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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
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
31
What are the Oral Features of HBID?
* **Thick White Lesions** * *Like in WSN and Pacyonychia Congenita*
32
What is the Clinical Presentation of Dyskeratosis Congenita?
* **Skin and Nail Pigmentation Changes** * *like in Pachyonchia Congenita* * **Pancytopenia - Marrow Failure** * **​**Shortens Lifespan to age 30
33
What are the Oral Features of Dyskeratosis Congenita?
* **Widespread Red and White** oral lesions * Transform into **SCCA at Early Age**
34
What is the treatment for Dyskeratosis Congenita?
**Bone Marrow Transplant** * Won't prevent transformation into SCCA * Marrow Failure is the biggest cause of Death
35
What are the Clinical Features of Xeroderma Pigmentosum? (3)
* **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**
36
What are the Clinical Features of Fanconi Anemia? (4)
* **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
What is the Clinical Features of Keratosis Follicularis (Darier Disease)?
* **Multiple Itchy, Foul-Smelling, Red Papules** all over **Trunk**
38
What is the Oral Feature of Keratosis Follicularis?
* 50% of pts have intraoral lesions on **Hard Palate** * If they wear a denture it looks like Papillary Hyperplasia clinically
39
What is the tx for Keratosis Follicularis?
**Vitamin A Analogues** to make lesions go away
40
What happens in Simplex Epidermolysis Bullosa?
* **Mild Form** of EB * Bullae form at sites of **skin trauma** - frictional blister
41
What are the Clinical Features of Recessive Dystrophic Epidermolysis Bullosa? (6)
* 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
What is Junctional Epidermolysis Bullosa?
**Fatal at Birth** Sloughing of all skin during birth
43
What population does Lichen Planus affect?
2% of **Women \> 40 yrs**
44
What is the Classic Feature of Lichen Planus?
**Wickham Striae** crisscrossed, by a fine, lacelike network of white lines
45
What is the Characteristic Skin Lesion associated with Lichen Planus?
**Itchy, Pink, Violaceous**, **Scaly Papules** and **Flat Rhomboid Plaques** on the **Flexor Surfaces** of **Wrists** and **Ankles**
46
What is the most classic LP form?
**Reticular LP** * Wickham Striae of bilateral buccal mucosa * Asymptomatic
47
What is the Presentation of Atrophic LP?
* **White Striae** on background of **Red, Peeling Atrophic Mucosa** * Typically on **Gingiva** as **Red** and **Shiny** * *Atrophic Desquamative Gingivitis*
48
What is the most common oral lesion of LP?
Erosive (ulcerative) LP
49
What is the Clinical Presentation of Erosive LP?
* Atrophic LP, but with **Peeling** or **Well-Demarcated** **Serpiginous Ulcers** * Wavy like a Snake * Look horrible
50
What is the Clinical Presentation of the Oral Lesions of Plaque-Like LP?
* **Flat, white plaques** with **Fissures** * Mostly on **Dorsal Tongue**, it loses surface papilla
51
What is the Clinical Differential Diagnosis of Lichen Planus? (7)
* Dysplasia, PVL, SCC * Pemphigoid * Lupus * Graft vs. Host Ds * Candidiasis * Cinnamon Stomatitis * Rxn to Dental Restorations
52
What are the favored locations of LP?
* Bilateral * Buccal Mucosa * Lateral and Dorsal Tongue * Gingiva
53
What is the histology of Lichen Planus?
* Hyperkeratosis * **Saw-Tooth Rete Ridges** * Linear infiltrate of chronic inflammatory cells (pure lymphocytes) that follow epithelium * Basal Cells show **Liquefaction Necrosis** * **Civatte Bodies**
54
What are the Lichenoid Lesions? (5)
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
What are the Skin Lesions of Erythema Multiforme? (3)
* **Target/Bull's Eye Lesion** * Palmar and Plantar Lesions not causing Hyperkeratosis * Rashes
56
What are the Clinical Features of EM Minor?
* **Skin Lesions** with or without Oral Lesions * **Confluent Oral Slough** * RARE on Gingiva and Hard Palate * **Bloody Crusty Ulcers** of the **Lips**
57
What is the Clinical Presentation of EM Major?
* Adds 2 Mucosal Sites * **Conjunctiva** and/or **Genital** * Already Skin and Oral
58
In what population does SJS occur?
Children
59
What is the Characteristic Feature of SJS?
* **Sloughing Lesions of Skin** in **\< 10% of body** with **Oral, Ocular,** and **Genital Lesions** * *EM can occur with or without oral lesions*
60
What is the most severe expression of SJS?
Lyle Disease (Toxic Epidermal Necrolysis)
61
What is the Clinical Appearance of Lyle Disease?
* **\> 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
What is the tx for SJS?
* **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
Where are the lesions of BMMP (Pemphigoid) ?
* Oral, Conjunctival, Genital, **Esophagus, Larynx** * **​**added 2 lesions * Occasionally can be seen on skin
64
What is the appearance of BMMP Oral Lesions?
* Most often without other lesions * **Buccal Gingiva** * Red, shiny, peels off, bleeds * Sensitivity to spicy foods
65
What population does BMMP most affect?
Women \> 50
66
What is the diagnostic histology of BMMP?
* **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
In what 2 diseases can you get a Positive Nikolsky Sign?
Pemphigus BMMP
68
What are the characteristics of Cicatricial Pemphigoid?
* 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
What is the Differential Diagnosis of BMMP?
* Lichen Planus * Pemphigus Vulgaris * Hypersensitivity Rxn/Hormonal
70
What is the histology of Pemphigus Vulgaris?
* **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
What is a the clinical feature of Pemphigus Vulgaris?
* **Oral bullae precede skin lesions** in **50%** * Ultimately **develop in 100%**
72
What is the Tx for Pemphigus Vulgaris?
Fatal Disease * Aggresively with long term high does steroid and steroid sparing agents * Tx early before skin lesions develop
73
What is Paraneoplastic Pemphigus?
* **Severe Form** of Acute Onset Pemphigus * Resembles SJS * Bloody, Crusty Lip and Oral Lesions * **Blood Drawn** to establish Diagnosis
74
What population is most affected in Lupus?
**Women** *BMMP and LP*
75
What are the Features of Systemic Lupus Erythematosus (SLE)?
* Affects skin, oral mucosa, **all vessels, kidney, heart** * Eventually **Fatal** w/o Tx * *Pemphigus is also Fatal*
76
What are the features of Discoid Lupus Erythematosus?
* Affects **Skin and Mucosa ONLY** * Not fatal
77
What is the Skin Lesions of Lupus Erythematosus?
* **Butterfly Rash** induced by sun exposure * Bridge of nose
78
What are the Oral Lesions of Lupus Erythematosus?
* **Lichenoid Lesions** * Palate, vermillion border, and buccal mucosa
79
What is the Histology of Lupus?
* 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
What is the Treatment of Lupus?
* Avoid sun exposure * Topical steroids * **Antimalarials (plaquenil)** * **​**can cause intraoral pigmentation
81
What population does Scleroderma occur in?
**Adult Women** *BMMP, LP, Lupus*
82
What is the Clinical Presentation of Scleroderma?
* 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
What is the treatment of Sclerderma?
* No effective tx * Progressive and **Fatal** in 2-12 years
84
What are the oral findings of Scleroderma?
* **Microstomia** * Gingival Recession * **Widening of PDL** around **ALL Teeth** * **Resorption of Posterior Ramus, Coronoid and Condyle**
85
What population is affected with CREST sx?
**Women \> 50 yrs** *BMMP, LP, Lupus, Scleroderma*
86
How does CREST sx differ from Scleroderma?
* No kidney, heart, vessel or lung involvement * NOT FATAL
87
What occurs in CREST Sx?
* Calcinosis Cutis * Raynud * Esophageal Dysfunction * Sclerodactyly * Telangiectatic Mats
88
What do the Skin and Oral lesions of Graft vs. Host ds resemble?
* LP, Lupus, Scleroderma * Striae are finer and closer together

Oral Pathology Exam 1 (8 decks)

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