Facial Pain, OFG, Vesiculobullous Diseases Flashcards
What is Oral Dysaesthesia known as?
Burning Mouth Syndrome
C/O: burning pain, dry mouth, tingling, altered taste.
Differential diagnosis?
oral dysaesthesia/burning mouth syndrome
What is burning mouth syndrome?
Oral dysaesthesia.
Burning mucosal pain with no clinical signs.
Management of oral dysaesthesia/bms?
drugs
Anxyiolytic-based medications:
1. Nortriptyline
2. Mirtazepine
3. Vortioxetine
Neuropathic medication:
1. Gabapentine/Pregabalin
2. Topical Clonazepam
What is oral dysaesthesia/bms usually associated with?
haematinic deficiency
What are the clinical features of oral dysaesthesia/BMS?
- usually anterior two-thirds of tongue
- spontaneous onset
- lasts from months to several years
- altered taste (dysguesia) / metallic
- tingling
- GI problems
- dry mouth
What is Persistent Idiopathic Facial Pain (PIFP)?
Persistent facial and/or oral pain, with varying presentations but recurring daily for >2 hours per day over >3 months, in the absence of cliical neurological deficit.
How is PIFP managed?
persistent idiopathic facial pain
- believe the patient
- multidisciplinary approach
- patient education (discourage from further invasive interventions aimed at pain relief in the absennce of a clear associated pathology e.g. tooth extractions)
What are the clinical features of PIFP?
persistent idiopathic facial pain
- usually deep, but can be superficial
- poorly localised, radiating, and mostly unilateral
- aching, burning, throbbing, stabbing pain
- persistent, long lasting (years) daily pain
C/O: unilateral pain, poorly localised and radiating. Aching, burning, throbbing, stabbing pain. Persistent and ongoing everyday for years.
Had took taken out but still pain.
Differential diagnosis?
Persistent Idiopathic Facial Pain (PIFP)
C/O: pain in face, head and neck that is worse in mornings/evenings + parafunctional clenching.
Differential diagnosis?
TMD pain
Management of TMD Pain?
- patient education/self-help advice
- physical therapy (CBT education and exercises. Soft diet and analgesia. Bite splint)
- biochemical manipulation (tricyclic)
- physiotherapy
- acupuncture
- clinical psychology
How would you investigate TMD pain?
Imaging:
* ultrasound scan - to assess functional visualisation of disc movement
* OPT/CBCT - to assess bone (if bony problem suspected)
* MRI (best image of disc)
Arthroscopy - to directly visualise the disc
How to assess TMJ?
clinical examination
Assess:
* muscles of mastications (any tenderness)
* sternomastoid and trapezius muscles (any tenderness)
* palpate TMJ (any tenderness)
* mouth opening (range of motion and deviation)
* TMJ clicking/crepitus
* dental occlusion
What is the difference between TMJ clicking and crepitus?
Clicking suggests disc displacement, while crepitus suggests a degenerative problem.
C/O: sharp, shooting pain on one side of the face.
Differential diagnosis?
Trigeminal Neuralgia
What are the causes of trigeminal neuralgia?
- idiopathic
- vascular compression of trigeminal nerve
- multiple sclerosis
- space-occupying lesion
- skull-base bone deformity
- connective tissue diseSe
- arteriovenous malformation
What are the clinical features of TN?
trigeminal neuralgia
- unilateral maxillary/mandibular division pain (> opthalmic division)
- stabbing pain
- 5-10 seconds duration
- triggers: cutaneous, wind/cold, touch, chewing
- sudden pain or concomitant continuous pain
General management of TN.
trigeminal neuralgia
medications
pain diary
LA
Surgical management of TN.
trigeminal neuralgia
**microvascular decompression (MVD)
**
destructive central procedures:
* balloon compression
* radiofrequency thermocoagulation
sterotactic radiosurgery (gamma knife)
destructive peripheral neurectomies
First-line drug therapy for TN.
trigeminal neuralgia
Carbamazepine
100mg tablets 2x daily for 10 days
Oxcarbamezapine
Lamotrigine
Carbamezapine is a first-line drug for trigeminal neuralgia.
What are the side effects of carbamezapine?
- blood dyscrasias (thrombocytopenia, neutropenia, pancytopania)
- electrolyte imbalance
- neurological deficits (paraesthesia, vestibular problems)
C/O:
orbital/temporal pain that is one-sided (unilateral). Rapid onset. Lasts for 15 mins to 3 hours. Feels like a migraine.
Assoc. symptoms: nausea, vomiting, photophobia, phonophobia.
Differential diagnosis?
Cluster headache
C/O:
orbital/temporal pain that is one-sided (unilateral). Rapid onset. Lasts for 2-30 mins. About 2-40 attacked per day.
Differential diagnosis?
Paroxysmal Hemicrania
Management of Cluster headache.
drug
Preventive:
* verapamil
* lithiun
* topiramate
Attack:
* subcutaneous sumatriptan 6mg or nasal zolmatriptan 5mg
* low O2 7-12 L/min via a non-rebreathing mask
Occipital lidocaine injection
Oral prednisolone
Management of paroxysmal hemicrania.
drug
indometacin (usually absolute response)
alternative: COX-II inhibitots, topiramate
What are the clinical features of Orofacial Granulomatosis (OFG)?
- perioral erythema
- perioral swelling
- lip swellinng (can cause lip fissures and dry, cracking lips)
- angular cheilitis
- proliferative erythematous gingivitis
- oedema of floor of mouth (staghorn sign)
- linear fissured ulcer in labial sulcus
What are the histological features of OFG?
orofacial granulomatosis
non-caseating granulomatous inflammation within the tissues.
giant cells obstructing lymphatics, no swelling.
C/O: swelling of the face, lips and oral tissues.
Differential diagnosis?
Orofacial granulomatosis
Crohn’s disease
Pathogenesis of OFG.
orofacial granulomatosis
blockage of lymphatic drainage due to immune reaction - type IV hypersensitivity
angio-oedema which settles down faster is a type I hypersensitivity
OFG symptoms are similar to that of Crohn’s disease.
How can you screen for Crohn’s disease?
crohn’s screening
- Parental awareness of importance of altered bowel habits of abdominal pain
- Growth and GI function monitoring
- Faecal calprotectin assay
Management of OFG.
initial management (primary care)
Identify possible cause of swelling. OFG or Crohns.
Monitor growth and GI symptoms. Faecal calprotectic assay.
Diet history for possible allergens.
3 months diet exclusion.
No benzoic acid, sorbic acid, cinnamon, chocolate, E10-E19
non-specialist treatment
Management of OFG in a specialist setting.
- Topical treatment to angular cheilitis/fissure (miconazole or hydrocortisone cream)
- Topical treatment to lip swelling or facial erythema (tacrolimus ointment 0.03%)
- Intralesional steroid injection (triamcinolone, weekly for 3 weeks)
- Pulsed azithromycin 3 months (3days/week)
- Prednisolone
- Azathioprine
Types of Pemphigus?
(5)
- Pemphigus Vulgaris (PV)
- Pemphigus Foliaceous
- Paraneoplastic pemphigus
- Drug-induced pemphigus
- IgA pemphigus
What is the type of pemphigus that causes oral lesions?
the common one
Pemphigus Vulgaris (PV)
What is the main antibody involved in PV?
pemphigus vulgaris
IgG polyclonal antibodies
What type of reaction is pemphigus?
hypersensitivity
Type II hypersensitivity immune reaction as it is antibody-mediated.
What are the main target antigens in PV?
pemphigus vulgaris
desmoglein3 (Dsg3)
desmoglein1 (Dsg1)
Which antigen gives rise to oral lesions in PV?
pemphigus vulgaris
desmoglein3 (Dsg3)
Which antigen gives rise to skin lesions in more severe cases of PV?
pemphigus vulgaris
desmoglein1 (Dsg1)
What is the difference between pemphigus vulgaris and pemphigoid?
PV: suprabasal clefts, intraepithelial clefts
Pemphigoid: sub-epithelial clefts
Pathogenesis of PV.
pemphigus vulgaris
IgG4 polyclonal antibodies attack Dsg3,
thus damaging desmosomes,
which results in the loss of cell-cell adhesion, leading to vesiculations, erosions, or ulcers.
How can PV be diagnosed?
Direct and Indirect immunofluorescnece.
Biopsy.
Test for presence of acantholytic cells.
Nikolsky sign (occasionally helpful).
What is the Nikolsky sign?
A somewhat helpful diagnostic test.
Rub mucosa to induce bulla.
What is Pemphigus?
A vesiculobullous disease.
Intraepithelial blisters, resulting in bullae that easily rupture (within hours), resulting in ulceration of mucosal and/or cutaneous sites (oral cavity, conjunctiva, genitalia and upper respiratory tract).
Histological features of PV.
pemphigus vulgaris
Acantholysis with intraepithelial bullae formation.
Serum IgG, IgM, sometimes IgA autoantibodies to Dsg.
Suprabasilar clefts annd separation of the epithelium from the basal cell layer.
Within the blister/bullae separated area, acantholytic cells present.
Basket-weave-like pattern on IF.
C/O:
erosions and ulcers in buccal mucosa, palate and lips.
slow-healing, no scarring.
severe desquamative or erosive gingivitis.
ulcers not well-defined.
persistent ulcers.
Differential diagnosis?
pemphigus vulgaris
Local management of PV.
pemphigus vulgaris
Betamethasone phosphate tablets 0.5mg
Dissolve in 10ml water. Hold 2mins. 4x/day
Prednisolone 5mg tablets
First-line management of PV. (systemic meds)
pemphigus vulgaris
oral prednisolone starting at 1mg/kg/day
(increase on 50-100% increments every 5-7 days if blistering continues)
consider pulsed IV corticosteroids.
assess osteoporosis risk.
corticosteroids + aduvant immunosuppressant (remission maintenance stage):
* azathioprinne 2-3mg/kg/day
* mycophenolate motefil 2-3g/day
* rituximab 1g x 2 infusions, 2 weeks apart
Second-line management of PV. (systemic meds)
pemphigus vulgaris
azathioprine
mycophenolate motefil
rituximab
Third-line management of PV. (systemic meds)
pemphigus vulgaris
methotrexate
IVIg
plasmaphoresis
C/O:
itchy, urticated erythematous rash,
blisters which appear weeks to months after initial puritis,
lesions are localised to limbs or may be generalised
oral lesions are mild, transient. lesions heal without scarring
Differential diagnosis?
Bullous Pemphigoid
What are the types of Pemphigoid?
(3)
- Bullous Pemphigoid
- Mucous Membrane Pemphigoid
- Cicatritial Pemphigoid
What are the main target antigens in bullous pemphigoid?
BP180 and BP230
How can BP be diagnnosed?
bulloud pemphigoid
biopsy
DIF
IIF
serology
clinical presentation
What are the histological features of BP?
bullous pemphigoid
subepidermal blister with an inflammatory infiltrate (lymphocytes and eosinophils).
DIF shows linear BMZ (basement membrane zone) deposition of IgG and/or complement.
Management of BP.
mild/localised:
* topical corticosteroids
moderate/severe:
* oral prednisolone with or without adjuvant therapy (immune modulating agents) (azathioprine, mycophenolate motefil).
steroid ± immune modulating agents
What is mucous membrane pemphigoid?
MMP
It is a rare type of pemphigoid,
which involves the oral mucosa, conunctiva (symblepharon - leading to impaired vision & blindness), genitalia, upper aerodigestive tract, some skin.
The oral mucosa is most frequently affected, especially the gingiva (desquamative gingivitis).
What is the main target antigen for MMP?
mucous membrane pemphigoid
BP180
What are the main antibodies for MMP?
mucous membrane pemphigoid
IgG and/or IgA autoantibodies
How is MMP diagnosed?
mucous membrane pemphigoid
postive DIF for IgG, IgA, IgM or C3 at BMZ
gold standard diagnostic test
What are the histological features of MMP?
mucous membrane pemphigoid
subepithelial cleft with mixed inflammatory infiltrate
DIF shows IgG, IgA, IgM and/or C3 at basement membrane
What is Erythema Multiforme?
EM
Target skin lesions, with oral or ocular involvement.
Can recur.
Associated with HSV infection or drugs.
EM is cytotoxic immunologic attack on keratinocytes exppressing non-self-antigenns due to HSV/drugs (allopuriol, antibiotics, anticonvulsant, NSAIDs).
What are the types of EM?
(4)
- EM minor (affects only 1 mucosa)
- EM major (affects 2 or more mucous membranes)
- Stevens-Johnson Syndrome (SJS) (extensively skin)
- Toxic Epidermal Necrolysis (TEN)
Which vesiculobullous disease can be triggered by a HSV infection?
EM type
Major/Minor EM
Which vesiculobullous disease can be triggered by drugs?
EM type
Stevens-Johnsonn Syndrome
Toxic Epidermal Necrolysis
C/O:
extensive painful erythmatous and ulcerative lesions of the mucous membranes that leaves scars,
irrefgular shallow ulcers and crusting of the lips
Differential diagnosis?
erythema multiforme
What is the pathogenesis of EM?
erythema multiforme
CD8+ T lymphocytes in epitheliium inducing apoptosis of scattered keratinocytes,
leading to satellite cell necrosis.
What are the histologival features of EM?
- lichenoic infiltrate inn basement membrane zone of epithelium.
- T lymphocytes and monoiuclar cells present in dermis and lamia propia and extend into epithelium.
- epithelium appears edematous and spongiotic.
- necrosis of basal and suprabasal epithelial ells, resulting in intra- and subepithelial bullae formation.
- IF shows granular staining for C3 at BMZ and occasionally within vessels or apoptotic keratocytes.
Management of EM.
erythema multiforme
urgent medical therapy:
* systemic steroids (up to 60mg/day)
* systemic aciclovir
encourage fluid intake
* may require admission for IV fluid if unable to drink
encourage analgesia
What is Angina Bullosa Haemorrhagica?
ABH
Benign subepithelial oral mucosal blistera filled with blood in the absence of an identifiable cause or systemic disorder.
What causes ABH?
angina bullosa haemorrhagica
trauma
long-term steroid inhaler use
zinc deficiency
hypertension
What are the clinical features of ABH?
angina bullosa haemorrhagica
- acute, sometimes painful oral blood-filled vesicles and bullae (not due to blood dyscrasia or other known causes)
- mainly soft palate affected (can develop on buccal mucosa, lip and lateral surface of tongue
- haemorrhagic bullae spontaneously burst after a short time resulting in ragged, painless, superficial erosions that heal spontaneously within 1 week without scarring
- intact bullae is red to purple in colour
- bulla 2-3cm in diameter
- lesions may recur
Management of ABH.
no treatment.
reassure patient disease is benign.
explain known triggers and course of disease.
What are the histologic features of ABH?
- parakeratotic epithelium with a subepithelial separation from the underlying lamina propria.
- bullae filled with erythrocytes and fibrin
- IF shows no IgG, IgM, IgA or C3 antibodies within basement membrane zone or epithelium
