Chapter 16 - Part 1 Flashcards
What term describes a group of inherited conditions in which two or more ectodermal derived anatomic structures fail to develop?
Ectodermal dysplasia
What are the ectodermal structures that fail to develop in ectodermal dysplasia?
Skin, hair, nails, teeth, sweat glands
How many types of ectodermal dysplasia are there and what is the best known type?
Over 170 types, and the best known is hypohidrotic ectodermal dysplasia
What are the characteristics of hypohidrotic ectodermal dysplasia?
X linked, heat intolerance, fine sparse hair, periocular wrinkling with hyperpigmentation, dystrophic or britrle nails, hypoplastic or absent salivary glands, reduced number of teeth and have abnormal crowns
What is the treatment for hypohidrotic ectodermal dysplasia?
Tx includes genetic counseling and various dental prosthetics
What term describes a benign, genodermatosis that is symmetrical, thickened, white, corrugated, asymptomatic, diffuse plaques on the bilateral buccal mucosa?
White sponge nevus
What is the genetic component of white sponge nevus?
Autosomal dominant, that causes defects in keratins 4 and 13
When do white sponge nevus lesions first appear?
At birth or early childhood
What is the treatment for white sponge nevus?
It is a benign condition so no treatment is required
What are the characteristics of Peutz-Jeghers synrome?
Freckle like lesion of the hands, perioral skin, and oral mucosa; intestinal polyps and obstruction
What is significant for patients with Peutz-Jegher’s syndrome?
Patients are 18x more likely to have a malignancy in their lifetime, but not from the intestinal polyps!! (GI adenocarcinoma, breat cancer)
Is Peutz-Jegher’s syndrome autosomal dominant or recessive?
It is autosomal dominant, but is a rare condition
When do skin lesions develop in Peutz-Jeghers syndrome? and what do they resemble?
Lesions develop during childhood and resemble freckles, but do not wax or wane with sun exposure
What is significant about the intestinal polyps in Peutz-Jeghers syndrome?
Intestinal polyps are hamartomatous growths, and are not premalignant
Why do patients with Peutz-Jegher’s syndrome have a problem with intestinal obstruction?
Due to intussusception = “telescoping” of a proximal segment of the bowel into the distal portion
What do oral lesions look like in Peutz-Jegher’s syndrome?
Oral lesions are an extension of the perioral freckling so the are 1-4 mm brown macules and primarily affect the labial/buccal mucosa and the tongue
What is Tuberous Sclerosis characterized by?
CNS manifestations, mental retardation, seizure disorders, potato like growths, angiofibromas of the skin, ungual fibromas, Shagreen patches and Ash leaf spots, cardiac rhabdomyoma, renal angiomyolipomas
Where do ungual fibromas occur?
Seen around or under the margins of the nails
What is a Shagreen patch?
A connective tissue hamartomas
What is an ash leaf spot?
An ovoid area of hypopigmentation (*reverse of cafe au lait)
What are the dental considerations of Tuberous Sclerosis?
Developmental enamel pitting on the facial aspect of the anterior permanent teeth, and multiple fibrous papules
What is the treatment and prognosis of tuberous sclerosis?
Treatment is directed to management of the seizures (which can lead to drug induced gingival hyperplasia), and patients have a reduced life span due to CNS or kidney disease
What term describes a group of inherited blistering mucocutaneous disorders?
Epidermolysis Bullosa
What are the 4 types of epidermolysis bullosa?
Simplex, Junctional, Dystrophic, Hemidesmosomal
What is simplex epidermolysis bullosa?
Blistering of the hands and feet, mucosal involvement is uncommon, no scarring and good prognsosi
What is junctional epidermolysis bullosa?
Severe blistering at birth, granulation tissue around the mouth, oral lesions are common, often fatal
What is dystrophic epidemolysis bullosa?
Dominant forms are usually not life threatening and blistering with scarring occurs on areas exposed to chronic trauma (knuckles, knees).
Recessive form is severely debilitating , where blisters occur with minor trauma (eating), then secondary infections occur, die before adulthood
What term describes an autoimmune disease where antibodies are directed against desmosomes? What kind of epithelial split would this cause?
Pemphigus –> intraepithelial split
What is the most common pemphigus disorder and what does it affect?
Pemphigus vulgaris, which affects the oral mucosa
What is the first sign of pemphigus vulgaris and the last to resolve with therapy?
“first to show, last to go” –> oral lesions!!
In what age population does pemphigus vulgaris most commonly occur?
Average age is 50,
What is the clinical presentation of pemphigus vulgaris?
oral pain, multiple ulcerations in oral cavity
Patients rarely report vesicle/bullae because they ruptures so quickly then leave an ulceration
What is a characteristic feature of pemphigus vulagris that can be done to test for pemphigus vulgaris?
Nikolsky sign = a positive sign means that bulla can be induced on normal appearing skin if firm lateral pressure is exerted
What are the histopathological features of pemphigus vulgaris?
Cells of the epithelium fall apart (acantholysis), and loose cells are termed Tzanck cells
What is direct immunofluorescence positive for in pemphigus vulgaris?
C3 and IgG within the epithelium
How is pemphigus vulgaris treated?
Send to a dermatologist, who will administer corticosteroids and maybe combine it with other immunosuppressive drugs
What is the prognosis of pemphigus vulgaris?
75% will have resolution in 10 years; 15% will remain of steroids throughout life and 10% of those will succumb to the disease
What term describes a condition where antibodies are directed against hemidesmosomes? what type of epithelial split is this known as?
Mucous Membrane Pemphigoid –> interepithelial spilt
What is another name for mucous membrane pemphigoid?
Cicatricial pemphigoid
How common is pemphigoid?
It is 2x as common as pemphigus
What is the oral clinical presentation of mucous membrane pemphigoid?
intraoral blisters (intraoral blood blister) that don’t scar
What is another significant complication of mucous membrane pemphigoid?
Ocular involvement in up to 25%
How can early ocular involvement of mucous membrane pemphigoid be detected?
Slit lamp examination by an ophthalmologist
What is the pathological progression of the ocular involvement of mucous membrane pemphigoid?
Conjuctiva become inflamed and eroded –> scarring and adhesions (symblepharons) –> turn eyelids inward –> more scarring and keratin production in the eye = blindness
What does direct immunofluorescence show in mucous membrane pemphigoid?
C3 and IgG along the basement membrane
What is the treatment of mucous membrane pemphigoid?
Refer to an ophthalmologist! Use topical corticosteroids first
What term describes a blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis?
Erythema multiforme
What may precede/”trigger” development of erythema multiforme?
Preceding infection or exposure to medications
- either herpes simplex or mycoplasma pneumoniae
- ABX or analgesic
What are the types/spectrum of erythema multiforme?
Erythema multiforme minor, Erythema multiforme major (Steven-Johnson syndrome), Toxic epidermal necrolysis (Lyell’s disease)
In what population does erythema multiform usually occur?
Young adults (20-30’s), and usually more common in males
What is the prodromal syndrome associated with erythema multiforme?
Fever, headache, cough, sore throat that occurs 1 week before onset
How long does erythema multiform last? Does it recur?
2-6 weeks, and yes 20% recur
What is the early presentation of erythema multiforme?
It has an acute onset! Quick and painful onset! Flat, round, red lesions that become elevated and evolve into bulla with necrotic centers
*skin lesions look lik econcentric circular erythematous rigns resembling a target
- oral lesions may cover entire oral cavity except hard palate and gingiva
- hemorrhagic crusting of vermillion zone of the lips is common
What is a concern in patients with erythema multiforme?
patients may become dehydrated due to inability to ingest liquids
What is Steven-Johnson syndrome?
Erythema multiform major –> invovlement of skin, oral mucosa and either ocular or genital mucosa
- usually triggered by a drug
What is Lyell’s disease?
Toxic Epidermal Necrolysis, the most severe form of erythema multiforme –> diffuse sloughing of the skin and mucosa that occurs in older people + females
- always triggered by a drug
What is the treatment for erythema multiforme?
Management is controversial, but discontinuation of the causative drug is paramount. Admit to hospital for IV fluids
Antivirals if due to herpes
Steroids in EM minor and major NOT TEN
What is mortality of of erythema multiforme major and toxic epidermal necrolysis
Major = up to 10% TEN = 35%
What term describes a common condition primarily affecting the tongue?
Geographic tongue
What are other names for geographic tongue?
Erythema migrans, benign migratory glossitis
What disease may geographic tongue be related to?
Psoriasis
Where does geographic tongue occur?
Typically on the anterior 2/3 of the dorsal tongue, concentrated on the tip and lateral border
What is characteristic of erythema migrans lesions that are not on the tongue?
They will have a raised, yellow-white serpentine border.
Begin as multiple, small white patches which develop a central erythematous atrophic zones and enlarge into well demarcated zones of erythema
Why is geographic tongue/erythema migrans “migratory”?
The lesions will heal in one area and then develop in a different area
What is geographic tongue associated with?
Fissured tongue
What is the treatment of geographic tongue?
No treatment required, however if the patient experiences burning topical corticosteroids are appropriate. Also ensure patient they have a BENIGN condition, and if they have fissured tongue, recommend a tongue scraper
What term describes a chronic, common dermatological disease that can affect the oral mucosa?
Lichen planus
What might lichen planus be due to?
Medications, amalgam (this is better known as “lichenoid mucositis”)
In what population is lichen planus common?
Middle aged adults, females more thanmales
What are the 4 P’s associated with lichen planus?
Purple, pruritus, polygonla, papules
What is characteristic about the papules in lichen planus?
A lacelike network of white lines = Wickham’s striae
What are the two forms of oral lesions?
Reticular and erosive
What is reticular lichen planus?
Much more common, asymptomatic, involves posterial buccal mucosa bilaterally, but can involve the tongue. Lesions tend to wax and wane
What is erosive lichen planus?
Symptomatic, lesions are atrophic, erythematous areas with a central ulceration
What terms describes erosive lichen planus limited to the gingiva?
Desquamative gingivitis
What are the histopathological features of lichen planus?
Saw tooth rete ridges, destruction of the basal layer, band like infiltrate of lymphocytes subadjacent to the epithelium, degeneration of keratinocytes (Civatte bodies), deposition of fibrinogen at the basement membrane
What is the treatment of lichen planus?
Biopsy is always indicated
What can induce lichen planus lesions?
Stress, Trauma, Advil (NSAIDS), Yeast
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