Lecture 6 - White Lesions

Question 1

5 Reasons a lesion may appear white

Answer 1

1. Plaque covering mucosa 2. Hyperkeratosis (thick keratin layer) 3. Hyperplasia (thick spinous layer) 4. Fluid accumulation in epithelium 5. Epithelium alteration

Question 2

Three main groups of white lesions

Answer 2

Developmental Inflammatory Cancer / dysplasia

Question 3

Developmental White Lesions

Answer 3

Leukoedema White spongy nevus Dyskeratosis congenita

Question 4

Inflammatory White Lesions

Answer 4

Frictional Infectious Autoimmune Tobacco-related

Question 5

Cancerous or Dysplastic White Lesions

Answer 5

Dysplastic leukoplakia Verrucous leukoplakia Proliferative leukoplakia Squamous Cell Carcinoma

Question 6

Leukoedema

Answer 6

Opalescent, milky, white film that disappears when mucosa is stretched Usually asymptomatic BIlateral on buccal mucosa No tx needed More prevalent in blacks More severe in smokers

Question 7

Leukoedema Histologic Findings

Answer 7

Intracellular edema of spinous layer Acanthosis Parakeratosis Broad/elongated rete ridges

Question 8

White Spongy Nevus

Answer 8

Genetic skin disorder High penetrance, autosomal dominant Defect in normal keratinization of the epithelium Develops in childhood or adolescence Benign - no tx needed

Question 9

White Spongy Nevus Histologic Findings

Answer 9

Hyperparakeratosis Acanthosis Basketweave appearance Clearing of cytoplasm in spinous layer **Peri-nuclear eosinophilic condensation in superficial epithelium

Question 10

Hereditary benign intraepithelial dyskeratosis (Witkop - Von Sallmann syndrome)

Answer 10

Genetic, very rare, autosomal dominant Predominantly in tri-racial area of N. Carolina (Native American, white, black) Lesions develop in childhood No tx for oral plaques, refer to opthamologist

Question 11

White Spongy Nevus Appearance

Answer 11

Bilateral, symmetric lesions Thick, spongy texture Buccal mucosa, lip, alveolar ridge, soft palate, or floor of mouth

Question 12

Hereditary benign intraepithelial dyskeratosis (Witkop - Von Sallmann syndrome) Clinical Findings

Answer 12

Affects ORAL MUCOSA and CONJUNCTIVA of eyes Oral lesions: -Thick, corrugated, white plaques (similar to white spongy nevus) -Floor of mouth and lateral tongue Occular lesions: -Opaque, gelatinous plaques -Tearing, photophobia -Worse in spring -Can lead to blindness

Question 13

Hereditary benign intraepithelial dyskeratosis (Witkop - Von Sallmann syndrome) Histologic Findings

Answer 13

Hyperparakeratosis Acanthosis “Cell-within-a-cell” - dyskeratotic process

Question 14

Dyskeratosis Congenita Pathology

Answer 14

Mutation disrupts normal maintenance of telomerase (determines cellular longevity) Lesions may undergo malignant transformation (SCC) 70% of pts develop aplastic anemia

Question 15

Dyskeratosis Congenita Clinical Findings

Answer 15

Triad: -reticular skin hyperpigmentation -nail dystrophy -tongue/buccal mucosal bullae (-> pre-malignant/malignant leukoplakia) Can also see rapidly progressive perio disease or cognitive delays

Question 16

Dyskeratosis Congenita Histologic Findings

Answer 16

Oral lesions have hyperorthokeratosis with epithelia atrophy, which can progress into dysplasia and SCC

Question 17

Dyskeratosis Congenita Treatment

Answer 17

FATAL DISEASE Severely affect patients live ~32yrs Careful oral examinations for malignant transformation Careful monitoring for aplastic anemia (may need BM transplant)

Question 18

Pachyonychia Congenita Types

Answer 18

Jadassohn-Lewandowsky Type Jackson-Lawler Type

Question 19

Pachyonychia Congenita Jackson-Lawler Type

Answer 19

Nails dramatically affected No oral lesions Why do we need to know this? Seriously Mutated keratin 17 gene

Question 20

Pachyonychia Congenita

Answer 20

Rare autosomal dominant Less than 200 cases ever VERY VERY RARE you will never see this Why are we learning this? No tx, nails fall off

Question 21

Pachyonychia Congenita Jadassohn-Lewandowsky Type

Answer 21

Probably named after the only two people to have this Mutated keratin 16 gene Hyperkeratinaceous nails - pinched, tube-shaped, nail falls off Hyperkeratosis - callous-like palmar/plantar surfaces Hyperhidrosis (increased sweating) Painful blisters on soles of feet Oral lesions - thick, white plaques on tongue lateral margins and dorsal surface