Dermatology Flashcards
White Sponge Nevus: Etiology
Inherited condition due to mutations in keratin 4 and keratin 13
White Sponge Nevus: Inheritance Pattern
Autosomal dominant
White Sponge Nevus: When do signs appear?
Childhood to adolescence
Clinical Appearance of White Sponge Nevus
Symmetrical, thickened, white, corrugated/velvety, diffuse plaques
Location of White Sponge Nevus
Most often affecting the bilateral buccal mucosa; can be found at other intra-or extraoral sites
Symptoms of White Sponge Nevus
Asymptomatic
Clinical Differential Diagnosis for White Sponge Nevus
White sponge nevus, morsicatio buccarum, hereditary benign intrepithelial dyskeratosis, leukoedema
Treatment/Prognosis for White Sponge Nevus
Benign condition that does not require treatment
Keratoacathoma: Describe
Self-limiting epithelial proliferation on the skin.
Keratoacanthoma: Causes
Sun damage, HPV 26/37, trauma, immunosupression, tar exposure
What is Keratoacathoma mistaken for? Why?
Mistaken for SCC both clinically and histologically but is BENIGN
Population affected by keratoacathoma
> 45 years old; male predilection
Location affected by keratoacathoma
95% occurs on sun-exposed skin; 8% found on outer edge of vermillion boarder
Clinical Apperance of Keratocathoma
Presents as firm, non-tender, well-demarcated, sessile, dome-shaped nodule with central keratin plug
Growth Pattern of Keratoacanthoma
Rapid growth occurs to 1-2 cm in 6 weeks
Stabilizes in size at 6 weeks
Involutes in size at 6 weeks
Treatment for Keratoacanthoma
Surgical excision due to similarity with SCC, cyrotherapy or ablative techniques are typically recommended
Recurrence rate for Keratoacahtoma
4-8% recurrence after excision
Seborrheic Keratosis: Describe
Benign proliferation of the epidermal basal cells.
Location of Seborrheic Keratosis; where does it not occur?
More common on sun exposed skin
Does not occur in mouth
When does Seborrheic Keratosis appear?
Begins to develop in 30’s and are more prevalent with increasing age
Clinical Appearance of Seborrheic Keratosis
Sharply demarcated pigmented lesion; “stuck on” appearance
How does a seborrheic keratosis start out?
As a flat lesion and gradually enlarges and elevates
Saborrheic Keratosis Surface Texture
Fissured, pitted, verrucous, or smoth
Treatment For Seborrheic Keratosis
Benign lesion, consider referral to dermatologist for further evaluation and potential biopsy if showing any concerning characteristics
ABCDE (Signs concerning for melanoma)
Deratosis Papulosa Nigra: Describe Clinical Appearance and location
Multiple small dark papules scarred peri-orbital and zygomatic region
Dermatosis Papulosa Nigra: When do these lesions develop?
Begin to develop at adolescence
Dermatosis Papulosa Nigra: Inheritance pattern
Autosomal dominant inheritance
Population affected by Dermatosis Papulosa Nigra
Most commonly affects black population
Treatment/Prognosis for Dermatosis Papulosa Nigra
Benign condition; no treatment indicated
Leser Trelat Sign: Define
Paraneoplastic cutaneous marker of internal malignancy with the hallmark finding being an abrupt eruption of multiple seborrheic keratoses
Symptoms & TIme frame for Leser-Trelat Sign
Arise in a short period of time and are associated with pruiritus (itching)
Leser-Trelat Sign is associated with
Internal Malignancy
How does Leser-Trelat Sign resolve?
Treatment of underlying malignancy
Melasma: AKA
Mask of Pregnancy
Melasma: Describe
Acquired, symmetrical hyperpigmentation of sun-exposed skin of the face and neck
Cause of Melasma
UV light exposure and hormonal influences (hence association with pregnancy)
Can Melasma occur in males?
Yes but it less common
Population affected by Melasma
Mainly affects medium/dark complexioned patients
Clinical Features of Melasma
Brown or gray macules
Sites affected by Melasma
Midface, forehead, upper lip, chin, mandibular ramus region, rarely arms
Treatment for Melasma
Treatment unnecessary but may be desired for esthetics
What are some esthetic treatment options for melasma?
First line is combination cream
Laser therapy, light therapy, microdermabrasion
Avoidance of UV, sunscreen
Lesions may resolve after parturition
Potential for malignant potential of melasma
No malignant potential
Other names for actinic lentigo
Age spot, liver spot, solar lentigo
Describe actinic lentigo and what causes it
Pigmented lesion of the skin associated with UV exposure
Population affected by actinic lentigo
Typically seen in older adults
Most commonly affects fair complected individuals
What part of the body does actinic lentigo not occur?
The mouth
Actinic lentigo: Clinical Features
Uniformly pigmented macule well-demarcated, but irregular borders
Common sites of actinic lentigo
Face, dorsal of hand, shoulders, upper back
Actinic Lentigo: Treatment and Prognosis
-Benign lesion that does not show malignant transformation
-Can be removed or treated for esthetic reasons
-Sunscreen for prevention
Developmental types of nevi
Nevus Flammeus
Acquired types of nevi
Mole
Three less common types of nevus
Halo Nevus
Spitz Nevus
Blue Nevus
Define Halo Nevus
Nevus with a strong inflammatory reaction
Define Spitz Nevus
Primarily occur in children, histologically can be mistaken for melanoma
Define Blue Nevus
Nevus with a blue hue
Nevus Flammeus is better known as
Port wine stain
Define Nevus Flammeus
Congential dermal capillary vascular malformation
How does the nevus flammeus occur?
Lesions may occur independently or as a component of Sturge-Weber Syndrome
Sturge Weber Syndrome is _______ condition
Developmental condition; not hereditary
Sturge-Weber Sydnrome affects what parts of the body?
Skin, brain and eyes
Sturge-Weber Syndrome Major Features
Convulsive disorder, intellectual disability, migraines, stroke-like episodes, glaucoma
Acquired Melanocytic Nevus: Cause
Acquired
When do acquired melanocytic nevus occur?
Not at birth, rarely begin to devolop over age 40 and show regression in later decades (5-7th decades)
Clinical features of acquired melanocytic nevus
-Well circumscribed macules, papules or nodules
-Almost always <1 cm in diameter
-Range in color from skin colored to pink, brown and black/blue
Melanocytic nevus that are greater than 1 cm could be what three things?
Congenital nevus, atypical nevus, or melanoma
Treatment for Acquired Melanocytic Nevus
About one third of melanomas develop from a pre-existing nevus, so clinicans should take care in examining any moles that a patient notes have changed
Common Premalignant and malignant skin lesions (4)
Cutaneous melanoma
Basal cell carcinoma
Actinic keratosis
Cutaneous SCC
“A” in Melanoma
Asymmetry due to its uncontrolled growth pattern
“B” in Melanoma
Border irregularity (often with notching)
“C” in Melanoma
Color variegation (which varies from shades of brown to black, white, red, and blue, depending on the amount and depth of melanin pigmentation)
“D” in Melanoma
Diameter greater than 6 mm (which is diameter of pencil eraser)
“E” in Melanoma
Evolving (lesions that have changed with respect to size, shape, color, surface, or symptoms over time”
Cause of Cutaneous Melanoma
May arise de novo or from pre-existing benign melanocytic lesion such as acquired nevus
Clinical Features of Cutaneous Melanoma
ABCDE
Treatment for Cutaneous Melanoma
-Based on Clark’s Classification and TMN staging
-Surgical excision
-Immunotherapy
-Tyrosine Kinase Inhibitors for lesions with KIT mutations
Basal Cell Carcinoma: Describe
Epithelial malignancy arising from the basal cell layer of the skin and its appendages
Most common skin cancer and most common of all cancers overall
Basal Cell Carcinoma
Location of Basal Cell Carcinoma
80% occur on the skin of the head and neck
Cause of Basal cell carcinoma
Cumulative UV exposure
Clinical Features of Basal Cell Carcinoma
Locally invasive, slowly spreading tumor
What are three types of clinical presentations for basal cell carcinoma?
- Papule with shiny surface, telangiectasia and central ulcerative necrosis
- Gray-black papule with erosion giving the appearance of a melanocytic lesion
- Erythematous poorly defined plaque with erosions
What are are basal cell carcinoma lesions in young patients associated with?
Nevoid basal cell carcinoma syndrome
Treatment for Basal cell carcinoma
Surgical excision (may or may not use Mohs), topical chemotherapeutic agents
Prognosis for basal cell carcinoma
Recurrence is uncommon
Metastasis is exceptionally rare
Actinic Keratosis: Describe
Precancerous lesion of the skin
Cause of Actinic Keratosis
UV Radiation
Radiation causes mutations in the p53 tumor suppressor gene
Actinic Keratosis is the skin equivalent of
Oral dysplasia
Clinical features of actinic keratosis
White/gray/brown/red
Irregular scaly patches, vary in color
Population affected by Actinic Keratosis
> 40 years of age, no gender predilection
Affects 50% of all light skinned adults
Describe sandpaper appearance of Actinic Keratosis
Roughened texture
Describe keratin horn appearance of actinic keratosis
Arising from central area
Histology of Actinic Keratosis
Some degree of epithelial dysplasia + solar elastosis
Treatment of Actinic Keratosis
Removal of lesion to prevent progression to SCC (cyrotherapy, curettage, excision, topical chemotherapeutic agents, laser excision or ablation)
How often does actinic keratosis undergo malignant transformation over 2 year period
10%
What are topical chemotherapeutic agents?
Efudex, Carac, Aldara
Cutaneous Squamous Cell Carcinoma: Describe
A malignant tumor of keratinocytes of the skin.
Cutaneous Squamous Cell Carcinoma: Cause
UV Exposure -> genetic mutations (particularly p53 tumor suppressor mutation)
Second most common skin cancer in the US
Cutaneous Squamous Cell Carcinoma
Clinical Features of Cutaneous Squamous Cell Carcinoma
Small, firm, dull-red nodule, +/- ulceration, frequently with elevated rolled borders
Histology of Cutaneous Squamous Cell Carcinoma
Same as oral SCC
Treatment of Cutaneous Squamous Cell Carcinoma
Depends on TNM staging -> surgical excision, radiation, topical agents
Ectodomeral Dysplasia: Define
Group of inherited disorders defined by the failure to develop 2 or more ectodermal derived anatomic structures
What structures are affected by ectodermal dysplasia?
Skin, hair, nails, teeth, sweat glands
What is the most well known ectodermal dyslpasia syndrome?
Hypohidrotic ectodermal syndrome
What type of inheritance causes ectodermal dysplasia?
X-linked inheritance
Describe lyon hypothesis when it comes to ectodermal dysplasia
Females have a lesser phenotype because half of the x chromosomes express the normal gene
How are eccrine glands affected in ectodermal dysplasia?
Reduced eccrine glands causing heat intolerance
How is hair, nails, skin and eyes affected in ectodermal dysplasia?
Fine sparse hair, fine wrinkling, and hyperpigmentation around the eyes, dystrophic/brittle nails
Midlface ____ occurs in ectodermal dysplasia?
Midface hypoplasia
How are salivary glands affected by ectodermal dysplasia?
Hypoplastic or absent salivary glands
How are teeth affected in ectodermal dysplasia?
Oligo or hypodontia -> reduced number of teeth
Incisor crowns: tapered or conical, pointed
Molars: reduced in diameter
Rarely no teeth
Treatment for Ectodermal dysplasia
Replacement of missing teeth, increased preventative care for xerostomia related caries
Peutz Jeghers Syndrome: Inheritance pattern
Autosomal dominant inherited condition with an STK11 Mutation
Key features of Peutz Jeghers Syndrome
Freckle-like lesions of the hands
Perioral skin
Oral mucosa in conjunction with intestinal polyposis and predisposition to develop cancer
Peutz-Jeghers Syndrome: When do pigmented lesions develop?
Early childhood
Describe oral lesions and where they are located in peutz-jeghers syndrome
1-4 mm brown to blue-gray macules, occur on vermillion, labial and buccal mucosa and tongue
Where do skin lesions occur in peutz-jeghers syndrome?
On the extremities
Describe peutz-jeghers syndrome’s lesions compared to true freckles
Lesions do not wax and wane with sun exposure like a true freckle does
Describe intestinal polyps that occur in peutz-jeghers syndrome
Hamartomatous intestinal polyps throughout the GI tract that are not premalignant
Define Hamartoma
Benign growth of normal tissue
What are other GI findings in peutz-jeghers syndrome
Intussusception (telescoping) of the bowel
While the intestinal polyps are not premalignant, 33 percent of patients develop GI malignancy by 60 years of age
Treatment of Peutz-Jeghers Syndrome
Dentists can be screeners for this condition, characteristics pigmentation arise in childhood before patient is at significant risk for syndrome associated malignancy
Once peutz-jeghers syndrome is diagnosed, describe treatment
Pigmented lesions do not require treatment, they are benign
Patient should be monitored by GI doctor for malignancy and intussusception
Ehlers-Danlos Syndrome: Define
A group of inherited connective tissue disorders resulting in the abnormal production of collagen
Inheritance pattern of ehlers-danlos syndrome
Autosomal dominant
How are joints and skin affected in ehlers-danlos syndrome
Hypermobility of the joints, easy bruising, elasticity of the skin
What type of scarring occurs with ehlers-danlos syndrome?
Papyraceous scarring (resembling crumpled cigarette paper)
Oral manifestations of ehlers-danlos syndrome
Gorlin SIgn: touching the tip of the tongue to the nose
TMJ subluxation
Pulp Stones
Treatment of Ehlers-Danlos syndrome
Classical type usually normal lifespan
TMJ subluxation treated as normal
Marfan Syndrome: Describe
A multisystem connective tissue disorder due to a mutation in the fibrillin-1 gene
Marfan Syndrome Inheritance Pattern
Autosomal dominant
Marfan syndrome effect on body size
Tall, thin body habitus
Cardiovascular system effects on marfan syndrome
Risk of aortic aneurysm aortic dissection, valve malformations
Marfan syndrome effects on eyes
Early onset glaucoma, retinal problems, lens problems
Skeletal complications associated with marfan syndrome
Scoliosis
Oral cavity effects on marfan syndrome
High arched palate
Dental crowding
Treatment for marfan syndrome
Ideal dentition achievable through prosth and ortho
Systemic Sclerosis: Describe
Immunologically mediated pathogenesis involving abnormal interactions among vascular tissue, connective tissue and immune cells.
What is deposited in systemic sclerosis? Where?
Dense collagen deposited throughout the body in extraordinary amounts: mainly skin, but every organ affected.
Population affected by systemic sclerosis
Women affected 3-5x more than men, occurs in adults
How is opening affected by systemic sclerosis
Microsomia -> limited opening due to collagen deposition in the perioral tissues
Oral manifestions of systemic sclerosis
Loss of attached gingiva and recession
Dysphagia (due to lingual collage deposition)
Xerostomia
Widening of PDL
Generalized resportion of the mandible
Describe Raynaud phenomenon in systemic sclerosis
Vasoconstriction in cold weather
Describe how figures are affected in systemic sclerosis
Resportion of terminal phalanges, flexion contractures (claw-like fingers)
Skin: Diffuse, hard and smooth texture
Diagnosis of Systemic Sclerosis
Serum lab testing for Anti-topoisomerase I antibodies
Overall treatment for systemic sclerosis
Systemic medications like penicillamine to prevent collagen production, systemic steroids have little effect and photochemotherapy some benefit for the skin
Oral treatment for systemic sclerosis
-May need collapsible dental appliances to replace teeth
-Surgical correction of open bite caused by condylar resportion if necessary
Describe Prognosis of systemic sclerosis
-Fibrosis of lungs, heart, kidneys, GI tract -> organ failure within 3 years of dx
“C” in CREST
Calcinosis cutis (calcium deposits in the skin)
“R” in CREST
Raynaud phenomenon (vasospasm in cold weater causing less blood to flow to digits)
“E” in CREST
Esophageal dysfunction (due to abnormal collagen deposition)
“S” in CREST
Sclerodactyly (Claw like deformity of the fingers due to abnormal collagen deposition in the dermis)
“T” in CREST
Telangiectasia (superficial, dilated capillaries)
Describe Telangiectasia
Vascular lesion caused by dilation of a small, superficial blood vessel
-Visible dilated small blood vessels
-Blanch with light pressure
Describe Petechiae
Round, pinpoint area of hemorrhage
Describe Purpura/Ecchymosis
Non-elevated area of hemorrhage, larger than a petechia
Population affected by CREST
Female predominance
50-70 years old
Diagnosis for CREST
Serum lab testing for anticentromere antibiodies
What is less severe and has a better prognosis than systemic sclerosis?
CREST
Treatment for CREST
Monitored for increased risk of pulmonary hypertension and primary biliary cirrhosis (more than 10 years after diagnosis)
Hereditary Hemorrhagic Telangiectasia: Describe
Autosomal dominant disorder in which genetic mutations results in decreased blood vessel integrity
Clinical Features of Hereditary Hemorrhagic Telangiectasia
Oral lesions are most dramatic and easily identifiable
Telangiectatic vessels of vermillion zone of lips, tongue, buccal mucosa
Periodontal vascular malformations
Epistaxis
AV Fistulas: Lungs, liver, brain
Overall treatment for Hereditary Hemorrhagic Telangiectasia
Electrocautery, cryosurgery, laser ablation
Progesterone, estrogen therapy
Antibody therapy against endothelial growth factor
Dental treatment for hereditary hemorrhagic telangiectasia
Antibiotic prophylaxis until pulmonary AV fistula has been ruled out
