Ulcerative Conditions Associated With Immunlogic Dysfunctions
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Transcript of Ulcerative Conditions Associated With Immunlogic Dysfunctions
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ULCERATIVE CONDITIONS
CONDITIONS ASSOCIATED WITH IMMUNLOGIC DYSFUNCTIONS
APHTHOUS ULCERS
The most common of all types of non-traumatic ulceration that affects themucous membranes
Incidence ranges from 20-60%
Prevalence tends to be higher in professional persons and those in uppersocio-economic groups
Also known as canker sores & recurrent aphthous stomatitis
APHTHOUS ULCERS
etiology & pathogenesis
Cause is unknown
Etiologic factors:
Immunologic factors
Microbiologic factors
Nutritional factors
Other factors: hormonal alterations, stress, trauma & food allergies
APHTHOUS ULCERSetiologic factors: immunologic
The most promising avenue of investigation
Aphthous ulcers result from a focal immune dysfunction in which Tlymphocytes play a significant role
Causative agent
Endogenous antigen autoimmune
Exogenous antigen hyperimmune
APHTHOUS ULCERSetiologic factors: microbiologic
After several studies, this theory has been discarded
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APHTHOUS ULCERSetiologic factors: nutritional
Deficiencies of vitamin B12, folic acid & iron
Correction of the above deficiencies has produced improvement or cures
APHTHOUS ULCERSetiologic factors: others
Hormonal alterations
Stress
Trauma
Food allergies: nuts, chocolate & gluten
None of these is seriously regarded as being important in the primarycausation
But can have triggering role
APHTHOUS ULCERSclinical features
Three forms of aphthous ulcers
Minor aphthous ulcers
Major aphthous ulcers
Herpetiform aphthous ulcers
All are believed to be part of the same disease spectrum with a commonetiology
Differences are essentially clinical & correspond to degree of severity
APHTHOUS ULCERS
clinical features
All forms present as painful recurrent ulcers
Occasionally, patient have prodromal symptoms: tingling or burning prior tothe appearance of the lesions
Ulcers are not preceded by vesicles
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Rarely appear: gingiva & hard palate
APHTHOUS ULCERSclinical features
Appear on :
Vestibular & buccal mucosa
Tongue
Soft palate
Fauces
Floor of the mouth
MINOR APHTHOUS ULCERSclinical features
Most commonly encountered form
Appear as single, painful, oval ulcers
Less than 0.5 cm in size
Covered by a yellow fibrinous membrane
Surrounded by an erythematous halo
Lesion lasts 7-10 days
MINOR APHTHOUS ULCERSclinical features
Heals without scar formation
When the lateral or ventral surfaces of the tongue are affected, pain tends tobe out of proportion to the size of the lesion
Multiple oral aphthae may occasionally be seenRecurrences vary from one person to another
Periods of freedom from this disease may range from a matter of weeks to aslong as years
MINOR APHTHOUS ULCERS
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MINOR APHTHOUS ULCER
MINOR APHTHOUS ULCER
MAJOR APHTHOUS ULCERSclinical features
Was referred to as periadenitis mucosa necrotica recurrens (PMNR) orSuttons disease
Regarded as the most severe expression of aphthous stomatitis
Lesions are larger & more painful
Persist longer than minor aphthae
MAJOR APHTHOUS ULCERS
clinical features
Appear crateriform because of the depth of inflammation
Heal with scar
Lesions may take as long as 6 weeks to heal
as soon as one lesion disappears, another one starts
MAJOR APHTHOUS ULCERSclinical features
In patients who experience an unremitting course with significant pain anddiscomfort, systemic health may be compromised because of difficulty ineating and psychologic stress
MAJOR APHTHOUS ULCERS
MAJOR APHTHOUS ULCER
HERPETIFORM APHTHOUS ULCERS- clinical features
Unlike herpetic infection, this form are not preceded by vesicles
Exhibit no virus-infected cells
HERPETIFORM APHTHOUS ULCERS-clinical features
clinically presents as recurrent crops of small ulcers
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Although movable mucosa is predominantly affected, palate & gingiva maybe involved
Pain may be considerable
Healing occurs in 1-2 weeks
HERPETIFORM APHTHOUS ULCERS
HERPETIFORM APHTHOUS ULCER
APHTHOUS ULCERShistopathology
It is generally accepted that important clues to the etiology and pathogenesismay be found during the early stages , therefore biopsies are unnecessaryand rarely done
APHTHOUS ULCERSdifferential diagnosis
Diagnosis is generally based on history & clinical appearance
Secondary recurrent oral herpes is often confused with aphthous ulcers butcan be distinguished from it, because the latter is not precede by a vesicle
APHTHOUS ULCERStreatment
In patients with occasional or few minor aphthous ulcers usually no treatmentis needed because of minor discomfort
For patients who are affected severely, some form of treatment can providesignificant control but not necessarily cure
Because of its immunologic defect related factor, treatment includes drugsthat can regulate the immune response
APHTHOUS ULCERStreatment
Chemotherapeutic agents used:
Systemic steroids
Topical steroids
antibiotics
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Other drugs
APHTHOUS ULCERStreatment / systemic steroids
Appropriate for severe disease
Should not be use unless the clinician as experience in this treatment area/working with a knowledgeable consultant
Should know the effects & side effects of systemic steroids therapy
APHTHOUS ULCERStreatment / systemic steroids
Effects:
Anti-inflammation
Immunosuppression
Gluconeogenesis from protein & fat
Altered fat metabolism
Fluid retention from Na resorption & K excretion
Potentiation of vasopressors
Increased gastric secretions
Suppression of pituitary-adrenal axis
CNS effects
Ocular effects
Side effects:
Therapeutic
Aggravation of Tb & other infections, delayed wound healing
Aggravation of diabetes, muscle weakness & osteoporosis
Buffalo hump, hyperlipidemia
Moon face, weight gain
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Blood pressure elevation, aggravation of congestion, heart failure
Aggravation of peptic ulcer
Adrenal atrophy
Psychologic changes
Cataracts, glaucoma
APHTHOUS ULCERStreatment / systemic steroids
For immediate control of severe case, a lo to moderate dose of prednisoneover a short period of time is recommended
A typical regimen:
20-40 mg daily for 1 week
Followed by another week at half the initial dose
Prednisone should be taken in one bolus in the morning
APHTHOUS ULCERStreatment / systemic steroids
A slow steroid taper is not necessary if the treatment lasts for less than 4weeks
In patients requiring higher dosage prolonged, or maintenance therapy,
an alternate-day regimen may be used after initial daily therapy
APHTHOUS ULCERStreatment / topical steroids
If used judiciously, can be effective and safe in mild to moderate condition
Creams & gels: Orabase
APHTHOUS ULCERStreatment / topical steroids
Side effects :
Systemic
Suppression of pituitary-adrenal axis
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Iatrogenic Cushings syndrome
Local (skin)
Striae
Atrophy
Hypopigmentation
Telangiectasia
Induced acne
Folliculitis
candidiasis
APHTHOUS ULCERStreatment / antibiotics
Tetracycline oral suspension
Used to eliminate secondary bacterial infection of the ulcers
Combination of tetracycline oral suspension, nystatin oral suspensionanddephenhydramine hydrchloride ( Benadryl) provides more clinical benefits
Oral rinse containing chlorhexidine gluconate .12% ( Peridex, Orahex)
APHTHOUS ULCERStreatment / other drugs
Vitamin A derivatives
Anti-inflammatory drugs
Sulfones sulfonamides
BEHCETS SYNDROME
A multisystem disease in which recurrent oral aphthae are a consistentfeature
System involve are: GIT, cardiovascular, ocular, CNS, articular, pulmonary,dermal
Oral manifestations are relatively minor, involvement of other sites , esp. theeyes & CNS can be quite serious
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BEHCETS SYNDROMEetiology
Cause is basically unknown
Underlying disease mechanism may likely be immuno-dysfunction in whichvasculitis has a part
Genetic predisposition to the frequent presence of HLA-B15 within the group
BEHCETS SYNDROMEclinical features
Typically affect the oral cavity, eyes & genitalia
Recurrent arthritis of the wrists, ankles and knees
Cardiovascular manifestations results fro vasculitis and thrombosis
CNS manifestations are in the form of headaches, but infarcts have beenreported
BEHCETS SYNDROMEclinical features
Presence of pustular erythema nodosum-like skin lesions
Relapsing polychondritis ( e.g. auricular cartilage & nasal cartilage) in
association with Bechets stigmataBehcets stigmata known as MAGIC syndrome Mouth & genital ulcers withInflamed Cartilage
BEHCETS SYNDROMEclinical features
Oral manifestations are identical to the ulcers of aphthous stomatitis, minor,and with the same distribution
Ocular changes such as uveitis, conunctivitis and retinitis
Genital lesions are ulcerative in nature & may cause significant pain &discomfort
BEHCETS SYNDROMEclinical features
Painful ulcerative lesions may also occur around the anus
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Inflammatory bowel disease & neurological problems
BEHCETS SYNDROMEhistopathology
T-lymphocytes are very prominent
Infiltration of neutrophils appear within the vessel representingleukocytoclastic vasculitis
BEHCETS SYNDROMEdiagnosis
Diagnosis is based on clinical signs & symptoms described above
Biopsy & laboratory tests will produce non-specific results
BEHCETS SYNDROMEtreatment
Systemic steroids
Immunosuppressive drugs
Chlorambucil
Azathioprine
Dapsone, cyclosporine & interferon
REITERS SYNDROMEetiology
Cause is unknown
Abnormal immune response to microbial antigen regarded as a likelymechanism for the multiple manifestations of this syndrome
REITERS SYNDROMEclinical features
Major components are:
Arthritis
Non-gonococcal urethritis
Conjuctivitis or uveitis
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Urethritis precedes the appearance of other lesions
REITERS SYNDROMEclinical features
Mucocutaneous lesions seen in 50% of patients with this syndrome
Maculopapular lesions may occur on the genitalia
Oral lesions are relatively painless aphthous-like ulcers occurring anywhere inthe oral cavity
Tongue lesions is similar to that of georaphic tongue
REITERS SYNDROMEclinical features
Occur predominantly in white males in their third decade
Duration of the disease varies from weeks to months
Recurrences are not uncommon
REITERS SYNDROMEdiagnosis
Upon the recognition of various signs and symptoms
No specific laboratory tests
REITERS SYNDROMEtreatment
Non-steroidal anti-inflammatory agents
Antibiotics can be added to the treatment regimen
ERYTHEMA MULTIFORMEetiology & pathogenesis
Basic cause is unknown
Hypersensitivity reaction is suspected
Evidence shows that the disease mechanism may be related to antigen-antibody complexes that are targeted for small vessels of the upper dermis &submucosa
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ERYTHEMA MULTIFORMEetiology & pathogenesis
Precipitating factors can be identified in 50% of the cases
Infections
drugs
Other factors which trigger EM
Malignancy
Vaccination
Autoimmune disease
Radiotherapy
ERYTHEMA MULTIFORMEetiology & pathogenesis
Infections frequently reported include
Herpes simples ( HSV types I & II)
TB
Histoplasmosis
Various types of drugs that precipitate EM:
Barbiturates
sulfonamides
ERYTHEMA MULTIFORMEclinical features
Usually an acute self-limiting process that affects the skin &/or mucous
membranes25-50% of the patients with cutaneous EM will have oral manifestations
May be chronic in nature or recurrent acute type
In recurrent cases, prodromal symptoms are experienced prior to anyeruptions
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ERYTHEMA MULTIFORMEclinical features
Young adults are mostly affected
Often develop in the spring or fall & may have recurrence seasonally
Include multiple & varied clinical appearances that are associated withcutaneous manifestations ( multiforme)
ERYTHEMA MULTIFORMEclinical features
Target or iris lesion is the classic skin lesion of EM
It is consists of concentric erythematous rings separated by rings of near-normal color
The skin in the center of these lesions may be erythematous or tan,representing resolution
ERYTHEMA MULTIFORMEtarget or iris lesion in the skin
ERYTHEMA MULTIFORMEtarget lesion on the palmar skin
ERYTHEMA MULTIFORME
clinical features
Typically, the extremities are involved
Usually in symmetric distribution
Other types of skin manifestations include:
Macules
Papules
Vesicles
Bullae
Urticarial plaques
ERYTHEMA MULTIFORMEclinical features
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Orally, EM characteristically presents as an ulcerative disease
Varying from a few aphthous-type lesions to multiple, superficial, widespreadulcers
Areas involved are the lips, buccal mucosa, palate & tongue
ERYTHEMA MULTIFORMEon the lower lip
ERYTHEMA MULTIFORMEoral lesions: lower lip
ERYTHEMA MULTIFORMEoral lesions: gingiva
ERYTHEMA MULTIFORMEclinical features
Recurrent oral lesions may appear as multiple painful ulcers similar to theinitial episode
As less asymptomatic erythematous patches with limited ulceration
Symptoms range from mild discomfort to severe pain
ERYTHEMA MULTIFORMEclinical features
Patient experience considerable apprehension because of occasionalexplotive onset
Systemic signs & symptoms are:
Headache
Slightly elevated temperature
lymphadenopathy
ERYTHEMA MULTIFORMEclinical features
Stevens-Johnson syndrome- a major variant of EM
Intense involvement of the mouth, eyes, skin, genitalia & occasionally theesophagus & respiratory tract may be seen
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Systemic signs & symptoms are more pronounced
Cutaneous & mucosal lesions are more extensive
ERYTHEMA MULTIFORMEclinical features: stevens-johnson
Lips my become encrusted
Oral lesions may cause exquisite pain
Superficial ulceration, often preceded by a bullae is common to all affectedsites
Ocular inflammation ( conjunctivitis & uveitis) may lead to scarring &blindness in some patients
ERYTHEMA MULTIFORMEocular lesion
ERYTHEMA MULTIFORMEhistopathology
No specific or consistent microscopic pattern
Dermal vessels shown to have IgM complement and fibrin in their walls- thissupport an immune-complex vasculitis cause of EM
ERYTHEMA MULTIFORMEdifferential diagnosis
Target or iris skin lesion is present, clinical diagnosis is straightforward
If skin lesion is absent, it has to be compared with primary HSV infections,aphthous ulcers, pemphigus vulgaris, bullous pemphigoid & erosive lichenplanus
ERYTHEMA MULTIFORMEdiagnosis
To diagnose EM, the ff. should be present:
General lack of systemic symptoms
Favored oral location of lips, buccal mucosa, tongue, & palate
Larger sized ulcers- not precede by vesicles
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Presence of target skin lesions
History of recent drug ingestion or infection
ERYTHEMA MULTIFORMEtreatment
Symptomatic treatment for mild affected patients
Topical corticosteroids with anti-fungals
In severe cases, moderate dose of systemic corticosteroids may be used toshorten the course of the disease & abort recurrences or reduce its intensity
ERYTHEMA MULTIFORMEtreatment
Supportive measures to provide patients with substantial benefits:
Oral irrigation
Adequate fluid intake
Use of antipyretics
LUPUS ERYTHEMATOSUS
Encompasses three (3) recognized subsets:
Systemic (acute) LE
Subacute cutaneous LE
Discoid (chronic) LE- which may have oral manifestations
LUPUS ERYTHEMATOSUS
Systemic lupus erythematosus (SLE) is of greatest importance because of profound impact it has on many organ system
LUPUS ERYTHEMATOSUSDiscoid lupus erythematosus (DLE) is the least aggressive form, affectingpredominantly the skin and rarely progressing to a more severe form
It may be of great cosmetic significance because of its predilection for theface
LUPUS ERYTHEMATOSUS
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Subacute cutaneous lupus erythematosus (SCLE) lies intermediate betweenSLE and DLE
Skin lesions of mild to moderate severity
Mild systemic involvement
Appearance of some abnormal autoantibodies
LUPUS ERYTHEMATOSUSetiology & pathogenesis
Result from an autoimmune process that may be influenced by genetic orviral factors
Both humoral & cell-mediated arms of the immune system are involved
LUPUS ERYTHEMATOSUSclinical features- DLE
Characteristically seen in middle age, especially women
Lesions frequently appear solely on the skin, most commonly on the face &scalp
Oral & vermillion lesions are also frequently seen but usually accompanycutaneous lesions
LUPUS ERYTHEMATOSUSclinical features- DLE
On the skin, lesions appear as disk-shaped erythematous plaques withhyperpigmented margins
As the lesions expand peripherally, the center heals, with the formation of scar & formation of pigment
Involvement of hair follicles results in permanent hair loss (alopecia)
LUPUS ERYTHEMATOSUSclinical features- DLE
Mucous membrane lesions appear in 25% of the patients with cutaneous DLE
Most frequently affected are: buccal mucosa, gingiva, & vermilion
Lesions appear as erythematous plaques or erosions
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Usually present are delicate, white, keratotic striae radiating from theperiphery of the lesions
LUPUS ERYTHEMATOSUSchronic DLE
LUPUS ERYTHEMATOSUSLE on the mucosa of hard palate
LUPUS ERYTHEMATOSUSLE on the buccal mucosa
LUPUS ERYTHEMATOSUSclinical features- DLE
Diagnosis of oral lesions may not be evident on the basis on clinicalappearance, but it is often suspected in the presence of skin lesions
Progression to SLE is very unlikely although the potential does exist
LUPUS ERYTHEMATOSUSclinical features- SCLE
Skin lesions are annular or papulosquamous in nature
Lesion persist for weeks to months and heal without a scar
Oral lesions are those similar to DLE
Mild systemic symptoms in the form of musculoskeletal complains as well asserologic abnormalities are frequently seen
LUPUS ERYTHEMATOSUSclinical features- SCLE
Circulating antibodies to cytoplasmic components may be found in affectedpatients
One is known as anti-Ro or Sjogrens syndrome A antibody (SS-A) sinceit may also be found in the serum of patients with Sjogrens syndrome
anti-La (SS-B)- an auto-antibody
LUPUS ERYTHEMATOSUSclinical features-SCLE
Long term prognosis is believed to be good, with progressive to SLE anunlikely event
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LUPUS ERYTHEMATOSUSclinical features- SLE
Skin and mucosal lesion are relatively mild
Patient complaints are dominated by multiple system involvement
Numerous auto-antibodies directed against nuclear & cytoplasmic antigensare found in patients with SLE
These antibodies when complexed to their corresponding antigens either inserum or in the target organ resulting in a wide variety of clinical signs &symptoms
LUPUS ERYTHEMATOSUSclinical features- SLE
Skin involvement results in an erythematous rash, seen over the malarprocesses and bridge of the nose resulting to butterfly appearance
Other affected areas are: face, trunk and hands
Disk-shaped lesions are non-scarring may flare as systemic involvementprogresses
LUPUS ERYTHEMATOSUSclinical features-SLE
Oral lesions are similar to that of DLE
Ulceration
Erythema
Keratosis
Areas frequently involved are:
Vermilion
Buccal mucosa
Gingiva
palate
LUPUS ERYTHEMATOSUSclinical features-SLE
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Systemic symptoms:
initial
Fever
Weight loss
Malaise
Will progress into involving many organs:
Joints
Kidneys
Heart
lungs
LUPUS ERYTHEMATOSUSclinical features- SLE
The inflammatory of the various involved tissues result in a wide array of signs & symptoms
Kidney- glomerulopathy- most important & the most common cause of deathfor patient with SLE
LUPUS ERYTHEMATOSUSdiagnosis
Serologic tests for autoantibodies are positive in patients with SLE
The ANA test is the most reliable & relatively specific for SLE
Another serologic test for SLE is the LE test , but less sensitive & less specific
LUPUS ERYTHEMATOSUShistopathology
The most important microscopic feature diagnostically is the interfacechange, since it appears that the basal layer is the primary target in the skin& mucous membrane
LUPUS ERYTHEMATOSUSdifferential diagnosis
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Lesions similar with erosive lichen planus, but lupus lesions are lesssymmetrical in distribution
Keratotic striae in LE are more delicate & subtle than that of lichen planus
LUPUS ERYTHEMATOSUSdifferential diagnosis
The presence of characteristic skin lesions or systemic signs & symptomsmay help diagnose LE
Biopsy & direct immunofluorescent testing should help confirm the clinicalimpression
Negative serologic tests for autoantibodies would rule out systemicinvolvement
LUPUS ERYTHEMATOSUStreatment
DLE is usually treated with topical steroids
High-potency creams can be used intra-orally but should be used withcaution on the facial skin because of secondary cutaneous changes
LUPUS ERYTHEMATOSUStreatment
Systemic steroids may be utilized in the treatment of SLE and SCLE
Prednisone combined with immunosuppressive agends
Anti-malarials, non-steroidal anti-inflammatories, dapsone, & retinoids
DRUG REACTIONSetiology & pathogenesis
Although the skin is more commonly involved in adverse reactions to drugs
Oral mucosa ay occasionally be the target organ or maybe the sole site of involvement (or part of a skin reaction)
Drugs Known to Cause Adverse Reactions
Anti-malarials
Aspirins
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Barbiturates
Chlorpromazine
Cimetidine
Codeine
Erythromycin
Gold compounds
Indomethacin
Ketoconazole
Local anesthetics
Meprobamate
Methlydopa
Oxyprenolol hydrochloride
Penicillin
Phenytoin
Retinoids
Streptomycin
Sulfonamides
Tetracycline
Drug Reactionspathogenesis
Immunologic mechanism
Non-immunologic mechanism
Drug Reactionpathogenesis
Immunologic Mechanism
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Triggered an antigenic component on drug molecule ( allergicreaction)
Depend on the following factors:
immunogenecity of the drug
frequency of exposure
the route of administration
innate reactivity of the patients immune system
Drug Reactionpathogenesis
Non-immunologic Mechanism
Do not stimulate an immune response in the patient
Are not antibody-dependent
Drugs directly affecting the mast cells, causing the release of chemicalmediators
Reactions may be overdoe, toxicity or side effects
Drug Reactionclinical features
Cutaneous manifestations
Oral manifestations
Drug Reactionclinical features
Cutaneous manifestations
Varied depending upon many factors:
Type of drug
Drug dosage
Individual patient differences
Changes: (appear rapidly)
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Anaphylaxis
Angioedema
Urticaria
Drug Reactionclinical features
Acquired Angioedema
IgE-mediated allergic reactions
Precipitated by drug or foods (nuts or shellfish)
Substances act as sensitizing agent that elicit IgE production
Drug Reactionclinical features
Hereditary Angioedema
Produces the similar clinical changes as that of acquired type
Inherited through autosomal dominant trait having deficiency of the inhibitorof the first component of compemen, C1 esterase
Drug Reactionclinical features
Angioedema
Either acquired or hereditary
Appear soft, diffuse, painless swelling
Usually affecting the lips, neck or face
No color change
Condition subsides after 1-2 days which may recur at a later date
Emergency treatment may be required to prevent respiratory distress orglottic or laryngeal involvement
Drug Reactionclinical features
Other cutaneous manifestations
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Urticaria
Maculopapular rash
Erythema
Vesicles
Ulcers
Target lesions (EM)
Drug Reactionclinical features
Oral manifestations
Erythematous, vesicular or ulcerative
May mimic lichen planus ( lichenoid drug reations)
Widespread of ulcers typical or EM are often representative of a drug reaction
Drug Reactionshistopathology
Non-specific: spongiosis, necrotic keratinocytes, lymphoid infiltrates,eosinophils
Biopsy may be helpful but not diagnostic
Drug Reactiondiagnosis
Diagnosis requires a high index of suspicion and careful history taking
Recent use of drug
Withdrawal of suspected drug should result in improvement
Drug Reactiontreatment
Important measure in management is identification & withdrawal of thecausative agents
Antihistamines
Corticosteroids (occasionally)
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Contact Allergyetiology & pathogenesis
Antigenic stimulation of vast array of foreign substances
Immune response is cell-mediated
Sensitization phase:
Langerhans cell recognize the foreign antigen & present them it the T-lymphocytes
Local lymphocytes secrete chemical mediators producing allergicreactions
Contact Allergyclinical features
Lesions directly adjacent to the causativ agent
Presenting lesions ranges from erythematous to ulcerative
Frequently seen in skin, uncommon intraorally
Contact Allergyclinical features
Materials containing agents known to cause oral contact allergic reactions:
Toothpaste
Mouthwash, mouthrinse
Candy
Chewing gum
Topical antimicrobials
Topical steroids
Iodine
Essential oils
Denture base materials
Cinnamon white , lichenoid, red or ulcerative lesions
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Contact Allergyhistopathology
Epithelium & connective tissue sho inflammatory changes
Spongiosis & vesiculation is also seen
Dilated blood vessels
Eosinophils may be seen
Contact Allergydiagnosis
Careful history taking is essential
Establish a cause & effect relationship may not be possible
Biopsy may be helpful but non-conclusive
Patch testing may be helpful but may give a false-positive or false-negativeresult
Contact Allergytreatment
Elimination of the offending agent or material
Healing should take place in 1- 2 weeks
Topical steroids may hasten the healing process
Wegeners Granulomatosis
Inflammatory condition of unknown origin
Wegeners Granulomatosisclinical features
Triad of upper respiratory tract, lung and kidney involvement
Occasionally, only 2 of 3 sites are affected
Lesions may present in the oral cavity & skin & other organ system
Basic process common to all foci is necrotizing vasculitis with granulomaformation
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Wegeners Granulomatosisclinical features
Rare disease of middle age
Initial presentation often occur with head & neck
Sinusitis, rhinorrhea, nasal stuffiness & epistaxis seen w/ or w/o feverarthralgia & weight loss
In majority of the cases, nasal or sinus ( maxillary) involvement is seen & isoften seen in the course of the disease
Wegeners Granulomatosisclinical features
Destructive lesions are typically ulcerated
Necrosis & perforation of nasal septum
Perforation of hard palate is uncommon
Intraorally, red granular gingival lesions
Generalized & results in relatively uniform enlargement
Wegeners Granulomatosisclinical features
Most patients have kidney involvement
Focal necrotizing glomerulitis
Renal failure is the final outcome
Inflammatory lung lesions
Intensify from slight to severe
May lead to respiratory failure
Wegeners Granulomatosishistopathology
Basic pathologic process is granuloatous with necrotizing vasculitis
Presence of acute & chronic inflammatory cells
Affected small vessels show a mononuclear infiltrate within their walls
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Wegeners Granulomatosisdiagnosis
Depend upon the finding of granulomatous vasculitis in biopsy tissue of URT,evidence of lung involvemen or kidney lesions
Anti-neutrophil cytoplasmic antibodies (ANCA)
Negative culture & tissue identification of microorganisms would help rule outinfectious processes
Immunohistochemical staining could be used to rule out neoplasm
Wegeners Granulomatosistreatment
Cytotoxic agent
Cyclophosphamide
Corticosteroids
Remissions seen 75% of cases
Midline Granuloma
Diagnosis made exclusion of other granulomatous & necrotizing midfaciallesions
Represents an atypical or unrecognized lymphoma
Midline Granulomaetiology
Unknown etiology
Hyper-immune response to an unidentified antigen
Lymphoid neoplasia from chronic immune stimulation has been suggested
Midline Granulomaclinical features
Unifocal destructive process in the midline of the oronasal region
Lesions appear as aggressive necrotic ulcers that are progressive & non-healing
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Extension through soft tissue, cartilage & bone is typical
Perforation of the nasal septum & hard palate is characteristic
Midline Granulomaclinical features
Without treatment, the inflammatory process eventually consumes thepatient
Because of continuous erosion into vital structures especially blood vessels:death has been a typical outcome
Midline Granulomahistopathology
Appear like acute & chronic inflammation in partially necrotic tissue
Several biopsies are required to confirm the diagnosis
Midline Granulomadifferential diagnosis
Wegeners granulomatosis
Infectious disease
Bacterial infection ( TB)
Deep fungal diseases
Neoplasm
Poorly differentiated squamous cell carcinoma, sarcomas &lymphomas
Midline Granulomattreatment
Treatment of choice
High dose of local radiation
Optimistic prognosis
Corticosteroids
Chronic Granulomatous Disease
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Inherited condition
Resulting clinical defect: altered neutrophil macrophage function
Cells have the capacity to phagocyte microorganism but lack the ability to killcertain bacteria & fungi
Chronic Granulomatous Disease- clinical features
Manifestations appear during childhood
Predominantly occur in males
Affect different organs: lymph nodes, lung, liver, spleen, bone & skin
Recurrent & persistent
Oral lesions: multiple ulcers
Recurrent & persistent
Chronic Granulomatous Disease- histopathology
Granular nodular
Granulomas may exhibit central lesions
Chronic Granulomatous Disease- diagnosis
Clinical features & tissue samples
Neutrophil function tests confirm the diagnosis
Differential diagnosis: Crohns disease, TB, histoplasmosis, blastomycosis,tularemia
Chronic Granulomatous Disease - treatment
Use of specific antimicrobial agents
Neutropenia
Rare blood dyscrasia of unknown cause
Manifests as severe cyclic depletions of neutrophils from blood and marrow,with mean cycle of periodicity of about 21 days
Signs & symptoms: fever, malaise, oral ulcers, cervical lymphadenopathy &infections may appear neutropenic episodes
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Patients are prone to exaggeration of periodontal disease
No definitive treatment