Dermatological Diseases

  1. 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
  2. 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)
  3. 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
  4. X-linked recessive disorder that has oral lesions that progress: bullae-->erosions-->leukoplakia (premalignant)-->30% develop into SCCa
    Dyskeratosis Congenita
  5. This disease presents as reticulated hyper pigmented skin lesions, dystrophic nails, 8% develop aplastic anemia
    Dyskeratosis Congenita
  6. 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
  7. High risk areas for damage done by Xeroderma Pigmentosum (2)
    • lower lip
    • tip of the tongue
  8. 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
  9. 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)
  10. 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
  11. What are the four most common skin lesions with tuberous sclerosis
    • Facial angiofibromas
    • Periungual fibromas
    • Ash leaf spot 
    • Shagreen patch
  12. 4 Oral findings of tuberous sclerosis
    • Enamel pitting
    • gingival fibrous papules 
    • Gingival enlargement (phenytoin/dilantin--antiseizure drugs)
    • Bone fibrous proliferations
  13. 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)
  14. 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)
  15. Most common places to see geographic tongue (4)
    • Lateral or ventral tongue
    • Buccal mocosa
    • FOM
    • Labial mucosa
  16. Treatment for EM?
    Topical steroids only if symptomatic (otherwise, nothing)
  17. Skin disease associated with 
    Increased proliferative activity of keratinocytes
    Shows symmetrical, well-demarcted, erythematous plaques with silvery scales 
    Positive auspitz's sign
  18. 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)
  19. Most common "collagen vascular disease" in the US
    Lupus Erythematosus
  20. Subset of LE that manifests as: 
    multisysem disease, photosensitive malar rash,  and non-specific, lichenoid, ulcerative lesions and desquamative gingivitis
    Systemic LE
  21. 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
  22. 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
  23. 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
  24. 4 oral manifestations of scleroderma
    • Microstomia
    • Ankyloglossia
    • Dysphagia
    • Xerostomia
  25. 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
  26. 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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Card Set
Dermatological Diseases
Final Exam Material