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VB Flashcards

0
Q

What are the causes of non specific blisters?

A 
BAAFI
Burns 
Amyloidosis
ABH
False : cyst, mucocele, abscess
Infection: herpes simplex, Zoster, coxsackie
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1
Q

What are the two broad categories for blisters?

A

Non specific

Specific

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2
Q

What are the causes of true blisters?

A

Primary
Secondary
Congenital

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3
Q

What are the causes of primary VB diseases?

A

Pepmphigus
Pepmphigoid
DH
Linear IgA disease

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4
Q

What are the causes if secondary VB?

A

EM, TEN, bullous diabeticorum,

Acute contact dermatitis

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5
Q

What are the congenital causes for VB diseases?

A

Epidermolysis bullosa: simplex junctional and dystrophic

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6
Q

Where are the sites of cleavage for blistering?

A

Corneal
Intra epidermal
Sub epidermal

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7
Q

What is pephigus?

A

Life threatening diseases which causes blistering of Mucocutaneous surfaces

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8
Q

What are the types of pemphigys?

A

Vulgaris which includes vegetans
Foliaceus which includes erythmatosus
Drug related: pencillin and rifampicin. Drugs that have sulphdryl groups
Para neoplastic: associated with underlying neoplasm known or occult

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9
Q

Which variant of pemphigus targets desmoglein 1 and 3 ?

A

Vulgaris
This is an AB mediated AI disease
The most severe form on pemphigus
Patient often starts with oral lesions and later develops skin lesions

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10
Q

Which variant of pemphigus targets desmoglein 1?

A

Foliaceus

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11
Q

The oral mucosa contains mainly which desmoglein?

A

3 hence why Vulgaris produces bad lesions in mourn as skin has 3 and 1

Antibodies against DSg3 will affect skin and mouth
Antibodies against DSG1 will affect mainly skin

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12
Q

T/F

Pemphigus is rare?

A

T

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13
Q

Which demographic is mainly affected by pemphigus?

A

Jewish and Italian

Female = Male

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14
Q

What is the aetiology of pemphigus?

A

HLA association however most cases are idiopathic including some triggers being medications, radiation, surgery, certain foods, emotional stress

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15
Q

Which version of pemphigus affects the whole width of epithelium?

A

Pemphigus Vulgaris

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16
Q

Which extra oral sites are affected in pemphigus?

A

Nose
Rectum
Scalp

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17
Q

What are the features of pemphigus?

A

Oral lesions: blisters which form erosion, desquammative gingivitis, and positive nikolsky sign
Skin lesion: these are common and large flaccid blisters form, eryhtmrstous apples and blisters, genital lesions, phaygrngea lesions

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18
Q

What is the pathology behind pemphigus?

A

Intercellular epithelial IgG against DSG 3 and 1and plakoglobin
Then forms intra epithelial bullae followed by acanthylosis which is intra epithelial splitting

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19
Q

What is the serology behind pemphigus?

A

Intercellular ABs to epithelium

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20
Q

What is the mortality of pemphigus if untreated?

A

30%

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21
Q

What investigations are carried out for diagnosis pemphigus?

A

Biopsy for histopathology and DIF

Bloods for IMF

22
Q

Which our of DIF and IIF is more sensitive?

23
Q

Which out of DIF and IIF tests for antibodies in serum?

24
Management of Pemphigus involves?
``` Biopsy: Histopathnand DIF Bloods: FBC, UE, Glucose, LFT, IMF Autoimmune profile: IIF TPMT for azothioprine G6PD: for dapsone ```
25
How can we manage pemphigus?
Acute control: topical and systemic steroids 40mg prednisalone daily Manteance: topical and systemic steroids, Mycophenalte/AZTEC IV Ig Plasmapheresis
26
What are the two variants of pepmphigoid?
Mucous membrane and bullous
27
What are the features of MM pepmphigoid?
Late middl aged affecting females more than makes Oral lesions: blood filled blisters which erode , desquammative gingivitis and possible nikolsky sign Genital mucosa: blisters Eyes: symblepheron Skin lesions: rare Usually a protracted course, lasts a long time
28
What is the pathology of MMP?
IgM/IgG and C3 to BMZ and this leads to sub epithelial bullae Targets to BP1 and 2 and laminin 5
29
What are the features of Bullous pemphigoid?
Seen mainly in elderly and incidence is equal between males and females Affects skin more than mucous membrane. Due fined course which generally lasts for 5 years Skin lesions: axilla, groin, flexures and abdomen Oral lesions are rare and seen in 30% of people: affects gingiva , buccal and palatal mucosa
30
T/F | Bullous pemphigoid can occur secondary to drugs and UV light?
T
31
How do we investigate pemphigoid?
Same is pemphigus but consider referral for
32
How do we treat pemphigus?
Mild: topical steroids eg prednisalone and Immunosuppresants Moderate: dapsone tetracycline and nicotinamide Severe: dapsone
33
What are poor prognostic indicators for pemphigoid?
Circulating antibodies High titre antibodies Multi site involvement Dermal binding sera
34
What is the demographics behind Dermatitis Herpetiformis?
It is rare and more common in males than females. Seen in 2nd - 3 rd decade
35
What is the pathology relating to DH?
IgA mediated autoantibody destruction at basement membrane zone
36
What is the aetiology of DH?
Associated with gluten sensitive enteropathy Iodine allergy HLAB8 and HLAD3
37
How does DH present?
Skin lesions: itchy rash on shoulders and pressure Oral: palate, gingivae, buccal mucosa It appears as erythmatous, purpuric, vesicular, ulcerative Desquammative gingivitis and possible enamel Hypoplasia
38
What tests would you perform if you suspected DH?
Biopsy and direct IF
39
What would the biopsy for DH show?
IgA at BM zone Immune complexes AB to reticulin and endomycin
40
What does the direct immunofluorescence for DH show?
Granular IgA and C3 at tips of dermal papillae
41
How do you treat DH?
Dapsone Supra pyridine Gluten free diet
42
What is linear IgA disease?
This is a variant of DH where IgA is episodes along BM in a linear fashion
43
How does linear IgA disease present?
Oral vesicles and ulcers Buttock scalp lesions Treatment: mycophenalate mofetil
44
What is EM major?
Steven Johnson's syndrome
45
What are the causes of Steven Johnson's syndrome ?
MIID Malignancy Infection: 30% associated with herpes, HIV and mycoplasma eg M pneumoniae Drugs: PHABSS penicillin, hydantoins, anti malarial, barbiturates, salicylates, sulphonamide
46
What are the features of EM?
Seen in young (male) adolescents Swollen blood Staines crusted lips In the mouth see macular rash, blisters and ulcers at anterior of mouth Can get eye lesions too
47
How often does EM attack?
10-14 days Twice a year For three years
48
What are the signs of SJS?
Eyes: conjunctivtis, dry eyes, symblepheron Stomatitis Genital lesions: balanitis, urethritis, vulval ulceration Skin: target lesions Lung and renal involvement
49
What is TEN?
Childhood variant of EM
50
How do you diagnose EM?
Clinically Histopath Virology since need to compare with herpetic gingivo Direct IMF: basement membrane shows C3 and fibrin at Basement membrane Perivascular: c3 fine IGM
51
How do treat EM?
``` Biopsy Ophthalmology Topical and systemic steroids Immunosuppressant eg azothioprine Antiviral ```
52
What are the complications of MMP?
Skin: blistering and ulceration Genital: blistering, ulceration adm scarring Pharyngeal involvement: blistering,ulcer and scar. Strictures can lead to dysphgia Oral: blistering and periodontal tissue loss due to inability to maintains OH Eyes: entropian: inversion of eyelash, blepharitis: sclera inflam and irrataation

Oral Med (31 decks)

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