Bdent 3 Pathology: Immunological Vesico-Bullous Diseases

Front 1

What are 5 Vesiculo-bullous Diseases?

Back 1

Pemphigus vulgaris
Allergic Stomatitis
Phemphigoid
Lichen Planus
Erythema multiforme

Front 2

What is the cause of Pemphigus Vulgaris?

Epidemiology?

Back 2

Autoantibodies destory desmosomes (bind desmoglein 3 & 1)

No sex prediliction, average age 50 years.

Front 3

What is the prognosis for Pemphigus Vulgaris?

Back 3

Fatal if not treated

Front 4

What does Pemphigus Vulgaris present with?

Back 4

50% present with oral lesions.

Ragged erosions and ulcerations on any mucosal surface. Intact oral blisters rarely seen.

Flaccid bullae on skin

Nikolsky sign (new or extended erosive lesion created when lateral pressure is applied to the skin near an existing lesion)

Front 5

What test is used to diagnose Pemphigus Vulgaris?

Back 5

Immunofluorescence studies - normal tissue adjacent to the the ulceration or erosion should be sampled

Front 6

What is the treatment for Pemphigus Vulgaris?

What is the prognosis?

Back 6

Systemic Corticosteroids, often with Azathioprine

Topical corticosteroidsd have little effect.

Mortality 50-90% without corticosteroid therapy

Today 5-10% mortality, usually due to complications of therapy

Front 7

What is the epidemiology for Cicatricial Pemphigoid?

Back 7

5x more common than Pemphigus, 3:2 female predilection, average 60 years old.

Front 8

Clinically what is seen in Cicatricial Pemphigoid?

Back 8

May affect any mucosal surface, occasionally skin

Scarring seen with conjuctival and cutaneous lesions

Desquamative gingivitis

May see intact blisters intraorally

Front 9

What ist he most significant aspect of Cicatricial Pemphigoid?

Back 9

Ocular involvement can lead to scarring blocking the glands that produce tear film = dry eye = keratinization of corneal epithelium = blindness

Front 10

What test is done to confirm the diagnosis and what will it show?

Back 10

Generous biopsy of peripheral lesion including normal mucosa.

Microscopic examination show subepithelial cleft formation - seperation of the epithelium from the CT at the basement membrane

Front 11

How should tissue be submitted if Ciatricial Pemphigoid is suspected?

Back 11

in Michel's solution and formalin

Front 12

If a Ciatrical Pemphigoid specimen is subjected to DIF or IIF what are the results?

Back 12

DIF = positive
IIF = negative

Front 13

What is the treatment of Ciatrical Pemphigoid?

Back 13

Oral lesions only = topical steroids, tetracycline/niacinamide or dapsone may be sufficient

Ocular involvement = systemic immunosuppressive therapy

Front 14

What is the prognosis for Ciatrical Pemphigoid?

Back 14

Rarely fatal, rarely undergoes spontaneous resolution.

Risk of blindness if untreated ocular disease

Front 15

What % of people with Bullous Pemphigoid have oral involvement?

Back 15

20%. Lesions are usually cutaneous.

Front 16

What is the tx for Bullous Pemphigoid?

Back 16

Similar to Ciatrical pemphigoid, but most cases of BP resolve spontaneously in 1-2 years.

Front 17

What are the path lab results for BP?

Back 17

Subepithelial cleft similare to cicatrical pemphigoid

Positive DIF and IIF

Front 18

What is the epidemiology of Cutaneous Lichen Planus?

Back 18

Affects adults, 30-60 years. Female prediliction

Front 19

What does Cutaneous Lichen Planus present with?

Back 19

Purple polygonal pruritic papules with Wickham's striae, usually on the Flexor surface of wrists, lumbar regions, shins

Front 20

What is the epideamiology of Oral Lichen Planus and where is it seen?

Back 20

Occurs alone or with skin lesions, mostly in adults, females 3:2.

Reticuar form most common, on Bilateral buccal mucosa, tongue, gingiva.

Front 21

What are the 2 types of Oral Lichen Planus?

Back 21

Reticular: interlacing white line
Dorsal tongue involvement, patchy keratosis and atrophy

Erosive: shallow ulcers, peripheral erythema and radiating white lines

Front 22

What is the histology of Oral Lichen Planus?

Back 22

Hyperkeratosis, alternating atrophy and thickening of spinous layer, absent or pointed rete ridges, degerneration of the basal cell layer and a band-like infiltrate of lymphocytes

Front 23

What is Oral Lichen Planus associated with?

Back 23

Candidiasis

Front 24

What is the management for Oral Lichen Planus?

Back 24

Reticular LP - no therapy
Erosive LP - stronger topical corticosteroids

Also tx associated candidiasis

Front 25

What is the prognosis of OLP?

Back 25

good

Front 26

Does Oral Lichen Planus have any malignant transfomation potential?

Back 26

Controversial. No molecular evidence, and poorly documented cases

Front 27

What is the Etiology of Erythema Multiforme?

Epidemiology?

Back 27

50% unknown
25% viral (herpes)
25% medication related

Young adult males

Front 28

What is Erythema Multiforme?

Back 28

An acute, self-limitng ulcerative disorder, probably immune-mediated

Front 29

What is the Spectrum of Disease in Erythema Multiforme?

Back 29

EM Minor - skin or mucosa only

EM Major - (Stevens-Johnson Syndrome) at least 2 mucosal sites plus skin involvement

Toxic epidermal necrolysis - (Lyell's Disease) Diffuse bullous involvement of skin/mucosa

Front 30

What are the clinical features of Erythema Multiforme?

Back 30

Hemorrhagic crusting of lips

Widespread oral ulcers with ragged margins

"Target" lesions of skin

Front 31

What does Erythema Multiforme show under the microscope?

Back 31

Keratinocyte destruction, subepithelial edema, mised inflammatory infiltrate, perivascular inflammation

Front 32

What is the tx for EM?

Back 32

Supportive care in mild cases (analgesics, soft diet, hydration)

Major - controversial. Corticosteroids usually given empirically.

TEN managed in burn unit with IV pooled human immunoglobin showing promise

Front 33

What is the prognosis for EM

Back 33

Good for mild to moderate cases.


Mortality 2-10% in major cases and 30% in Toxic Epidermal Necrolysis

Front 34

Is EM recurrent?

Back 34

Perhaps in autumn and spring