OM Sheet #3 By Asma Hannoun

by **Sura** 13/11/2012, 2:27 am

https://www.4shared.com/file/Mv8_sS-z/OM_3_ASMA.html

by **Shadi Jarrar** 15/11/2012, 6:41 pm

lichen planus

lichen planus is a leratively common,chronic inflammatory disease that affects oral mucosa. It affects women more than men and occurs most often in middle aged adults. Skin lesions, when present consist of itchy pinkish flat top papules which affect flexor surfaces of the arms trunk and legs.

The Reticular type is the most common presentation and manifests as asymptomatic white lacy streaks on the mucosa known as wickhams striae.

The Erosive form may undergo malignant transformation.

Lichen planus may also affect mucous membranes of genital tract, rectum ,epiglottis and GIT. The clinical presentation of LP lesions may also resemble lichenoid eruptions which are identical clinically and histologically, however lichenoid lesions ’’reactions’’ may be single in comparison to the usual bilateral appearance of oral LP lesions .

Etiology:

The cause of lichen planus is not well-known, it is postulated to have a genetic predisposition that it is initiated by a variety of factors, including emotional stress, and hypersensitivity to drugs or foods.

Occasionally, it may be related to factors such as :materials used in dental work ‘’amalgam’’, hepatitis C infection, or graft versus host disease.

Treatment:

Reticular LP is asymptomatic and no treatment is needed.

In the Erosive type, topical corticosteroids and analgesic mouth washes are useful in controlling symptoms. Short term course of oral steroids may be used in difficult cases .

Pemphigus vulgaris:

Pemphigus vulgaris (PV) is a life threatening autoimmune visiculobullous disease that affects the skin and mucous membranes . the mouth is the only site of involvement in 50% of all cases of pemphigus , and is the initial site of presentation in almost 75% of cases.

The oral lesion is a fragile bullae arising on otherwise normal mucosa,almost always is ruptured by the time of diagnosis.

The lesion starts most commonly on the buccal mucosa, often areas of trauma along the occlusal line, the palate and gingival are other common sites of involvement . occasional patients have erosive lesions restricted completely to the gingiva (desquamative gingivitis ) .

Skin lesion are similar , except that the more heavily keratinized epidermis allows blisters to remain intact much longer. The overall mortality is less than 6%, either from electrolyte loss ( loss of body fluid from a large number of blisters), wound infectionor treatment complications.

Etiology:

PV is an autoimmune disease with IgG autoantibodies to desmoglein 3 .

It may coexist with other autoimmune disorders like myasthenia gravis and thymoma or with neoplasm such as lymphoma.

It may be triggered by medications, although most cases are idiopathic.

Diagnosis:

Positive Nikolsky sign( application of pressure to an apparently normal mucosa will result in formation of a new lesion )

Smear of the early bullous lesion will show tzank cells ( floating acantholytic cells )

Definitive diagnosis requires the demonstration of anti-desmoglein3 antibodies by direct immunofluorescence on a perilesional biopsy. These antibodies appear as IgG deposits along the desmosomes between epithelial cells, particularly of the prickle cell layer, a pattern reminiscent of chicken wire.

Anti-desmoglein3 antibodies can also be detected in a blood sample using the ELISA technique.

Treatment:

High doses of systemic corticosteroids ( prednisolone) is initially required, combined later with the so called steroid- sparing immunosuppressant azathioprine which is among those being used to reduce complications of high dose steroids.

If PV is restricted to the mouth, then lower doses of prednisolone can be used to control the disease.

Recently, rituximab, an anti-CD20 antibody, was found to improve otherwise untreatable severe cases of PV.

Mucous membrane pemphigoid:

MMP is a rare chronic autoimmune vesiculobullous disease characterized by blistering lesions that predominantly affect the various mucous membranes of the body. The mucous membranes of the mouth and eyes are most often affected.

Mouth involvement presents as recurrent painful erosions that most commonly involve the gingiva, followed by the palate and the buccal mucosa. The lesion of the gingiva is called desquamative gingivitis . when the cornea of the eye is affected, repeated scarring may result in blindness.

Diagnosis:

Positive Nikolsky sign ( slight rubbing of an apparently normal mucosa results in formation of a new lesion )

Biobsy is essential and should be taken from the edge of the ulcerative lesion for immunofluoresent analysis that shows linear deposition of IgG and C3 along the basement membrane zone

A low titre of circulating autoantibodies against bullous pemphegoid 1 ( bp1) antigen is depicted, although 50% of cases have bp2 antigen.

Treatment:

Patients with mild localized disease may benefit from topical steroids, either oral rinse or gel-based for oral lesions or ointment-based for skin lesions.

Patients with more extensive disease and progressive scarring require systemic therapy with prednisolone and immunosuppressants.

High dose intravenous immunoglobulin has been used successfully in the treatment of MMP in patients who were refractory to other therapies.

Ophthalmic referral is essential once the diagnosis is confirmed, because nearly 25% of patients with oral lesions will have ocular lesions .

Linear IgA disease:

Linear IgA disease( LAD) or dermatosis is a rare chronic autoimmune vesiculobullous disease characterized by the presence of linear IgA deposits along the basement membrane zone .

This disease can develop at any age, some authors differentiate between 2 clinical subtypes ,the adults subtype and the paediatric subtype, subtype which affects mainly children under the age of 5 years.

The clinical presentation is similar to other blistering diseases, such as bullous pemphigoid. Oral manifestations in LAD have been reported as minor clinical presentations. Linear IgA disease should be included in the differential diagnosis of bullous dermatosis with oral lesions .

Diagnosis:

Biopsy is essential and should be taken from the edge of the ulcerative lesion for immunofluorescent analysis that shows linear deposition of IgA along the basement membrane zone.

Treatment:

Withdrawal of the offending drug

Most cases have been reported to respond to dapsone or sulfapyridine. A response may be seen in 48-72 hours

Severe cases of linear IgA dermatosis respond to a short course of oral corticosteroids.

Bullous pemphigoid:

BP is a chronic autoimmune vesiculobullous skin disease that rarely involves mucous membranes. It usually occurs in people 70 years of age and older , very rarely seen in children. Bullous pemphigoid is the most common of the autoimmune bullous dermatoses, representing 70% of these diseases. Clinically the earliest lesions may appear urticarial ( like hives), eventually tense bullae erupt symmetrically, most commonly at the inner thighs and upper arms, but the trunk and extremities are frequently involved. Oral lesions are smaller, form more slowly and are less painfull than those seen in pemphigus vulgaris.

Desquamative gingivitis:

The term desquamative gingivitis(DG) is used as a clinical description of the gingiva which may manifest as aresult of various underlying conditions .

It is painfull, frequently spares the marginal gingival but can involve the whole thickness of the attached gingival. Plasma cell gingivitis (PCG) is a rare condition that presents as DG. It is generally believed to represent a hypersensitivity reaction to exogenous substances such as toothpastes, chewing gum. Chronic ulcerative stomatitis ( CUS) is a rare disease characterized by unique immunostaining properties. It seems to almost exclusively affect white women in late middle age with an average age at onset of 60 years. It mimics erosive oral lichen planus both clinically and histologically, and DG can be its presenting feature as well.

Diagnosis:

A definitive diagnosis depends on taking an incisional biopsy from a perilesional site (with intact epithelium) and sending a fresh specimen for immunostaining to exclude pemphigus vulgaris ( chicken wire appearance of epithelium) pemphigoid( linear IgA deposit along the basal cell layer), and CUS( a speckled pattern of IgG deposit in the basal one third of the epithelium ) .

In addition, a fixed specimen is required to exclude erosive oral lichen planus ( band-like lymphocytic infiltrate subepithelium), and PCG ( adense plasmacytic infiltrate in the lamina propria ).

Circulating ANA are detectable in case of CUS , whereas anti-desmoglein3 antibodies can be detected in a blood sample in case of pemphigus vulgaris.

Identification of the possible inciting agent to exclude PCG by taking a carefull clinical history( patch testing is usually negative ). Removal of the offending agent results in resolution of symptoms.

Treatment:

Oral hygiene measures.

Potent topical steroid gel may bring resolution in some cases. Custom trays ( occlusive therapy) may be used to localize topical steroid medications on the gingival tissues.

Corticosteroid gel should be used with systemic steroid ( prednisolone) in case of pemphigus vulgaris.

CUS does not respond to steroid therapy, but a long lasting favourable therapy appears to be achieved with systemic hydroxychloroquine.

PCG may respond to 2% fusidic acid gel.

Dermatitis herpitiformis:

DH is an uncommon chronic autoimmune and subepidermal papulovesicular eruption, mainly involving the skin and is associated with gluten-sensitive enterotherapy.

DH is characterized by intensely itchy,chronic papulovesicular eruptions, usually distributed symmetrically on extensor surfaces aspecially the elbows, knees, buttocks, back of neck, scalp and back. Oral lesions occur in up to 70% of those with skin lesions and affect mainly the palate, buccal mucosa or gingiva as an erythematous, purpuric,vesicular or ulcerated.

Aetiology:

Gluten sensitivity may be involved with IgA autoantibodies against gliaden and especially endomysium may be responsible.

Diagnosis:

Simple blood test for IgA antibodies ( anti-gliaden and anti-endomysial) and anti-tissue transglutaminase antibodies. these antibodies are present in the majority of patients as has been found with celiac disease.

Biopsy is essential and should be taken from the edge of the lesion for direct immunofluorescent analysis that shows granular IgA deposits along the tips of papillae of both involved skin and mucosa. This distinguishes it from linear IgA bullous dermatiosis.

Histologically, the typical of DH consists of microabscesses at the tips of connective tissue papillae.

These tests should be done before the patient starts on a gluten-free diet, otherwise they might produce false negatives.

If the patient has already started a gluten-free diet, it might be necessary for him to come off it for some weeks before the tests can be done reliably.

Treatment:

DH responds well to medication and changes in diet .

Dapsone is an effective treatment, and itching is significantly reduced within 2-3 days. It is an antibacterial drug and its role in the treatment of DH’’ which is not caused by bacteria!!’’ is poorly understood. Dapsone can cause adverse effects on the blood, and regular blood monitoring is required.

Strict gluten-free diet must also be followed, and this will usually be a life long requirement. This will reduce any associated intestinal damage, and the risk of other complications.

After some time on a gluten-free diet, the dosage of dapsone can usually be reduced or even stopped, although this can take up to anything from 1-3 years.

Epidermolysis bullosa:

EB is a rare inherited bullous disorder characterized by the presence of extremely fragile skin and recurrent blister formation, resulting from minor mechanical friction or trauma. The 2 skin layers lack the anchors that hold them together, and any action that creates friction between the layers( like rubbing or pressure) will create blisters and painfull sores. There are 3 major types of EB based on different sites of blister formation within the skin structure and these can be classified as: EB simplex, dystrophic, and lethalis.

Depending on the type of EB, the condition can vary from minor blistering of the skin to a lethal form involving other organs. The condition generally starts at birth or soon after. the simplex type usually commences in the first year of life. the oral mucosa can be affected , but not severely. The dystrophic type is characterized by scar formation that follows healing but physical and mental growth remain unaffected. scarring can restrict mobility and impair daily activities. The oral mucosa and other mucous membranes including the larynx, pharynx, and esophagus may be involved. Associated findings include dystrophic changes in the nails, hair and teeth. Severe anemia and amyloidoses usually gives this type an unfavourable prognosis. in EB lethalis, there is a high incidence of abortion and stillbirths, but if the infant is born alive extensive bullae usually develop rapidly and there is little tendency to heal. EB should be distinguishedfrom EB acquisita, which is an autoimmune blistering disease that is not inherited and often doesn’t develop until adult life.

Aetiology:

Epidormolysis bullosa is a rare genetic disorder.

Diagnosis:

Evaluate infection using bacterial cultures from poorly healing wounds or wounds that appear infected.

Evaluate anemia using CBC with iron studies in patients with severe EB.

Obtain 2 biopsy specimens, one for transmission electron microscopy(TEM) and the other using immunofluorescence microscopy for subtyping EB.

If there are swallowing or feeding difficulties, upper endoscopy or an upper GI series may be needed.

Because most forms of EB are inherited, the results of genetic testing, usually done with a blood sample sent to a lab for analysis, can confirm the diagnosis.

Treatment:

Good dental hygiene is very important, including regular dental visits.

Working with a physical therapist can help keep the full range of motion in the joints and minimize contractures.

Measures should be taken to avoid skin trauma and avoid hot environments.

Prevent skin infections by applying antibiotic ointments to wound-like areas.

Skin grafting for denuded or ulcerated areas of the skin may be necessary

When there are blisters in the mouth or esophagus, eating soft foods can help prevent making the sores worse. Antifungal therapy is used if there is candida infection.

If there are swallowing difficulties, use of oral steroids for short periods of time may be prescribed. Long-term use of steroids is generally not recommended.

EB acquisita may be treated with oral steroids and immunosuppressants.

Current clinical research has included a bone marrow transplant, strongly suggesting that a cure may have been found.

Angina bullosa haemorrhagica:

ABH is the term used to describe sudden appearance of one or more blood blisters within the mouth. The disease accurs usually in the middle aged or elderly patients and there is no famililial history .

The lesions may be confused with other more serious disorders such as mucus membrane pemphigoid ,epidermolysis bullosa,linear IgA disease and dermatitis herpetiformis, However, the isolated nature ,rapid healing, and rare recurrence of ABH blisters generally are sufficient findings to rule out the previously mentioned conditions.

Good luck

Done by :Asma Hanoun

OM Sheet #3 By Asma Hannoun

OM Sheet #3 By Asma Hannoun

Re: OM Sheet #3 By Asma Hannoun