areas. There may also be small slit-like ulcers just short of the gingival
margins. In about 30% of cases, Sjogren's syndrome develops and rarely,
cervical lymphadenopathy is the first sign.
Diagnosis of systemic lupus erythematosus should be confirmed by the pattern
of antinuclear autoantibodies. The most specific is that to double-stranded
(native) DNA. Haematological findings in active SLE include a raised ESR,
anaemia and, often, leukopenia or thrombocytopenia.
Management
Oral lesions of discoid lupus erythematosus may respond in some degree
to topical corticosteroids. However, oral lesions in acute systemic lupus
erythematosus may not respond to doses of corticosteroids adequate to control
systemic effects of the disease. Under such circumstances, palliative treatment is
needed until disease activity decrease.
5. Wegener’s granulomatosis (Lethal midline granuloma syndrome)
These formerly ill-defined diseases can cause variable degrees of
destruction of the central facial tissues and a fatal outcome as their main
features. They start in the upper respiratory tract and are not clinically
distinguishable in their early stages. Important features of Wegener's
granulomatosis are illustrated below:-
• Granulomatous inflammation of nasal tract.
• Cavitation of the lungs.
• Potentially fatal glomerulonephritis.
• Proliferative gingivitis occasionally.
• Oral mucosal ulceration occasionally.
 Antineutrophil cytoplasmic antibodies (ANCAs) typically present.
 Histologically, granulomatous inflammation with giant cells and vasculitis.

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6. Eosinophilic ulcer (atypical or traumatic eosinophilic granuloma)
Tumour-like ulcerated lesions with a microscopic picture resembling that
of Langerhans' cell histiocytosis, may occasionally form in the oral soft tissues,
particularly the tongue, but also the gingivae and, occasionally, other sites.
Sometimes there is a history of trauma and, experimentally, crush injury to
muscle can induce a proliferative response with tissue eosinophilia. Clinically,
the ulcerated mass may be mistaken for a carcinoma but is typically soft: almost
any age can be affected.
Pathology and Treatment
There is typically a dense aggregation of eosinophils and cells which
resemble histiocytes beneath the ulcerated surface. The differential diagnosis is
from Langerhans' cell histiocytosis, which can be excluded by the absence of
Langerhans' cells markers. In practice, lesions usually heal spontaneously within
3-8 weeks, and management of an isolated soft tissue mass having these
histological characteristics, should be expectant. If not resolve, the surgical
excision is required and sent the lesion to biopsy.