Transcript Mucous Membrane Disorders KCOM/Texas Dermatology Residency Consortium

Mucous Membrane Disorders
KCOM/Texas Dermatology
Residency Consortium
Cheilitis Exfoliativa
• Desquamative, recurrent, fissures if severe.
• MC upper lip if cause is unknown.
• MC lower lip if it is a reaction to other disease
states, ie SD, AD, PV, Plummer-Vinson syndrome.
• Irritation: lipsticks, dentrifices, mouthwashes,
shaving/aftershave, nail enamel, lip licking, UV
• Tx: Remove cause, topical steroids, ointments
Allergic Contact Cheilitis
• Vermillion border MC, dryness, fissuring,
edema, crusting, angular cheilitis.
• Topicals - meds, dental, lipsticks, sunscreen
lip balms, cosmetics, nail polish, cigarette
holders, rubber, metals, toothpaste.
• Foods – oranges, lemons, artichokes,
mangoes
• Saxophone or Clarinet cane reeds
• Tx: avoid antigen, topical steroids
Actinic Cheilitis
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Lower lip MC, UV induced
Ulceration is rare unless SCC is present
Hereditary PMLE may resemble
Treatment: Biopsy if thickened or ulcerated
Cyro, 5-FU, CO2 laser, Vermilionectomy,
Photodynamic Therapy with 5-ALA
H&E same as AK
Cheilitis Glandularis
• Swelling and eversion of lower lip with
patulous openings of the ducts of the
mucous glands - Chronic, inflammatory.
• Mucous exudes freely to form a glue-like
film, lips stick together, palpation feels like
pebbles beneath the surface.
• Apostematosa variant has abcess formation.
• Etiology: irritation, atopic, factitious, actinic
H&E – infiltration of lymphocytes,
histiocytes and plasma cells in and
around the ectatic glands.
Cheilitis Glandularis
“STICKY LIPS”
TX SAME
AS ACTINIC
CHEILITIS
Angular Cheilitis (Perleche)
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MC Candida albicans
Labial commisures, moist fissures
Elderly – facial and dental architecture
Youth – thumbsucking, lollipops,
Thrush in DM II or HIV, Tumoral
calcinosis, Deficiency of Iron, Riboflavin,
Vitamin A, E, etc.
Treatment: Dental consultation - dentures
Topical nystatin with iodochlorhydroxyquin (Vioform)
in hydrocortisone ointment.
Injection of dermal filler substances, excision, flap.
Plasma Cell Cheilitis
• Sharply outlined, infiltrated, dark red plaque
with a laquer-like glazing of the surface of
the lower lip
• Similar to Zoon’s balanitis plasmacellularis
• Band-like infiltrate of plasma cells
• Reaction pattern to any number of stimuli
• Clobetasol propionate bid, Griseofulvin
500mg qd.
BAND-LIKE
INFILTRATE OF
PLASMA CELLS
CHARACTERISTIC
KERATINOCYTES ARE
DIAMOND-SHAPED
OR LOZENGE SHAPED
Plasmoacanthoma
• Advanced version of Plasma Cell Cheilitis
• Verrucous tumor with plasma cell infiltrate
• Candida albicans may be found in the
lesions
• Usually grows along the angles of the
mouth
Drug-Induced Ulcer of the Lip
• May be confused with
ulcers of DLE or SCC
• Phenylbutazone
• Chlorpromazine
• Phenobarbital
• Methyldopa
• Thiazide diuretics
• Fixed-Drug/Photo?
Other forms of Cheilitis
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Lichen Planus
SLE
Psoriasis
Lip Biting
Oral Crohn’s Disease
• 10-20% of Crohn’s patients
• 90% have granulomas on biopsy
• Inflammatory hyperplasia of oral mucosa,
cobblestoning, fissuring,
• Metallic dysgeusia
• Gingival bleeding
• Metastatic Crohn’s – non-caseating granulomatous
skin lesions in patients with Crohn’s.
Treatment – oral budesonide, mouthwash containing
triamcinolone, tetracycline and lidocaine, oral metronidazole,
Curettage & Zinc by mouth. Sulfasalazine, Asacol, Pentasa.
Pyostomatitis Vegetans
Inflammatory stomatitis in setting of UC.
Edema and erythema with deep folding of the
buccal mucosa as well as pustules, small
vegetating projections, erosions, ulcers and
fibrinopurulent exudate.
Pustules fuse into shallow ulcers resulting in
characteristic “SNAIL TRACK” ulcers
Skin lesions favor axilla, groin, scalp, crusted
erythematous papulopustules that coalesce into
asymmetrical annular plaques.
Red – edema
Black – perivascular infiltrate
Blue – abcess formation with
eosinophils
TX – Systemic Steroids
Cheilitis Granulomatosa
• Sudden onset of lip swelling that progresses
to permanent lip enlargment
• Upper lip usually swells first
• Cause unknown
• Histology shows tuberculoid granulomas
and an inflammatory reaction pattern
• TX- IL steroids.
Pathology – tuberculoid granulomas with
epithelioid and Langerhan’s giant cells
Melkersson-Rosenthal Syndrome
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Classic Triad starts in adolescence
Lip enlargement (other areas may swell too)
Scrotal Tongue
Recurring facial paralysis
Pathology similar to Cheilitis Granulomatosa
R/O Ascher Syndrome – lip swelling, edema of eyelids
(blepharochalasis)
• IL Steroids, Surgical nerve decompression, cosmetic
surgery for lip reduction, Clofazimine, Thalidomide
Fordyce’s Disease (Spots)
• Ectopically
located sebaceous
glands
• Minute orange or
yellowish pinhead
sized macules in
mucosa of lips
• Tx: Isotretinoin
Stomatitis Nicotina
• “Smokers keratosis” “Smokers patches”
• Ostia of the mucous ducts appear as red
pinpoints surrounded by milky white,
slightly umbilicated papules on the palate
• Maceration, ulceration and aphthae.
• Heat may be causative event.
• Tx: Stop smoking, stop drinking hot liquids.
Torus Palatinus
• Bony protuberance in the midline of the hard palate, asymtomatic
Scrotal Tongue
May be congenital or familial.
Large tongue with plicate superficial or deep
grooves, longitudinal along the median raphe
Associations: Melkersson-Rosenthal
Syndrome, Down’s Syndrome, Pachyonychia
Congenita, Pemphigus Vegetans, Cowden’s
Syndrome
Geographic Tongue
• Isolated finding or associated with
Atopic Dermatitis or Psoriasis
• Annular atrophic areas looks like
outlines of countries on a map,
locations of lesions change weekly.
• Asymptomatic usually
• Topical 0.1% Tretinoin solution
may clear in 4-6 days
Histology: epidermal hyperkeratosis and marked
transepidermal migration of neutrophils (aka munro’s
microabcesses). Cannot be differentiated
histologically from pustular psoriasis or Reiter’s
syndrome.
Black Hairy Tongue
• Benign hyperplasia of the filiform papillae
of the anterior 2/3 of the tongue
• Etio: smoking, oral antibiotics, Candida
• Histo: elongated and stratified filaments
composed of ortho and parakeratotic cells.
• TX: toothbrush, tretinoin, 40% urea, stop
predisposing factors.
Moller’s Glossitis
• Painful, chronic, makes
eating difficult
• Tip and lateral tongue
• Intensely red, well defined
irregular patches in which the
filiform papillae are absent
and thinned and the
fungiform papillae are
swollen.
• Check for macrocytic
anemia, if present Pernicious
Anemia is likely
Hypersegmented
neutrophil
Glossitis of Pellagra
• Sides & tip of
tongue are
erythematous and
edematous with
imprints of teeth,
“Beefy red” with
smooth “glazed”
appearance.
• Niacin, Tryptophan
(Niacin precursor),
Alcoholism.
4 D’s of Pellagra: diarrhea,
dermatitis, dementia, death.
Median Rhomboid Glossitis
• Shiny oval or diamond shaped elevation,
midline, directly in front of the
circumvallate papillae.
• Candida species may be present
• Histo: Chronic inflammation with fibrosis
with occas. hyphae in areas of parakeratosis
• Itraconazole helpful.
Eosinophilic Ulcer of the Tongue
• Ulcer with elevated borders usually covered
by a pseudomembrane.
• MC posterior aspect of tongue
• Rapid onset, spontaneously resolves in a
few weeks. Benign, self-limited.
• Histo: Predominantly eosinophilic infiltrate
with histiocytes and neutrophils
• If multifocal and recurrent CD30 positive
lymphoproliferative disease may be present.
Caviar Tongue
• Small round purplish
capillary
telangiectasias
• Commonly found on
underside of tongue
after age 50
• Etio: elastic tissue
deterioration
Dental Sinus
• Tooth abcess forms a
sinus tract that opens on
the skin as an inflammed
nodule
• MC chin or jawline
• May palpate a cord-like
tract beneath the lesion
• Dental X-Ray diagnostic
• Other fistulas: SCC,
Actinomycosis,
osteomyelitis, deep
fungal, foreign body
Leukoplakia
• Once regarded as
precancerous
• Whitish patches or
plaques of the
mucous
membranes.
• May or may not
reveal cellular
atypia
• Discussed in SCC
lecture
Erythroplakia
• Leukoplakia that has lost the thick macerated
keratin layer.
• MC in mucocutaneous junctions
• Histologically cellular atypia, pleomorphism,
hyperchromatism, increased mitotic figures
• 90% are SCC in situ or invasive.
• Moral: biopsy red areas in the setting of
leukoplakia
Proliferative
Verrucous
Leukoplakia
• 70% become SCC
• F>M 4 to 1.
• Flat white areas on
mucous membranes
that thicken and
become exophytic
• Assoc with HPV 16
• Aggressive early
therapy is best.
SCC
• Lower lip has high metastatic rate.
• Intraoral lesions more likely in those who
consume: Cigarettes, Chewing Tobacco,
Betel Nuts, Alcohol.
• May complicate DEB, Erosive LP, XP,
Dyskeratosis Congenita
• Intraoral SCC has only 30% survival rate.
Aquired Dyskeratotic
Leukoplakia
• 1 patient
• White plaques on palate, gingiva, lips and
genitalia
• Unique finding of clusters of dyskeratotic
cells in the prickle cell layer
• Laser, Etretinate
Melanocytic Oral Lesions
• Intramucosal MC > Compound > Junctional
• Labial melanotic macule – solitary @
vermillion border of lower lip, sharply
demarcated, young women
• Blue nevus – dendritic cells in submucosa
• Oral melanoacanthoma, young blacks,
buccal mucosa after trauma, resolves in
40%
• Melanoma rare, mostly in elderly patients
LABIAL MELANOTIC MACULE
Melanoacanthoma,
variant of pigmented
SK, melanocytes not
restricted to basal
layer
Oral Melanosis
• MC African Americans
• Things to keep in mind:
• McCune Albright Syndrome (dimple over 4th
knuckle, Coast of Maine border, unilateral café au
lait macule with bony abnormalities below it)
• Peutz-Jeghers (polyposis, colon cancer)
• Addison’s Disease
• Tar, Heavy metal poisoning, dental amalgams
• Cis-platinum – gingival platinum line
Osseous Choristoma of the
Tongue
• Nodule on
dorsum of
tongue.
• Contains
mature
lamellar bone
or cartilage
• Does not recur
after excision.
Peripheral Ameloblastoma
• Rare
invasive
neoplasm
of gingiva
• MC lower
jaw
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BCC of
oral
mucosa
per Lever
Trumpeter’s Wart
• Simply a callus
• Upper lip = trumpeter
• Lower lip = trombone
Epulis
• Benign lesion situated on the gingiva.
• Reactive, inflammatory
• Peripheral giant cell granuloma solitary
bluish red, 10-20 mm tumor between or
near bicuspids, incisors.
Pyogenic
Granuloma
• Exuberant
overgrowth of
granulation
tissue
• Bleeds easily
• Rapidly
growing
• Asymptomatic
Low power shows a well
circumscribed nodule
with lobules of dilated
and congested capillaries
High power shows
myxoid stroma and bland
endothelial cells
Granuloma Fissuratum
• Discoid, folded “like a bent coin”,
• Chronic inflammatory fibrous hyperplasia
Angina Bullosa Haemorrhagica
• Sudden appearance of one or more blood
blisters in the oral mucosa
• No associated skin or systemic disease
• May be recurrent
• MC on soft palate of middle-aged or elderly
patients
• No treatment is necessary
Angina Bullosa Haemorrhagica
Subepidermal bullae
KEY: bulla is filled with
red blood cells.
Mucocele
• Result of trauma
or obstruction of
salivary ducts,
usually on the
lower lip
• Soft rounded
translucent
projection often
with a bluish
tint.
Acute Necrotizing Ulcerative
Gingivostomatitis (Trench Mouth,
Vincent’s Disease)
• “Punched out”
ulcerations, rapid onset,
painful, foul, fetid odor
• Bacteroides fusiformis &
Borrelia vincentii
• TX: PCN, 3% H2O2
mouthwash, debridement
• R/O herpes infection
NOMA: severe variant in children with poor nutrition,
gangrenous spread to bone may result in death.
• NOMA, means “to devour”
gangrenous, starts in the mouth
as a benign oral lesion and
rapidly destroys tissues of the
mouth and face. MC under six
years of age.
• Fatal in 70% and 90% of cases,
survivors disfigured for life
• Flourishes where poverty is
greatest, nutrition is poorest and
hygiene is neglected.
• “Face of poverty”
Acatalasemia
• AKA “Takahara’s disease”
• AR, Rare, Japan
• Deficiency of Catalase enzyme in liver muscles,
bone marrow, erythrocytes and skin.
• Recurrent alveolar ulcerations may progress to
gangrene, tooth loss, resolves in puberty.
• Add H2O2 to blood: it turns blackish brown and
the peroxide does not foam
• TX: Antibiotics and dental extractions.
Cyclic Neutropenia
• Decrease of circulating neutrophils
• Every 21 days neutropenia, mouth
ulcerations, fever, malaise, arthralgias.
• Ulcers irregularly outlined and covered with
grayish white slough
• TX: Recombinant Colony Stimulating
Factor, Cyclosporine, Antibiotics for
infections, good dental hygiene.
Recurrent Apthous Stomatitis
Approach to Recurrent Apthous
Stomatitis
• CBC – Anemia, B-12, Folate, Iron, Neutropenia
• GI symptoms or surgeries ? – UC, Crohn’s,
Celiac Dz, Malabsorption (B1, B2, B6)
• Genital or Ocular lesions? – think Behcet’s, or
Reiter’s.
• Risk factors for HIV, AIDS ???
• Tzanck to R/O Herpes, RPR to R/O Syphillis
• Biopsy to rule out pemphigus, LP
RAS - Treatment
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Viscous Lidocaine 2% solution
Dyclonine HCl, 0.5%
50/50 mix of Benadryl and Maalox
Fluocinonide or Triamcinolone in Orabase.
Beconase nasal Spray
TCN 250mg dissolved in 5ml solution
gargled for 2 minutes then swallowed
• Apthasol paste (Amlexanox)
• Dapsone, Colchicine, Thalidomide
Recurrent Intraoral Herpes
Simplex Infection
• Numerous small vesicles in clusters
• Rupture abruptly to form punctate erosions with a
red base.
• Tzanck smear shows multinucleated epithelial
cells.
• MC Palate (Pearl: Herpangina and Apthous
ulcers occur on non-attached mucosa, whereas
recurrent Herpes simplex occurs on mucosa fixed
to bone)
Periadenitis Mucosa Necrotica
Recurrens (Major Apthous Ulcer)
• Sutton’s Dz
• Sharply
circumscribed
ulcer with deeply
punched out and
depressed crater
• Heals with scar
• Tx: IL steroids
Behcet’s Syndrome (Oculo-OralGenital Syndrome)
• Oral ulcers that recur at least 3 times per year
in the presence of any 2 of the following:
• 1) Recurrent genital ulceration
• 2) Retinal vasculitis, Ant./Post. Uveitis
• 3) EN, Folliculitis, Papulopustular, Acneiform
• 4) Positive pathergy test
Behcet’s Disease
• Ulcers are 2-10mm, sharply circumscribed, with a
dirty grayish base and a surrounding bright red
halo.
• Eating may be painful
• Genital lesions are similar
• Ocular lesions start with intense periorbital pain
and photophobia, conjunctivitis
• CNS – multiple sclerosis-like
• GI symptoms due to intestinal ulcerations.
• Thrombophlebitis, Vasculitis, Arthralgia
Pathergy test – pustule forms 3-4 days after needle-stick
Helpful for diagnosis, but usually negative even in the
presence of Behcets’ Syndrome
HISTOLOGY OF BEHCET’S = LCV - A neutrophilic
infiltrate surrounds the superficial capillary plexus in the
papillary dermis, with destruction of small venules. There
is fibrin deposition in the blood vessel walls, transmural
migration of PMNs, extravasated RBCs and nuclear dust
FIBRIN
C-3
IgM
Behcet’s Treatment
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Ulcerations usually heal spontaneously
Oral hygiene – mild toothpastes
Sucralfate suspension
Colchicine 0.6mg bid
Dapsone 100mg daily
Thalidomide 200mg bid x 5 days then 100mg bid
x 15-60 days
• Methotrexate for severe refractory cases
The End