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Oral Pathology Exam 1 > 6. Mucocutaneous Disorders > Flashcards

6. Mucocutaneous Disorders Flashcards

1
Q

What is the etiology of Ectodermal Dysplasia?

A

X-Linked Recessive

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2
Q

What is the etiology of Pachyonychia Congenita?

A

Autosomal Dominant

mutation of keratin genes

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3
Q

What is the etiology of White Sponge Nevus?

A

Autosomal Dominant

mutation in keratin genes

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4
Q

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

A

Autosomal Dominant

Tri-racial isolate from North Carolina

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5
Q

What is the etiology of Dyskeratosis Congenita?

A

X-linked Recessive

Impared telomerase

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6
Q

What is the etiology of Xeroderma Pigmentosum?

A

Autosomal Recessive

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

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7
Q

What is the etiology of Fanconi Anemia?

A

Autosomal Recessive

disorder of chromosomal repair

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8
Q

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

A

Autosomal Dominant

Defective cohesion of keratinized cells

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9
Q

What is the etiology of Epidermolysis Bullosa?

A

Genetic disorder

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

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10
Q

What is the etiology of Lichen Planus?

A

Unknown

Type IV Cytotoxic Rxn

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

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11
Q

What is the Pathogenesis of Lichenoid Lesions?

A

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

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12
Q

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

A
  • Anti-Hypertensives
    • EXCEPT Ca2+ Channel Blockers
  • Beta Blockers
  • ACE Inhibitors
  • NSAIDS
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13
Q

What is the Pathogenesis of Erythema Multiforme?

A

Acute Type IV Cytotoxic Hypersensitivity Rxn

acute = triggered by something

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14
Q

What are the common triggers of Erythema Multiforme?

A
  • Herpes
  • URI (mycoplasma pneumonia)
  • Medications (antibiotics)
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15
Q

What triggers Stevens-Johnson Syndrome?

A

Medication

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16
Q

What is the Pathogenesis of Benign Mucous Membrane Pemphigoid?

A

Autoimmune Disease

Igs made against basement membrane

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17
Q

What is the Pathogenesis of Pemphigus Vulgaris?

A

Type 2 Autoimmune Ds

Antibody is produced against intercellular bridges

Attacks desmogleins

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18
Q

What is the Pathogenesis of Paraneoplastic Pemphigus?

A

Internal Malignancy

lymphoma or leukemia

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19
Q

What is the Pathogenesis of Lupus Erythematosus?

A

Type III Hypersensitivity

immune complex triggers tisue destruction

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20
Q

What is the Pathogenesis of Scleroderma?

A

Autoimmune

Continual deposition of collagen throughout the body

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21
Q

What is the Pathogenesis of Graft vs. Host Ds?

A

Graft T-cells react against host HLA antigens

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22
Q

What is the Clinical Presentation of Ectodermal Dysplasia?

A
  • 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
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23
Q

What is the Clinical Presentation of Pachyonychia Congenita?

A
  • Thick Keratin under Finger Nails
    • Pushes the nail bed up and loses nails
  • Palmar and Plantar Hyperkeratosis
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24
Q

What are the Oral Features of Pachyonychia Congenita?

A
  • 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
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25
Q

What is the Histology of Pachyonychia Congenita?

A

Hyperparakeratosis and Acanthosis with clear perinuclear spaces

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26
Q

What are the Oral Features of White Sponge Nevus?

A
  • Thick white plaques, throughout oral mucosa
    • Particularly buccal mucosa
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27
Q

What is the Histology of White Sponge Nevus?

A
  • Hyperparakeratosis and Acanthosis with “Fried Egg Cells”
    • ​​Clear keratinocytes with pink condensed cytoplasm around nucleus
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28
Q

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

A

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
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29
Q

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

A
  • Hyperparakeratosi and acanthosis with Dyskeratosis
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30
Q

What is the Clinical Presentation fo HBID?

A
  • Conjunctival gelatinous plaques that arise each Spring that cause temporary blindness, but then these plaques fall off
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31
Q

What are the Oral Features of HBID?

A
  • Thick White Lesions
    • Like in WSN and Pacyonychia Congenita
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32
Q

What is the Clinical Presentation of Dyskeratosis Congenita?

A
  • Skin and Nail Pigmentation Changes
    • like in Pachyonchia Congenita
  • Pancytopenia - Marrow Failure
    • ​Shortens Lifespan to age 30
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33
Q

What are the Oral Features of Dyskeratosis Congenita?

A
  • Widespread Red and White oral lesions
    • Transform into SCCA at Early Age
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34
Q

What is the treatment for Dyskeratosis Congenita?

A

Bone Marrow Transplant

  • Won’t prevent transformation into SCCA
  • Marrow Failure is the biggest cause of Death
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35
Q

What are the Clinical Features of Xeroderma Pigmentosum? (3)

A
  • Widespread Skin Atrophy and blotchy pigment/depigmentation
  • Multiple Sun-induced Cancers by age 20
    • Lip and Tongue Cancer due to UV light
    • Melanoma, SCC, BCC
  • Most die by age 30
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36
Q

What are the Clinical Features of Fanconi Anemia? (4)

A
  • Aplastic Anemia, Leukemia
    • Dysfunctional Marrow
  • Widespread Oral Lesions that transform to SCC at Early Age
    • Die by age 25
  • Microstomia
  • Disorders of Thumb and Radius
37
Q

What is the Clinical Features of Keratosis Follicularis (Darier Disease)?

A
  • Multiple Itchy, Foul-Smelling, Red Papules all over Trunk
38
Q

What is the Oral Feature of Keratosis Follicularis?

A
  • 50% of pts have intraoral lesions on Hard Palate
    • If they wear a denture it looks like Papillary Hyperplasia clinically
39
Q

What is the tx for Keratosis Follicularis?

A

Vitamin A Analogues to make lesions go away

40
Q

What happens in Simplex Epidermolysis Bullosa?

A
  • Mild Form of EB
  • Bullae form at sites of skin trauma - frictional blister
41
Q

What are the Clinical Features of Recessive Dystrophic Epidermolysis Bullosa? (6)

A
  • Terrible debilitating ds that Shortens Lifespan, with a high morbidity
  • Causes formation of bullae at points of very minor trauma
  • Repeated cycles of scaring often result in Microstomia
  • Mouth and Esophageal Scars are susceptible to SCCA
  • Fusion of fingers into a Mitten-like Deformity
  • Severe Enamel Hypoplasia
42
Q

What is Junctional Epidermolysis Bullosa?

A

Fatal at Birth

Sloughing of all skin during birth

43
Q

What population does Lichen Planus affect?

A

2% of Women > 40 yrs

44
Q

What is the Classic Feature of Lichen Planus?

A

Wickham Striae

crisscrossed, by a fine, lacelike network of white lines

45
Q

What is the Characteristic Skin Lesion associated with Lichen Planus?

A

Itchy, Pink, Violaceous, Scaly Papules and Flat Rhomboid Plaques on the Flexor Surfaces of Wrists and Ankles

46
Q

What is the most classic LP form?

A

Reticular LP

  • Wickham Striae of bilateral buccal mucosa
  • Asymptomatic
47
Q

What is the Presentation of Atrophic LP?

A
  • White Striae on background of Red, Peeling Atrophic Mucosa
  • Typically on Gingiva as Red and Shiny
    • Atrophic Desquamative Gingivitis
48
Q

What is the most common oral lesion of LP?

A

Erosive (ulcerative) LP

49
Q

What is the Clinical Presentation of Erosive LP?

A
  • Atrophic LP, but with Peeling or Well-Demarcated Serpiginous Ulcers
    • Wavy like a Snake
    • Look horrible
50
Q

What is the Clinical Presentation of the Oral Lesions of Plaque-Like LP?

A
  • Flat, white plaques with Fissures
  • Mostly on Dorsal Tongue, it loses surface papilla
51
Q

What is the Clinical Differential Diagnosis of Lichen Planus? (7)

A
  • Dysplasia, PVL, SCC
  • Pemphigoid
  • Lupus
  • Graft vs. Host Ds
  • Candidiasis
  • Cinnamon Stomatitis
  • Rxn to Dental Restorations
52
Q

What are the favored locations of LP?

A
  • Bilateral
  • Buccal Mucosa
  • Lateral and Dorsal Tongue
  • Gingiva
53
Q

What is the histology of Lichen Planus?

A
  • Hyperkeratosis
  • Saw-Tooth Rete Ridges
  • Linear infiltrate of chronic inflammatory cells (pure lymphocytes) that follow epithelium
  • Basal Cells show Liquefaction Necrosis
  • Civatte Bodies
54
Q

What are the Lichenoid Lesions? (5)

A

Resemble Atypical LP clinically or microscopically

  • Lichenoid Drug Rxn
    • ​Anti-Hypertensivs (except ca channel blockers)
    • Beta Blockers
    • ACE Inhibitors
    • ASAIDS
  • Contact Stomatitis
  • Lichenoid Dysplasia
    • Dysplasia originating in LP
    • Primary dysplasia that evokes a lichenoid rxn
  • Lupus Erythematosus
  • Graft vs. Host Disease
    • LP where donor lymphocytes attack host basal epithelium
55
Q

What are the Skin Lesions of Erythema Multiforme? (3)

A
  • Target/Bull’s Eye Lesion
  • Palmar and Plantar Lesions not causing Hyperkeratosis
  • Rashes
56
Q

What are the Clinical Features of EM Minor?

A
  • Skin Lesions with or without Oral Lesions
  • Confluent Oral Slough
    • RARE on Gingiva and Hard Palate
  • Bloody Crusty Ulcers of the Lips
57
Q

What is the Clinical Presentation of EM Major?

A
  • Adds 2 Mucosal Sites
    • Conjunctiva and/or Genital
  • Already Skin and Oral
58
Q

In what population does SJS occur?

59
Q

What is the Characteristic Feature of SJS?

A
  • Sloughing Lesions of Skin in < 10% of body with Oral, Ocular, and Genital Lesions
    • EM can occur with or without oral lesions
60
Q

What is the most severe expression of SJS?

A

Lyle Disease (Toxic Epidermal Necrolysis)

61
Q

What is the Clinical Appearance of Lyle Disease?

A
  • > 10% of body will blister and slough off
    • diffuse bullous skin lesions
  • 30% Fatal due to fluid electrolyte loss or secondary infection
  • Mostly Adults > 60 yrs
62
Q

What is the tx for SJS?

A
  • Avoid Steroids, once the skin slough off
    • Can use Steroids to tx EM Minor
  • Pooled Human Immunoglobulin
    • may block ligand that causes epidermal necrosis
  • Discontinue and Avoid Triggering Disease
63
Q

Where are the lesions of BMMP (Pemphigoid) ?

A
  • Oral, Conjunctival, Genital, Esophagus, Larynx
    • ​added 2 lesions
  • Occasionally can be seen on skin
64
Q

What is the appearance of BMMP Oral Lesions?

A
  • Most often without other lesions
  • Buccal Gingiva
    • Red, shiny, peels off, bleeds
    • Sensitivity to spicy foods
65
Q

What population does BMMP most affect?

A

Women > 50

66
Q

What is the diagnostic histology of BMMP?

A
  • Clean SUB-basilar separation of entire epithelium from CT, w/o underlying separation resulting in tense bulla and sloughing erosions
  • No inflammation
  • Immunofluorescence
    • linear band of IgG and C3 along BM zone
67
Q

In what 2 diseases can you get a Positive Nikolsky Sign?

A

Pemphigus

BMMP

68
Q

What are the characteristics of Cicatricial Pemphigoid?

A
  • Doesn’t happen in the oral cavity
  • When it gets in the Eye
    • Blisters and ulcers will heal with scarring that can cause blindness
  • If it occurs in the trachea, esophagus, genital mucosa it will lead to scaring and stricture
69
Q

What is the Differential Diagnosis of BMMP?

A
  • Lichen Planus
  • Pemphigus Vulgaris
  • Hypersensitivity Rxn/Hormonal
70
Q

What is the histology of Pemphigus Vulgaris?

A
  • Supra-basilar vesicles with acantholytic cells
    • basal cells stay attached to CT via hemidesmosomes
  • Positive Tzanck Test
  • Immunostain
    • IgG surrounding each individual epithelial cell
      • Fish-Netting Pattern
71
Q

What is a the clinical feature of Pemphigus Vulgaris?

A
  • Oral bullae precede skin lesions in 50%
  • Ultimately develop in 100%
72
Q

What is the Tx for Pemphigus Vulgaris?

A

Fatal Disease

  • Aggresively with long term high does steroid and steroid sparing agents
  • Tx early before skin lesions develop
73
Q

What is Paraneoplastic Pemphigus?

A
  • Severe Form of Acute Onset Pemphigus
  • Resembles SJS
    • Bloody, Crusty Lip and Oral Lesions
  • Blood Drawn to establish Diagnosis
74
Q

What population is most affected in Lupus?

A

Women

BMMP and LP

75
Q

What are the Features of Systemic Lupus Erythematosus (SLE)?

A
  • Affects skin, oral mucosa, all vessels, kidney, heart
  • Eventually Fatal w/o Tx
    • Pemphigus is also Fatal
76
Q

What are the features of Discoid Lupus Erythematosus?

A
  • Affects Skin and Mucosa ONLY
  • Not fatal
77
Q

What is the Skin Lesions of Lupus Erythematosus?

A
  • Butterfly Rash induced by sun exposure
    • Bridge of nose
78
Q

What are the Oral Lesions of Lupus Erythematosus?

A
  • Lichenoid Lesions
  • Palate, vermillion border, and buccal mucosa
79
Q

What is the Histology of Lupus?

A
  • Resembles LP, but add lymphocyte pervasculitis and salivary gland infiltrates
  • Liquefactive necrosis of basal cells
  • Lymphocytic infiltrates below epithelium
  • Hyperkeratosis
  • Edema
    • Less common in LP
  • Lupus Band Test
    • Inmmunofluorescence shows granular bands of Ig or C3 at the BM
80
Q

What is the Treatment of Lupus?

A
  • Avoid sun exposure
  • Topical steroids
  • Antimalarials (plaquenil)
    • ​can cause intraoral pigmentation
81
Q

What population does Scleroderma occur in?

A

Adult Women

BMMP, LP, Lupus

82
Q

What is the Clinical Presentation of Scleroderma?

A
  • Skin, esophagus, vessels, heart, lungs, and kidneys most affected with fibrosis
  • Mask-like Face
  • Sclerodactyly (claw hands)
  • Raynaud Phenomenon
    • Pain due to vascular consequences
83
Q

What is the treatment of Sclerderma?

A
  • No effective tx
  • Progressive and Fatal in 2-12 years
84
Q

What are the oral findings of Scleroderma?

A
  • Microstomia
  • Gingival Recession
  • Widening of PDL around ALL Teeth
  • Resorption of Posterior Ramus, Coronoid and Condyle
85
Q

What population is affected with CREST sx?

A

Women > 50 yrs

BMMP, LP, Lupus, Scleroderma

86
Q

How does CREST sx differ from Scleroderma?

A
  • No kidney, heart, vessel or lung involvement
    • NOT FATAL
87
Q

What occurs in CREST Sx?

A
  • Calcinosis Cutis
  • Raynud
  • Esophageal Dysfunction
  • Sclerodactyly
  • Telangiectatic Mats
88
Q

What do the Skin and Oral lesions of Graft vs. Host ds resemble?

A
  • LP, Lupus, Scleroderma
    • Striae are finer and closer together

Oral Pathology Exam 1 (8 decks)

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