Ch 16 Oral Dermatology Flashcards
what parts of the body may be affected by ectodermal dysplasia?
-skin, hair, nails, teeth and sweat glands
skin problems of ectodermal dysplasia?
- fine, sparse blond hair
- reduced eyebrow and eyelash hair
- periocular hyperpigmentation
- dystrophic brittle nails
what are the oral problems/manifestations of ectodermal dysplasia?
- xerostomia secondary to decrease salivary gland development
- hypodontia
- cone shaped teeth
which disorder has cone shaped teeth, hypodontia, and xerostomia?
-oral manifestations of ectodermal dysplasia
what is the treatment for ectodermal dysplasia?
- genetic couseling for the family
- prosthetic replacement of the teeth
- thick white buccal mucosa bilaterally
- may also be seen in other mucosa
white sponge nevus (AD disorder)
-benign hyperparakeratotic condition with no txt
benign hyperparakeratotic condition with no treatment
white sponge nevus
-AD disorder seen in NC Lumbi indians
Hereditary Benign Intraepithelial Dyskeratosis
- thick white plaques on the buccal mucosa and conjuctiva
- dyskeratosis in upper layers of epithelium
Hereditary Benign Intraepithelial Dyskeratosis
-benign, no txt
- Erythematous pruritic papules on the trunk and scalp that have foul odor
- AD
- nails show ridges and splits
- oral: white papules
- keratolytic agents
Darier’s Disease (Keratosis Follicularis)
-orally manifestation: single white papule located on hard palate or alveolar ridge
- Warty Dyskeratoma (Isolated Darier’s Disease)
- txt: excision
- identical histology to darier’s disease with suprabasilar cleft and dyskeratosis
- Freckly-like lesion in and around the oral cavity and hands
- intestinal polyposis with predisposition to change into adenocarcinoma
Peutz-Jeghers Syndrome
-genetic counseling
location of intestinal polyps in peutz-jeghers syndrome?
polyps in jejunum and ileum
- vascular hamartomas due to decreaded blood vessel wall integrity
- frequent epistaxis
- telangiectasia intraoral, hands, feet, GI tract, GU tract and eye
- iron deficiency anemia if severe
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu Syndrome)
- multiple variations of abnormal collagen production due to genetic abnormalities
- patients have hypermobility of joints and elasticity of the skin (“rubberman”)
Ehlers-Danlos Syndrome
- 50% can touch their nose with their tongue
- Subluxation of the TMJ
- Bruising and bleeding of the oral tissues
Ehlers-Danlos Syndrome
20% of these lesions are caused by drug reactions, the others we don’t know the cause
Lichen Planus
chronic inflammatory disease
-white striations, papules or plaques on the buccal mucosa, tongue and gingiva
lichen planus
age rage and sex predilection for lichen planus?
45-60 Women 2:1, skin lesions common
what is thought to be a possibility for lichen planus?
thought to be T-cell mediated autoimmune disease in which CD8+T cells trigger apoptosis of oral epithelial cells
patients with lichen planus with persistent mouth sores are at increased risk to get…
SCCa
-Allergic or immunologic reaction
-20% medication induced
-Linked to chronic hepatic diseases especially Hepatitis C
-Psycogenic (stress and anxiety)
-Associated with LP of skin, HBP
and diabetes
-Dental materials
causes of lichen planus
- purple
- polygonal
- pruitic
- papules (papular)
skin lichen planus
- used to diagnose pemphigus
- dislodgement of skin by lateral pressure (or air syring)
- used for multiple diseases
Nikolsky Sign
BMMP
Benign Mucous Membrane Pemphigoid
- average age 60
- women 2:1
- oral lesions (conjunctival, nasal, esophageal, laryngeal and vaginal
- clinical vesticles and bullae
- Ocular involvement most significant (may leave to blindness, and 25% one eye before the other)
BMMO (Benign Mucous Membrane Pemphigoid)
what are the eye findings of BMMP?
25% oral develop ocular:
- symblepharons (adhesions between upper and lower eyelid)
- entropion (scarring/eyelids turn inward)
- trichiasis (eyelashes rub cornea and globe and scarring closes openings of lacrimal glands)
Histology: subepithelial slefting
-Imuno Fluorescence to basement membrane area
BMMP
treatment for BMMP
- Dapsone (cannot be G6PD deficient)
- Steroids
- Tetracycline and niacinamide
- immunosuppresive agents
two types of pemphigus that affect oral tissues
- pemphigus vulgaris
- pemphigus vegetans
- a mucocutaneous AI disease characterized by intraepithelial acanthoslysis due to immune complex deposition at cellular attachment bridges
- intraepithelial clefting (seaparation)
- blistering due to AI attack of desmosomes
pemphigus vulgaris
- txt: steroids
- 60-80% will die without txt
average age for pemphigus vulgaris
- sex predilection
- race predilection
- location
50 -male=female -jewish -ragged erosions and ulcers: ANY oral mucosa (positive Nkolsky sign)
“the oral lesions are the first to show and the last to go”
pemphigus vulgaris
Histology: intraepithelial separation just above the basal cell layer leaving “row of tombstones”
- Acontholsysis with floating Tzanck cells
- positive indirect immunofluorescence for intercellular IgG, IgM and C3
Pemphigus Vulgaris
- Cross-reacting antibodies in lymphoma or leukemia attack desmosomal complex.
- May precede the discovery of the underlying malignancy.
- Often fatal
Paraneoplastic Pemphigus (Neoplasia-Induced Pemphigus)
- occurs in recipients of allogenic bone marrow transplantation
- engrafted cells recognize body as foreign
Graft Versus Host Disease
- few weeks after transplant
- affects 50%
- mild rash to TEN
- diarrhea, nausea, vomiting, abdominal pain and liver dysfunction
acute graft versus host disease
-lasts 100 days to years and
develops 33-64%
-Mimics autoimmune disease
-Skin lesions resemble LP or systemic sclerosis
chronic GVHD
oral manifestations of GVHD
- 33-75% of AGVHD and 80% of CGVHD
- Resembles lichen planus
- Some complain of burning sensation (R/O candidiasis
- xerostomia common
- immunologic response destroying salivary glands
- mucoceles of soft palate
txt of GVHD
-Prophylactic therapy with immunomodulatory and immunsuppressive agents such as cyclosporine & prednisone
-Methotrexate has been added and reduced GVHD further
-Thalidomide has shown some
promise
-Topical CS oral lesions
-Psoralen and Ultraviolet A (PUVA) improve lichenoid form
-Treat xerostomia
-Increased proliferative activity of cutaneous keratinocytes
-Immunologic with activated
T-lymphocytes
-Well demarcated silvery plaque with silvery scale on surface
-Non-symptomatic to itching
Psoriasis
-no txt
histology:
- parakeratosis with elongated rete ridges
- Munroe abscesses
- perivacular chronic inflammation
- CT papillae dilated capullaries close to epithelial surface
Psoriasis
lupus erythematoosus
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