Skin and Mucosal Disease 2

Question 1

What are the 2 stains/analyzing tools used for biopsies to distinguish between pemphigus, pemphigoid, and lichen planus?

Answer 1

• Half formalin-fixed for H&E

* Half frozen for direct immunofluorescence (DIF) ->Michel’s solution

Question 2

________ immunofluorescence is a biopsy

• Performed on the patient’s tissue (biopsy specimen) using an anti-human antibody panel

Answer 2

• Direct immunofluorescence

Question 3

______ immunofluorescence is a serological test

• Performed on animal tissue (esophagus) using the patient’s serum antibodies

Answer 3

• Indirect immunofluorescence

Question 4

Histopathology of \_\_\_\_\_\_\_
•Hyperkeratosis
•Saw tooth rete ridges -> elongated
•Band- like infiltrate of lymphocytes
•Liquefaction degeneration of basal cell layer

Answer 4

Lichen Planus

Question 5

Does pemphigus destroy desmosomes or hemidesmosomes?

Answer 5

Desmosomes

Question 6

Does pemphigoid destroy desmosomes or hemidesmosomes?

Answer 6

hemi desmosomes

Question 7

Which disease shows a fish net pattern on DIF?

Answer 7

Pemphigus

Question 8

Which disease shows an intraepithelial split?

Answer 8

Pemphigus

Question 9

Which disease shows a sub epithetlial split?

Answer 9

Pemphigoid

Question 10

Which disease shows a dark green band at basement zone on DIF?

Answer 10

Pemphigoid

Question 11

An immunologically mediated self limited
mucocutaneous disease
Young adults
Sudden onset of widespread painful superficial
mucosal ulcers and target lesions of skin
Crusted ulcers on lips
May be recurrent

Answer 11

Erythema multiforme

Question 12

Which disease shows bullseye/target/iris lesions?

Answer 12

erythema multiforme

Question 13

Which type of hypersensitivity occurs in erythema multiforme?

Answer 13

Type 3

Question 14

•Antibody response to an exogenous antigen produces circulating
immune complexes which are deposited in vessels

Answer 14

Type III Hypersensitivity Response seen in EM

Question 15

The following are associated with \_\_\_\_\_\_:
Drugs
• Sulfonamides
• Penicillin
• Diphenylhydantoin
• Tetracycline
• Codeine
• Ibuprofen
• ~ 30 others
Bugs
• Herpes simplex
• Mycoplasma pneumoniae
• Epstein Barr Virus
• Hepatitis B
• Streptococci
Other
• Tumors (Leukemia, Lymphoma)
• Immunization (DPT)
• Inflammatory bowel disease 
• Sarcoidosis
• Sunlight

Answer 15

EM

Question 16

• Prodromal symptoms
• Sudden onset of 
mucocutaneous lesions
• Self-limiting disease
• Lasts 2-6 weeks
• Avoid dehydration
• Recurrent episodes
• Lesions may be limited 
to oral mucosa

Answer 16

Erythema Multiforme

Question 17

•Stevens-Johnson Syndrome, a severe form of erythema multiforme, usually triggered by a drug, rather than an infection

Answer 17

Erythema Multiforme Major

Question 18

• Most severe form of erythema multiforme, usually seen in older individuals, almost always triggered by drug exposure
• Diffuse sloughing of epidermis leading to a scalded appearance, fluid loss and infection
• Treated as severe burn victims

Answer 18

Toxic Epidermal Necrolysis (Lyell Disease)

Question 19

• A multisustem autoimmune disease most common in adult women in child-bearing years
• Autoantibodies - anti-nuclear antibodies (ANA)
• Immune complexes are deposited throughout the body, especially kidney and blood vessels

Answer 19

Lupus Erythematosus

Question 20

____ lupus erythematous
• Skin
• No systemic involvement

Answer 20

Discoid LE

Question 21

____ lupus erythematous
• Skin
• Limited systemic involvement

Answer 21

Subacute cutaneous LE

Question 22

____ lupus erythematous
• Systemic involvement
• Periodic skin rashes

Answer 22

Systemic LE

Question 23

• Chronic, limited to skin and mucosa
• Sun-exposed skin
• Scaly, erythematous patches
• Cosmetic problems
• Cutaneous atrophy
• Scarring
• Pigmentation

Answer 23

Discoid Lupus Erythematosus

Question 24

Oral Mucosal Lesions in \_\_\_\_\_\_\_\_\_
•May be seen in any form of disease
•Red and white lesions that 
may be clinically identical to 
erosive lichen planus

Answer 24

Lupus Erythematosus

Question 25

* Most common form of lupus * Highest morbidity * Kidney involvement –glomerulonephritis - renal failure * Butterfly rash 50% - malar area and nose, aggravated by sunlight * Libman-Sacks endocarditis * Arthritis, arthralgia, heart and lung involvement, anemia, bone marrow depression, vasculitis, skin rashes

Answer 25

Systemic Lupus Erythematosus SLE

Question 26

Tx of ________: • Systemic corticosteroids • Antimalarial drugs - Plaquenil • Avoid excessive exposure to sunlight • Prognosis depends on organs affected and disease activity • Renal failure most frequent cause of death

Answer 26

Systemic lupus ery

Question 27

* An autoimmune disease of adults, predominately females, characterized by excessive fibrosis * May be limited to the skin or be widespread affecting various organ systems * May be associated with other autoimmune diseases

Answer 27

Systemic Sclerosis

Question 28

* Slow continuous replacement of loose fibrovascular connective tissue with dense collagen * Fibrosis causes loss of mobility and altered function of organs - skin, esophagus, salivary glands, kidneys, lungs, heart, muscle

Answer 28

Systemic sclerosis

Question 29

``` Oral manifestations of _______ •Microstomia •Xerostomia •Generalized widening of PDL space •Mandibular resorption ```

Answer 29

Systemic scleorsis

Question 30

Mask-like face •Telangiectasia •Raynaud phenomenon

Answer 30

Systemic sclerosis

Question 31

``` • Arterial spasm in response to cold or emotional stress • Pallor, cyanosis and then erythema • Numbness, tingling or pain on recovery. ```

Answer 31

Raynaud Phenomenon

Question 32

``` Hands and Fingers •Fibrosis, stiffness, deformity •Ischemia, atrophy and ulceration ```

Answer 32

Systemic sclerosis

Question 33

What makes up CREST syndrome seen in systemic sclerosis?

Answer 33

``` • Calcinosis • Raynaud’s Phenomenon • Esophageal dysfunction • Sclerodactly • Telangiectasia ```

Question 34

• A group of mechanobullous diseases involving structural defects in the attachment apparatus • Many hereditary forms involving different defects in the attachment apparatus • Recessive dystrophic form is most severe, involving defective type VII collagen anchoring fibrils • Not an autoimmune disease. Defective structural proteins

Answer 34

Hereditary | Epidermolysis Bullosa

Question 35

• Primarily a severe and debilitating skin disease in which blisters form at sites of minor trauma and may heal with scarring (Nikolsky + )

Answer 35

Hereditary Epidermolysis Bullosa

Question 36

Oral Sequalae of _________ •Normal diet produces bullae that heal with scarring leading to obliteration of vestibule, ankyloglossia, microstomia and esophageal stricture -Can have enamel hypoplasia in junctional type

Answer 36

Epidermolysis bullosa

Question 37

What condition does this person have? • My hands have been like this as long as I can remember • I lost all my fingernails by age 2 and they fused into this fist shape • I have never had hand surgery because I have chosen not to • I have a lot of thumb space still free, meaning my thumbs have not fused, so I can do things such as write, type etc. with no problem

Answer 37

Epidermolysis Bullosa –Recessive Dystrophic

Question 38

* A common, chronic, genetically-determined, inflammatory and hyperproliferative skin disease * Exacerbations and remissions * Association with certain HLA types * Immunoregulatory disorder –T cells trigger inflammation * Defect in control of keratinocyte proliferation -turnover rate 8x normal

Answer 38

Psoriasis

Question 39

* Well-demarcated, red plaques covered by silvery scales * Auspitz Sign - removal of scale leaves pinpoint bleeding area * Koebner Phenomenon - lesions develop following trauma to normal-appearing skin

Answer 39

Psoriasis

Question 40

•Removal of scale leaves small pinpoint bleeding -associated with psoriasis

Answer 40

Auspitz Sign

Question 41

Development of lesions following trauma to normal- appearing skin -associated with psoriasis

Answer 41

Koebner Phenomenon

Question 42

Histopathology of ______ | Intra-epithelial microabscesses

Answer 42

Psoriasis

Question 43

What condition presents as the same lesion intra-orally as psoriasis?

Answer 43

Erythema migrans

Question 44

``` Treatment of _______ •Topical medications - corticosteroids •Phototherapy - UVB •Photochemotherapy –PUVA (psoralen and UVA) ```

Answer 44

Psoriasis

Question 45

•Hereditary, autosomal dominant mutation of keratin genes that requires no treatment

Answer 45

White Sponge | Nevus

Question 46

• Asymptomatic, bilateral, white lesions with a thick, folded, consistency “spongy” appear before puberty • Primarily involves buccal mucosa, but may affect other mucosal surfaces –anogenital, esophageal

Answer 46

White sponge nevus

Question 47

Histopath of ______: | Perinuclear keratin condensation

Answer 47

White Sponge Nevus