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People with this disease present with heat intolerance, fine/sparse hair, periocular wrinkling, mid face hypoplasia, abnormal nails, ano or hypodontia, cone-shaped teeth, xerostomia
Ectodermal dysplasia
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Inherited disorder due to defect in keratin
Bilateral thickened, white corrugated plaques of the buccal mucosa
**Inherited so occurs earlier in life
White Sponge Nevus (Cannon's Disease)
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Inherited disorder of keratinization that affects a tri-racial isolate
Lesions develop during childhood
See thick, corrugated white plaques of the buccal and labial mucosa
also see thick gelatinous conjunctival plaques
Hereditary benign intraepithelial dyskeratosis (HBID)
*the eye lesions are what differentiate it from white sponge nevus
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X-linked recessive disorder that has oral lesions that progress: bullae-->erosions-->leukoplakia (premalignant)-->30% develop into SCCa
Dyskeratosis Congenita
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This disease presents as reticulated hyper pigmented skin lesions, dystrophic nails, 8% develop aplastic anemia
Dyskeratosis Congenita
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AR disease that results in a defect in DNA repair
Patients are extremely sensitive to UV damage and are at a greater increased risk of cutaneous basal cell carcinomas and squamous cell carcinomas
Xeroderma Pigmentosum
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High risk areas for damage done by Xeroderma Pigmentosum (2)
- lower lip
- tip of the tongue
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Inherited disease that results in mutated calcium levels
presents as erythematous, crusted skin papules, palmer and plantar keratosis, nail changes
(V shaped notches in nails are characteristic)
Flat-topped papules of hard palate and alveolar mucosa (“cobblestone”)
Darier' s Disease
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Gene mutations linked to abnormal blood vessel development
Develop vascular malformations: telangiectasias, Epistaxis, GI bleeding, Lung AVMs predispose to brain abscesses
Oral lesions may be the first sign
Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Syndrome)
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Disease that presents as benign tumors of the CNS, heart, kidney and skin
CNS: Intellectual disability (30%), seizures (80%)
Cardiac rhabdomyoma,
Renal angiomyolipoma
Tuberous Sclerosis
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What are the four most common skin lesions with tuberous sclerosis
- Facial angiofibromas
- Periungual fibromas
- Ash leaf spot
- Shagreen patch
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4 Oral findings of tuberous sclerosis
- Enamel pitting
- gingival fibrous papules
- Gingival enlargement (phenytoin/dilantin--antiseizure drugs)
- Bone fibrous proliferations
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Inherited disorder that comes with an increased risk of breast (50%), thyroid and GI cancers
Associated with facial trichilemmomonas and acral keratosis
Assosciated with pink papillose of gingiva, tongue and buccal mucoa
Multiple Hamartoma Syndrome (Cowden Syndrome)
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Benign condition of unknown cause
Multiple, demarcated, atrophic patches with slightly elevated yellowish-white scalloped margins (interrupted boarders are important features of geographic tongue)
may be associated with geographic tongue
Erythema Migrans (Geographic Tongue)
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Most common places to see geographic tongue (4)
- Lateral or ventral tongue
- Buccal mocosa
- FOM
- Labial mucosa
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Treatment for EM?
Topical steroids only if symptomatic (otherwise, nothing)
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Skin disease associated with
Increased proliferative activity of keratinocytes
Shows symmetrical, well-demarcted, erythematous plaques with silvery scales
Positive auspitz's sign
Psoriasis
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Oral lesions associated with this diseases present as the following:
uncommon and non-specific
activity must parallel that of the skin lesions (look very erythematous)
Psoriasis
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Most common "collagen vascular disease" in the US
Lupus Erythematosus
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Subset of LE that manifests as:
multisysem disease, photosensitive malar rash, and non-specific, lichenoid, ulcerative lesions and desquamative gingivitis
Systemic LE
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Subset of LE that manifests as:
lesions that are limited to the skin and mucosa
Oral lesions identical to erosive lichen planus
Chronic Cutaneous (discoid) LE
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Subset of LE that manifests as:
intermediate between SLE and discoid lupus
Skin lesions are the most prominent (less scarring)
oral lesions similar to discoid
May have arthritis and musculoskeletal involvement
Subacute Cutaneous LE
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Autoimmune disease characterized by dense deposition of collagen in various organs and tissues
Skin changes: raynaud's phenomenon, ulcerations of the fingertips, acro-osteolysis, sclerodactylyl, mask-liked pinched ale of nose
Scleroderma
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4 oral manifestations of scleroderma
- Microstomia
- Ankyloglossia
- Dysphagia
- Xerostomia
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Radiographic findings in which disease?
Diffuse widening of the PDL
Resorption of the posterior ramus of the mandible, coronoid, condyle
Individual tooth resorption
systemic sclerosis
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Crest syndrome is a mild variant of systemic sclerosis. What does CREST stand for?
- C: calcinosis cutis
- R: raynaud's syndrome
- E: esophageal strictures
- S: sclerodactlyl
- T: telangiectasia
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