6. Mucocutaneous Disorders Flashcards Preview

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Flashcards in 6. Mucocutaneous Disorders Deck (88)
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1

What is the etiology of Ectodermal Dysplasia?

X-Linked Recessive

2

What is the etiology of Pachyonychia Congenita?

Autosomal Dominant 

mutation of keratin genes 

3

What is the etiology of White Sponge Nevus?

Autosomal Dominant 

mutation in keratin genes

4

What is the etiology of Hereditary Benign Intraepithelial Dyskeratosis (HBID)?

Autosomal Dominant

Tri-racial isolate from North Carolina 

5

What is the etiology of Dyskeratosis Congenita? 

X-linked Recessive 

Impared telomerase

6

What is the etiology of Xeroderma Pigmentosum?

Autosomal Recessive

disorder of chromosomal repair - epithelium can't repair UV damage 

7

What is the etiology of Fanconi Anemia?

Autosomal Recessive

disorder of chromosomal repair 

8

What is the etiology of Keratosis Follicularis (Darier Disease)?

Autosomal Dominant 

Defective cohesion of keratinized cells 

9

What is the etiology of Epidermolysis Bullosa?

Genetic disorder

epithelial attachment disorders of keratin, desomosomes, or collagen of CT

10

What is the etiology of Lichen Planus?

Unknown

Type IV Cytotoxic Rxn 

T8-cells + lymphocytes attack basal cells of skin/mucosa, finding them antigenic

 

 

11

What is the Pathogenesis of Lichenoid Lesions?

Medications cause an antigenic change in the epithelium evoking a T-cell rxn 

 

12

What Classes of Drugs Cause LP (Lichenoid Drug Rxn)?

  • Anti-Hypertensives 
    • EXCEPT Ca2+ Channel Blockers
  • Beta Blockers
  • ACE Inhibitors
  • NSAIDS

13

What is the Pathogenesis of Erythema Multiforme?

Acute Type IV Cytotoxic Hypersensitivity Rxn

acute = triggered by something

14

What are the common triggers of Erythema Multiforme?

  • Herpes
  • URI (mycoplasma pneumonia)
  • Medications (antibiotics)

15

What triggers Stevens-Johnson Syndrome?

Medication 

16

What is the Pathogenesis of Benign Mucous Membrane Pemphigoid?

Autoimmune Disease 

Igs made against basement membrane 

17

What is the Pathogenesis of Pemphigus Vulgaris?

Type 2 Autoimmune Ds

Antibody is produced against intercellular bridges 

Attacks desmogleins 

18

What is the Pathogenesis of Paraneoplastic Pemphigus?

Internal Malignancy 

lymphoma or leukemia

19

What is the Pathogenesis of Lupus Erythematosus?

 Type III Hypersensitivity

immune complex triggers tisue destruction 

20

What is the Pathogenesis of Scleroderma?

Autoimmune

Continual deposition of collagen throughout the body

21

What is the Pathogenesis of Graft vs. Host Ds?

Graft T-cells react against host HLA antigens

22

What is the Clinical Presentation of Ectodermal Dysplasia?

  • Defect of Skin and Oral Adnexal Structures
    • No Sweat Glands
    • No Sebaceous Glands
    • Sparse Blonde Hair
    • Few Teeth (peg shaped)
    • Hypoplastic or Missing Salivary Glands
      • Xerostomia
      • URI Infections 
  • Depressed Midface, Frontal Bossing, Protuberent Lips 

23

What is the Clinical Presentation of Pachyonychia Congenita?

  • Thick Keratin under Finger Nails 
    • Pushes the nail bed up and loses nails
  • Palmar and Plantar Hyperkeratosis 

24

What are the Oral Features of Pachyonychia Congenita?

  • Diffuse White oral lesions primarily on dorsal tongue, lateral tongue, buccal mucosa
    • Not premalignant 
    • In a young persion, it is there their entire lives 
    • Some family members may have it 

25

What is the Histology of Pachyonychia Congenita?

Hyperparakeratosis and Acanthosis with clear perinuclear spaces 

26

What are the Oral Features of White Sponge Nevus?

  • Thick white plaques, throughout oral mucosa 
    • Particularly buccal mucosa 

27

What is the Histology of White Sponge Nevus?

  • Hyperparakeratosis and Acanthosis with "Fried Egg Cells"
    • ​​Clear keratinocytes with pink condensed cytoplasm around nucleus 

28

What is the big difference between Pachyonychia Congenita and White Sponge Nevus?

White Sponge Nevus does NOT affect the Skin 

  • PC has nail lesions, palmar and plantar keratosis
  • Both:
    • ​Autosomal Dominant 
    • Mutation in Keratin genes
    • Diffuse thick white plaques of oral (buccal) mucosa 
    • Totally Benign 

29

What is the histology of Hereditary Benign Intraepithelial Dyskeratosis (HBID)?

  • Hyperparakeratosi and acanthosis with Dyskeratosis 

30

What is the Clinical Presentation fo HBID?

  • Conjunctival gelatinous plaques that arise each Spring that cause temporary blindness, but then these plaques fall off