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?

A

DIF

23
Q

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

A

IIF

24
Q

Management of Pemphigus involves?

A
Biopsy: Histopathnand DIF
Bloods: FBC, UE, Glucose, LFT, IMF
Autoimmune profile: IIF
TPMT for azothioprine
G6PD: for dapsone
25
Q

How can we manage pemphigus?

A

Acute control: topical and systemic steroids 40mg prednisalone daily
Manteance: topical and systemic steroids, Mycophenalte/AZTEC
IV Ig
Plasmapheresis

26
Q

What are the two variants of pepmphigoid?

A

Mucous membrane and bullous

27
Q

What are the features of MM pepmphigoid?

A

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
Q

What is the pathology of MMP?

A

IgM/IgG and C3 to BMZ and this leads to sub epithelial bullae

Targets to BP1 and 2 and laminin 5

29
Q

What are the features of Bullous pemphigoid?

A

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
Q

T/F

Bullous pemphigoid can occur secondary to drugs and UV light?

A

T

31
Q

How do we investigate pemphigoid?

A

Same is pemphigus but consider referral for

32
Q

How do we treat pemphigus?

A

Mild: topical steroids eg prednisalone and Immunosuppresants
Moderate: dapsone tetracycline and nicotinamide
Severe: dapsone

33
Q

What are poor prognostic indicators for pemphigoid?

A

Circulating antibodies
High titre antibodies
Multi site involvement
Dermal binding sera

34
Q

What is the demographics behind Dermatitis Herpetiformis?

A

It is rare and more common in males than females. Seen in 2nd - 3 rd decade

35
Q

What is the pathology relating to DH?

A

IgA mediated autoantibody destruction at basement membrane zone

36
Q

What is the aetiology of DH?

A

Associated with gluten sensitive enteropathy
Iodine allergy
HLAB8 and HLAD3

37
Q

How does DH present?

A

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
Q

What tests would you perform if you suspected DH?

A

Biopsy and direct IF

39
Q

What would the biopsy for DH show?

A

IgA at BM zone
Immune complexes
AB to reticulin and endomycin

40
Q

What does the direct immunofluorescence for DH show?

A

Granular IgA and C3 at tips of dermal papillae

41
Q

How do you treat DH?

A

Dapsone
Supra pyridine
Gluten free diet

42
Q

What is linear IgA disease?

A

This is a variant of DH where IgA is episodes along BM in a linear fashion

43
Q

How does linear IgA disease present?

A

Oral vesicles and ulcers

Buttock scalp lesions

Treatment: mycophenalate mofetil

44
Q

What is EM major?

A

Steven Johnson’s syndrome

45
Q

What are the causes of Steven Johnson’s syndrome ?

A

MIID
Malignancy
Infection: 30% associated with herpes, HIV and mycoplasma eg M pneumoniae
Drugs: PHABSS penicillin, hydantoins, anti malarial, barbiturates, salicylates, sulphonamide

46
Q

What are the features of EM?

A

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
Q

How often does EM attack?

A

10-14 days
Twice a year
For three years

48
Q

What are the signs of SJS?

A

Eyes: conjunctivtis, dry eyes, symblepheron
Stomatitis
Genital lesions: balanitis, urethritis, vulval ulceration
Skin: target lesions
Lung and renal involvement

49
Q

What is TEN?

A

Childhood variant of EM

50
Q

How do you diagnose EM?

A

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
Q

How do treat EM?

A
Biopsy 
Ophthalmology
Topical and systemic steroids
Immunosuppressant eg azothioprine 
Antiviral
52
Q

What are the complications of MMP?

A

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