Mucocutaneous Disease Flashcards
What is a lichenoid reaction and its aetiology?
= clinically + histologically similar to LP
A:
1. Drug induced
2. Dental materials
3. Idiopathic
State the clinical presentation of lichenoid reactions?
1) Unilateral or bilateral (often unilateral/asymmetric if reaction induced by local materials)
2) Can be ulcerative
3) Soreness as in LP (esp. erosive form)
4) Resolves on stopping of drug
State 10 drugs a/w lichenoid reactions?
- Beta blockers (lol)
- Ace inhibitors (pril)
- Diuretics (semide/thiazide)
- Hypoglycaemics (ide)
- NSAIDS (Naproxen, Aspirin, Celecoxib, Ibruprofren, Diclofenac ~NACID)
- Anti-malarials
- Penicillamine (RA tx)
- Gold salts (RA tx)
- Allopurinol (Gout tx)
- Methyldopa (hypertension tx)
State all key facts about oral contact hypersensitivity reactions to dental materials:
= A subgroup of oral lichenoid reactions
- Individual sensitised to a component of dental material:
o Amalgam alloy (nickel, mercury), gold, bis-GMA (resin).
- Lesion is confined to area of mucosa in direct contact with restoration
Amalgam contact hypersensitivity reactions (ACHRs):
o Lichenoid lesion localised to area of amalgam contact.
o Individual is patch test positive to ‘amalgam component’.
o Amalgam should be removed under rubber dam + restored with ‘inert’ material.
State management (i.e. diagnosis + tx) of lichenoid reactions:
Diagnosis:
1. Careful drug history and establish time relationship with starting the drug and appearance of lesions. (resolution once drug withdrawn)
- Consider patch testing if local causes
- Histological sample + analysis
Tx: Resolution of lesions should occur with withdrawal of drug.
Management as for LP until it resolves.
What would a histopathology report for a lichenoid reaction state? (5)
(remember similar to LP; hard to distinguish from histology)
o Infiltrate deeper + less well defined
o Large number of plasma cells + eosinophils (allergic reactions)
o Perivascular infiltrate
o Colloid bodies in the epithelium
o Basal cell destruction + basal cell autoantibodies
What is SLE and DLE?
SLE = systemic lupus erythematosus, while DLE= discoid lupus erythematosus.
Both are types of LUPUS, an autoimmune disease that causes inflammation.
SLE= affects many organs (multi-system disease that affects vascular + connective tissues).
DLE= mainly affects the skin
What is lupus? (general definition)
chronic cutaneous + oral disorder resulting in scaly skin patches in sun
exposed areas + lichen planus like oral lesions.
Who does lupus affect?
- Adults (women childbearing age)
- F>M
- 20-50% cases present with oral lesions
What is the aetiology of lupus?
Autoimmune disorder, precipitated by:
o Drugs
o Environment
o Hormones
o Viral factors
* Autoantibodies against normal cellular components e.g. nuclei
What is the oral presentation of lupus?
- Lesions occur BILATERALLY on labial, buccal, or alveolar mucosa + vermillion border (lesions on palate rarely seen)
- Erythematous areas surrounded by border of fine white striae (less well-
demarcated than OLP but similar) - Lesions ulcerate (active cases/ in those that are developing SLE)
What cutaneous features can be observed in Lupus?
- Found in areas of exposed to sunlight.
- One or more oval plaques appear on face, scalp, or hands.
- Lesions well demarcated, red, atrophic, scaly, and show keratin plugs in
dilated follicles, generalised telangiectasia. - Scarring results in alopecia on scalp and pigmentation
What is the management (diagnosis + tx) of Lupus?
Diagnosis:
1. Biopsy
2. Autoantibodies (blood test)
Post biopsy, what would the histopathology show for Lupus diagnosis?
Histology shows:
o Parakeratosis or orthokeratosis
o Degeneration of basal layer
o Hyalinization of sub epithelial connective tissue
o Chronic inflammatory cell infiltration of the sub epithelial connective tissue!
o Irregular pattern of acanthosis
o Colloid bodies present!
o Keratotic plugging present!
Tx:
* Chloroquine and potent topical steroids
* SPF 50
* Drugs include – dapsone, cytotoxic drugs, thalidomide, and retinoids.
What is Chronic Graft Versus Host Disease? (cGVHD)
= Complication following allogeneic bone marrow transplantation within 6-24m of BMT
- Involves immunological reaction of graft lymphocytes against recipient host cells
Who is affected by GVHD?
- Older recipients/donors
- Poorly matched graOs
What is the clinical presentation of GVHD? (including oral)
- Involves eyes, mouth, skin + liver
- Burning oral discomfort/ asymptomatic
- If lesions present can be reticular, erosive, or ulcerative
- Oral dryness may follow salivary gland involvement (superficial palatal + labial mucoceles)
- Reduced oral opening post development of sclerotic form of cGVHD
State tx for GVHD:
Topical analgesic- lidocaine, benzydamine
Corticosteroid preparations- betamethasone m/w
Tracolimus ointment
Note: sig. increased risk of OSCC development - regular specialist monitoring! (as part of tx plan)
What is a Mucocutaneous disease?
Mucocutaneous diseases are conditions that affect the mucous membranes and skin
What is the definition of Lichen Planus?
- chronic inflammatory condition that affects mucous membranes inside your mouth
-premalignant condition - spectrum of oral, cutaneous, + genital disease
Who does Lichen Planus affect?
40-80 years old
F>M
Can affect children
Europeans, Indians, Chinese, Malay
What is the aetiology of Lichen Planus?
Immunologically mediated reaction where cytotoxic T cells target basal keratinocytes (autoimmune)
Idiopathic disease triggered by stress, spiced foods, T2DM, liver disease (related)
What is the IO presentation of Lichen Planus? (for varied time ~mean= 7yrs)
Where? buccal/ labial mucosa, tongue, gingiva (very rarely palatal or lingual)
Lesions often in areas of increased friction = Koebner phenomenon e.g. occlusal line
OFTEN SYMMETRICAL/ mirror image presentation (BILATERAL)
Asymptomatic
Pain/discomfort when eating spicy/acidic/citrus foods or when brushing teeth
Desquamative gingivitis
(Pt might be concerned re appearance if gingiva + lips involved!)
(CHECK DRUG HISTORY ALWAYS)
What are the 7 different morphological variants/ presentations of lichen planus?
PAPREBC
Papular
Atrophic (areas of erythematous lesion surrounded by reticular components)
Plaque-like
Reticular (fine white striae cross each other in the lesion)
Erosive (ulcerative)
Bullous
Circinate
–> can present w/ several forms at once/variants can coexist
What are the extra oral presentations of lichen planus?
- Scarring alopecia
- Occular, nasal, laryngeal, oesophageal
- Cutaneous (skin) - purple polygonal, pruritic papules, koebner phenomenon, dystrophic nails, Wickham’s striae
- Genital involvement (F>M) -
vulvovaginal -gingival syndrome (ulcerative, symptomatic, progressive vulval disease)
State the management for LP:
a) diagnosis?
Incisional Biopsy (taken from the edge to include both affected and healthy tissue)
Histology would show?
- sub-epithelial band of inflammatory cells (lymphocyte dominant)
- basal cell liquefaction (destruction of basement membrane)
- +/- hyperkeratosis (thick scarring)
- Saw tooth rete ridges in epidermis
AI:
Band-like lymphocytic infiltrate at the epithelial-connective tissue interface.
Basal cell liquefaction degeneration.
No significant cellular atypia (dysplasia absent).
Lichenoid Dysplasia (LD) (Lichenoid Lesion with Dysplasia)
🚨 Potentially premalignant condition with features of OLP but also epithelial dysplasia.
State the management for LP:
b) tx aims for LP?
Tx aims:
- realistic (non-curative approach)
- Reduction of pain + inflammation
- Prevention of complications
State the management for LP:
b) INITIAL TX OF ASYMPTOMATIC LP?
- Reassure pt
- Encourage pt to regularly attend so you can monitor
- Encourage pt to monitor disease themselves (pictures etc)
- Maintain good OH (including smoking + alcohol cessation advice)
- Raise awareness about EO lesions developing
- Provide information leaflet (+conselling i
MUST:
- inform pt OLP is pre-cancerous condition with 0.5%-2% risk of malignant transformation
- Transformation is independent of smoking + alcohol exposure
State the management for LP:
c) INITIAL TX OF SYMPTOMATIC LP?
1) Eliminate provoking factors
- eliminate mechanical trauma i.e. sharp cusps, rough restorations, ill-fitting prostheses
- reduce chemical irritation i.e. acidic, spicy foods/beverages
2) Reduce bacterial plaque accumulation + enforce OH measure i.e. alcohol free chlorhaxdine m/w, smoking/alcohol cessation
3) Eliminate SLS (skin irritant that may alter taste, can exacerbate recurrent oral ulcers, toxic effect on epithelial cells causing sloughing of gingiva.
Therefore, recommend SLS free toothpaste in patient’s with desquamative gingivitis + soaps for cutaneous management.
State the management for LP:
d) PHARMACOLOGICAL TX OF SYMPTOMATIC LP?
Summary:
1. Difflam benzydamine (NSAID)
2. Topical + systemic corticosteroids
3. Topical (calcineurin inhibitors) or systemic immunosuppressants
State the management for LP:
d) DETAILED PHARMACOLOGICAL TX OF SYMPTOMATIC LP?
1st line tx = DIFFLAM benzydamine (NSAID)
2nd line tx= topical corticosteroid betamethasone m/w or hydrocortisone ointment for skin
3rd line tx= systemic corticosteroids
e.g:
* Beclometasone inhaler (corticosteroid)
* Prednisolone (corticosteroid)
* Dapsone (steroid- sparing antibiotic)
* Azathioprine (steroid-sparing
immunosuppressant)
* Ciclosporin (steroid-sparing immunosuppressant)
–>Risks of corticosteroids – suppression of HPA axis
4th lin tx= triorasol m/w (betamethasone, doxycycline, nystatin)
5th line tx= tacrolimus ointment (Calcineurin inhibitor -immunosuppresant)
–> (Calcineurin is a protein in the synthesis of IL-2 which is pivotal in T-cell activation)
–> Tacrolimus -potential cancer risk when used on skin (therefore assess pt, not really used)
Which morphological variants for OLP have increased chance of malignant transformation?
Erosive + Atrophic Type
What is the risk of malignant transformation of OLP?
~1% chance over a 10-year period
What are the differentials for OLP?
- Lichenoid reactions
- Dysplasia (SCC)
- Discoid lupus erythematosus (DLE)
- Graft versus host reaction (GVHD)
- Desquamative gingivitis
- Pemphigus vulgaris
- Mucous membrane pemphigoid
- Leukoplakia
- Leukoedema
- Frictional Keratosis
