lichen Planus and oral vesicullobullous disease

Question 1

what areas of the body can lichen planus effect?

Answer 1

• Skin
• Scalp
• Genital
• Oesophageal

Question 2

what is oral lichen planus (OLP)?

Answer 2

OLP is a cell mediated autoimmune condition which targets (oral) keratinocytes.

Question 3

what causes OLP?

Answer 3

Mostly idiopathic however aetiological factors may be:

drugs (drug induced OLL)

mercury( amalgam associated OLL).

Question 4

what is this?

Answer 4

signs of oral lichen planus

Question 5

what are the intraoral features of OLP?

Answer 5

o Keratosis: reticular (web like) / annular(ring like) / plaque like keratosis may be present.

o Desquamative Gingivitis o Areas of atrophic

inflamed oral mucosa

o Mucosal erosion or even frank ulceration

o Bullous lesions.

Question 6

what type of OLP is this?

Answer 6

Reticular

Question 7

what does this show?

Answer 7

Lichenoid drug reaction

Question 8

what are the potential complications of OLP?

Answer 8

Pain –quality of life, nutrition and oral hygiene measures

Periodontal attachment loss: this is an indirect effect when oral hygiene measures are compromised secondary to discomfort.

Malignancy risk: OLP is a potentially malignant lesion (transformation risk incidence of 1%)

Question 9

what are the differential diagnosis of OLP?

Answer 9

GVHD, HCV, Lupus

Question 10

what is GVHD?

Answer 10

Graft vs host disease. Where the graft which has been transplanted starts attacking the bodies own self tissues.

Question 11

what is this

Answer 11

Presentation of GVHD but may present similarly to OLP

Question 12

What is HCV?

Answer 12

Hep C. It may present similarly to OLP

Question 13

what does this show?

Answer 13

Both Lupus and OLP present identically even histopathologically they both have lichenoid appearances

Question 14

Incisional or punch biopsys are often taken of OLP. what would they show histopathologically?

Answer 14

evidence of basal membrane immune mediated damage

sub basal lymphocytic band

hyper/ hypokeratosis, although this may not be evident in ulcerated areas

Question 15

what other investigations can be done to diagnose OLP?

Answer 15

• DirectIF
• Epidermal patch testing
• HCV serology
• Autoimmune profile

Question 16

How do you manage OLP?

Answer 16

identify and eliminate the cause.

Remove amalgam if they are believed to be the cause

Drug lichnoid reactions- liase

Question 17

What topical steroids could be use?

Answer 17

Betnasol mouthwash

Barrier agents such as orobase which is a carboxymethylcellulose or hyaluronic based preparations such as Gelclair or Gengigel.

Chlorhexidene

Question 18

When should you refer?

Answer 18

In medium to severe cases which are unresponsive to first line therapy should be referred to an oral medicine specialist.

High Potency Topical Steroids such as Clobetasol Proprionate 0.05% ointment mixed on a 1:1 basis with Orabase

Topical Tacrolimus 0.01% ointment mixed on a 1:1 basis with Orabase

Question 19

what systemic therapy can be used for OLP?

Answer 19

Reducing course of Prednisolone eg.40 -30- 20-10 mgs reducing over a period of 1 month.

Long term Systemic Therapy for specialist use in recalcitrant OLP

Question 20

Should you review OLP?

Answer 20

Yes! Potentially malignant.

Need to involve other care teams if extra oral

Question 21

What are the two types of pemphigoid?

Answer 21

Bullous pemphigoid- usually just skin

Mucous membrane pemphigoid- Effects mucosa and +- skin

Question 22

what causes mmp?

Answer 22

t is an autoimmune disease with antibodies directed at antigen/s in epithelial basement membrane resulting in a split and blister formation at this level.

Specifically BP 180 at the basement membrane

Question 23

How does MMP present?

Answer 23

Desquamative gingivitis

Can be oral, pharyngeal and genital.

Question 24

what is this?

Answer 24

The presentation of MMP

Question 25

what are the intra oral findings with MMP?

Answer 25

Mucosal inflammation Mucosal erosion and frank ulceration Blister formation Desquamative gingivitis Scarring

Question 26

what does this histopathology slide show?

Answer 26

Pemphigoid ( blister at the basement membrane\_

Question 27

what technique is this and what does it show?

Answer 27

Direct immunofluoresence

Question 28

how does indirect immunofluoresence differ from direct?

Answer 28

indirect uses a blood sample.

Question 29

what are the complications of mmp?

Answer 29

Skin involvement: blistering, ulceration Genital involvement: blistering, ulceration and scarring leading deformities Pharayngeal involvement: blistering, ulceration and scarring →stricture formation and dysphagia Oral involvement: blistering, ulceration and periodontal tissue loss ( secondary to inability to maintain adequate oral hygiene)

Question 30

what are the complications of MMP in the eye?

Answer 30

Tethering of conjunctiva to sclera (symblepharon) Inversion of eyelashes (entropion) Scleral irritation and inflammation (blepharitis).

Question 31

what are the topical treatments for mmp?

Answer 31

topical steroid- dermovate topical immunosuppressants

Question 32

what systemic treatment is given?

Answer 32

Steroids- prednisolone sliding dose- ( decreases as time goes on)

Question 33

Give an example of a systemic immunosuppressant?

Answer 33

Mycophenolate Mofetil ## Footnote Inhibits the synthesis of the purine nucleotide guanine Inhibits the proliferation of B and T cells in response to antigen stimulation

Question 34

what is pemphigus vulgaris?

Answer 34

Autoimmune Disease Antibody mediated Target: desmosomal proteins(DSG1,3) PV often starts with oral lesions with later skin involvement Complications of disease and therapy

Question 35

what causes PV?

Answer 35

Antibodies are directed against: – Desmoglein (DSG) 1(mainly expressed in oral mucosal desmosomes – DSG 3 which is mainly expressed in skin desmosomes.

Question 36

what are the intraoral findings of PV?

Answer 36

– Mucosal erosion and frank ulceration even including hard and soft palate – Blisters: typically burst – Desquamative gingivitis

Question 37

what are the Extra oral findings of PV?

Answer 37

Skin lesions: Itchy erythematous papules and blisters » Genital lesions and soreness, scarring » Pharyngeal lesions, possibly with scarring and dysphagia

Question 38

what does this slide show?

Answer 38

Pemphigus

Question 39

what does this direct immunoflouresence show?

Answer 39

pemphigus

Question 40

How do you treat and manage Pv?

Answer 40

Acute control: systemic & topical steroids Maintenance-topical & systemic steroids & MMF/ Azathiaprine mulitdisiplinary care!!

Question 41

what drugs can induce pemphigus oral ulceration?

Answer 41

induced by drugs with a sulphydryl group such as captopril

Question 42

what is paraneoplastic pemphigus?

Answer 42

the pemphigus related oral ulceration but with associated malignancy!

Question 43

what are the complications of PV?

Answer 43

**Skin involvement:** blistering, ulceration, fluid loss- shock – death. **Genital involvement:** blistering, ulceration **Pharayngeal involvement**: blistering, ulceration and scarring. **Oral involvement**: blistering, ulceration . Loss of periodontal tissues (secondary to inability to maintain adequate oral hygiene)

Question 44

what are the other vesiculobullousdisorders which have oral manifestations?

Answer 44

* Dermatitis Herpetiformis * EpidermolysisBullosa Acquisita * ErythemaMultiforme * Viral infections can cause blisters !!