Oral Manifestations Of Skin Diseases

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Ectodermal Dysplasia - A group of inherited conditions in which 2 or more ectodermally derived anatomical structures fail to develop.
- Deacreased # of sweat glands (intolerance to heat)
- Fine, sparse hair; possible periocular wrinkling and hyper pigmentation; possible midface hypoplasia
Oral - Marked decrease in number of teeth (hypodontia)
- Abnormal shape of teeth, tapered anterior crowns
- In most severe cases, cuspids and 1st molars are present but usually abnormal n shape

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Autosomal dominant transmitted condtions (due to defect of normal keratinization)

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White Sponge Nevus - Discussed under epithelial lesions
usually bilateral
Histo - epithelium shows acanthuses and hyperparakeratosis

biopsy to confirm - no malignant transformation

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Peutz-Jeghers syndrome (see pigmented lesions)
- Childhood onset, familial condition
- Oral perioral melanotic macules
- intestinal polyposis
- Pigmentation of 1-5 mm found around lips, eyes, nose...
- Oral pigmentation includes buccal mucosa, gingiva, palate (seldom tongue)
- Polyps may cause abdominal pain and cause obstruction due to intussusception.

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Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Disease) [soft tissue lesions lecture]
- Autosomal dominant
- tend to undergo repeated hemorrhage
- Epistaxis may be an early sign
- Pt. may suffer from anemia, but not usually life threatening.

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Lichen Planus - Reticular Form- Lacy-like pattern (have Stiae of Wickham) - MOST common type
- seldom has symptoms, Often bilateral
Location:
- Buccal mucosa - 80%
- tongue - 65%
- Lips - 20%
HISTO: Hyperkeratosis in reticular type.

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Relatively common, chronic dermatologic disease that affects the oral mucosa. Unknown etiology, but appears to me a cell-mediated immune process. Severity may parallel pt's stress level in some cases.

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Lichen Planus (seen in 1-2% of population)
- F:M = 3:2 (but Dr. T says 90% female)
- Primary symptoms = pruritis
- Small papules (a few mm in diameter)
- Seen in flexor surfaces. wrist, knees, inner thigh...
Most pt.s have oral lesions. If pt. has oral lesion, 20-40% chance of having a skin lesion.
- several patterns seen

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Names the patterns of Lichen planus and which is most common.

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Reticular form - mentioned above
Plaque form - may resemble Leukoplakia - usually dorsum of tongue.
Atrophic form - smooth, poorly defined red areas (may be seen in conjunction w/ reticular or erosive types).
Vesicle and bulla formation (rare)
Erosive Type - (granular and erythematous) Ulcerative type, painful. May have radiating striae around ulcer
MAIN TWO ARE: Reticular and Erosive.

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Erosive lichen planus - same pt. after systemic corticosteroid therapy.
Erosive lochen planus cont. (not necessary this pt.)
- many pts also have secondary oral candidiasis
- Can be confused w/ pemphigoid or pemphigus
- Touching areas during an exam may produce pain and bleeding.

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Lichen planus - Lichenoid mucositis w/ superimposed candidiasis. - same pt. 2 weeks after antifungal therapy
HISTO for lichen planus:
- "Saw toothed" rete ridges
- Basilar cell degeneration
- Infiltrating of lymphocytes under basement membrane
- Thin band of eosinophilic coagulum

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What is desquamative gingivitis?

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Describes gingival epithelium that spontaneously sloughs or can be removed w/ minor manipulation.
- should be biopsied b/c mucous membrane pemphigoid and pemphigus vulgarism may appear in a similar fashion.
- seen here w/ erosive lichen plans.

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Treatment for Lichen planus

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Palliative - not a cure
- Reticular type often does not require treatment.
Topical application of steroids
- Kenalog and orabase: put on lesions & swish for 2 min.
- .05 Fluocinonide (lidex): (3X a day in severe cases) - don't take for a long time b/c causes adrenal suppression
- Dexamethasone (Decadron) elixir swish around
- Clobetasol (Temovate) - one of the strongest topical steroids must also use topical antifungal.
Systemic steroids
Antifungal meidcation
Vitamin A analogues
Standard of care is that all lichen planus pt.s should be biopsied.

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serious cicatricial skin disease characterized by the appearance of vesicle and bullae that develop in cycles

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Pemphigus Family
- Possible Autoimmune - Ab. to intercellular cementing substance (autoantibodies against desmosomes)
- Usually adults (esp. Jews) (average age = 50)
- 1-5 per million diagnosed each year.
Start with rapid bullae (or vesicles)
Rupture - leave ulcerations
Painful

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Pemphigus Vulgaris (most common type - and the one we discuss) (other types are P. foliaceous, p. megatons, p. erythamatosus - the are rarely oral)
- Seldom before 30
- Skin lesion may be accompanied by fever or malaise
- Nikolsky's sign - pressure of friction causes bullae or vesicle
- mainly affects torso

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Oral - Often first manifestation (50% of cases)

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Pemphigus Vulgairs
- All sites can be affected orally
- Painful - cannot eat
HISTO:
- Acantholysis -loss of cohesiveness between cells lying in vesicular space (epithelial cells lying free w/in bulla).
- "Tzanck cells" - the cell called Tzanck cell undergoes changes that include swelling of nuclei and hyperchromatin staining
- Supravasilar vesicles or intraepithelial vesicles(above basal layer)
- "Rows of tombstones"

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Diagnosis and Treatment of Pemphigus Vulgaris

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Standard biopsy
Direct immunofluorescence - best way to confirm
- Long term steroid therapy has improved prognosis, still 5-10% of cases are possibly fatal.
- Corticosteroirds -high doses cause remission
- Steroids used w/ non-steriod immunosuppresant drugs such as azathioprine.

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Location - oral mucosa, skin, genetalia, and ocular involvement (25% of the time)

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Benign Mucous Membrane Pemphigoid (Cicatricial Pemphigoid) (BMMP)
- Twice as common as pemphigus vulgarism.
- Autoimmune
- Deposits of immunoglobulins and complement are found along the basement membrane
ocular involvement (important) (25%)
- Conjuctivitis
- adhesions
- scarring
- blindness (30%)

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BMMP - oral involvment
- seen in most cases
- mainly gingiva (may be misdiagnosed as desquamative gingivitis); also palatal mucosa
- Vesicles thick-walled - last longer before rupture
- leave bleeding surface
- gingiva may show persistent erythema for weeks
- + Nikolsky's sign - pushing on gingiva and a blister will form in a few min.

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Differential Diagnosis for Benign Mucous Membrane Pemphigoid

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- Pemphigus
- Bullous pemphigous
- Erythema Multiforme
- Chronic desquamative gingivitis

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Antibodies attacking the basement membrane (sub-epithelial)

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BMMP
- hemidesmosome is involved
- No acantholysis
2:1= Female: Male 50-60 yrs old.
Treatment - use of steroids, both local and systemic (may use custom tray)
- Lack of oral hygiene often aggravates gingival condition, cholorodexidine may be useful.

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Bullous Pemphigoid (many fetures similar to BMMP)
- Rash on libs, many weeks before bullae
- Bullae are thick-walled (sub-epithelial)
- No acantholysis
Oral involvem - Less frequent than BMMP - occurs in 10-45% of cases.
Treat w/ corticosteroids, may have spontaneous remission.

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"target" or "Iris" lesion - peripheral gets larger as central area undergoes healing.

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Erythema Multiforme (EM)
- An ulcerative and blistering mucocutaneous condition caused by a hypersensitivity reaction (probably to drugs/food/pollen) - some may appear spontaneously
- Viral lesions may trigger disease ( up to 50%)
- Other precipitating factors are GI condition such as chron's dz, malignancies, radiation therapy, vaccinations.
- Usually rapid onset w/ prodromic period or 3-7 days with headache, fever, malaise

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Erythema Multiforme
- oral lesions are less common than skin (hands, arms, feet, face, neck...)
- Painful, and my involve any area of the mouth
- Macules, papules or skin vesicles may become eroded and bleed
There are two main forms, mild and chronic.
Chronic is the mildest and may have continuos lesions for more than a year.

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What is one of the very noticeable oral manifestations of EM

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Bloody crusty lips.

No specific treatment - self limiting but may have chronic episodes.

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What are the associated syndromes of EM?

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Stevens-Johnson syndrom (EM major) -sever
- Skin 10% of total body affected, and oral cavity
- eyes - conjunctivitis (may lead to reccurent infection and blindness.
- genitalia (urethritis)

Toxic Epidermal Necrosis (TEN) - Very Bad
- Blistering over more than 20% of body
- High mortality rate.

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An immunologically mediated condition that presents in one of serval forms, and affects skin, CT and specific internal organs

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Systemic Lupus Erythematosus (L.E.)
- there are two other forms Chronic cutaneous LE and subacute cutaneous LE.
- average age is 30-40 w/ a 8:1 female predilection
- common findings are: fever, weight loss, arthritis, & general malaise.
- butterfly rash is seen in 50% of pt.s
- Kidney is affected in 50% of pt.s
- oral lesion in 5 - 40% of cases - appear as lichenoid areas
- cardiac condition are common.

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oral lesion in 20% of cases

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Chronic cutaneous LE
- Few or no symptoms, sun exposure affects
- Pts have scaly, erythematous patches on skin, also may have butterfly rash over nose.

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Chronic cutaneous LE
- oral lesions appear similar to lichen planus (leukoplakic areas with or without ulcerations)
Diagnosis - microscopic, direct immunofluorescence, Antinuclear antibodies (ANAs)
- For systemic LE 5 year survival is 95% and 15 yr is 75%
- overall prognosis depends on organ involvement
- For chronic 50% resolve after several years.

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Skin disease affecting 2% of population, with positive Auspitz sign. Oral lesions are uncommon and if there are any, they appear as whit or red plaques and possible ulceration.

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Psoriasis
- symmetrical scaly papules of extensor surface, face, back, chess, scalp, elbows (Erythromatous plaque coved by silvery scales). Plaques bleed if picked off - positive Auspitz sign
-12 % have arthritis
Rx - sunlight, steroids, tar soaps, calcipotriene ( Vit D3 analog) tazarotene (a Vit A compound)

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Inherited blistering mucocutaneous disorder, that in the severe form is not compatible with life.

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Epidermolysis Bullosa
- Vesicles form in mild areas of trauma

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Epidermolysis Bullosa
- Severe caries associated with a soft cariogenic diet (less trauma to oral tissue)
- Whole body is affected, fingernails may be lost.
Oral manifestation inclued:
- Gingival erythema
- anodontia
- Enamel hypoplasia
- Bulla and vesicle formation.

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Fixation of dermis due to excess dense collagen deposition - hands become stiff and skin cannot wrinkle

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Scleroderma (systemic sclerosis)
- Face looks mask like
- Organs involved lead to organ failure
- Raynaud's phenomenon of hands, face, and trunk
- Neuralgia and parasthesia develops
Oral involvement mostly in tongue, soft palate, larynx. lips become rigid.

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Uncommon dermatologic condition that has striking skin lesions along with subtle oral mucosal lesion.
Altered epithelial cells - build up a lot of keratin

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Darier's Disease (keratosis Follicularis)
- Numerous erythematous papules on skin (esp. trunk and scalp)
- Palms and soles exhibit pits and keratoses - stinks
Oral lesion consists of white flat papules and are seen in 50% of pts. Kind of a cobblestone looking appearance on palate.

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An uncommon solitary lesion on the skin or mucosa hisopathologically identical to Darier's disease. But just one leison

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Warty Dyskeratoma
- Seen in pts older than 40
- roughed surface
- less and 5 mm
- no symptoms.

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Darier's Disease

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Immunologically mediated condition that may be triggered by one of several infectious agents in a susceptible person.
Has a classic triad of signs

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Reiter's syndrome (Reactive Arthritis)
Classic triad of signs
- Nongonococcal urethritis
- Arthritis
- Conjuctivitis
- usually seen in young men, and develops 1-4 weeks after an episode of dysentery or venereal disease. May develop skin lesion on penis
- 20% may have oral lesions such as erythematous papules and ulcerations.