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Mucous Membrane
Disorders
Andrews Chapter 34
Michael Hohnadel, D.O.
May 25th, 2004
KCOM/Texas Dermatology
Residency Consortium
Cheilitis Exfoliativa
• Desquamative, mildly inflammatory, recurrent
condition of the lips. Fissures if severe.
• Etiology:
– upper lip: cause is often unknown. Primary disorder.
– lower lip: 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 is most common with
dryness, fissuring, edema, crusting, angular
cheilitis.
• Etiology:
– 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
Allergic Contact Cheilitis
Toothpaste
Actinic Cheilitis
•
•
•
•
Lower lip, UV induced.
Ulceration is rare unless SCC is present
Hereditary PMLE may mimic.
Treatment:
– Biopsy if thickened or ulcerated.
– Cyro, 5-FU, CO2 laser, Vermilionectomy,
Photodynamic Therapy with 5-ALA, H&E
same as AK.
Cheilitis Glandularis
• Presentation: 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 abscess formation.
• Etiology: irritation, atopic, factitious, actinic.
Cheilitis Glandularis
“STICKY LIPS”
TX SAME
AS ACTINIC
CHEILITIS
Cheilitis Glandularis
H&E – infiltration of lymphocytes, histiocytes
and plasma cells in and around the ectatic glands.
Angular Cheilitis (Perleche)
• Etiology: Labial commisures, moist fissures with
overlying Candida albicans infection.
– Elderly – facial and dental architecture
– Youth – thumbsucking, lollipops,
• Other Inciting factors: 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, Sealing agents.
Last resort is excision, flap.
Angular Cheilitis (Perleche)
Plasma Cell Cheilitis
• Sharply outlined, infiltrated, dark red plaque
with a laquer-like glazing of the surface of
the lower lip
• Reaction pattern to any number of stimuli
• Histology:
– Similar to Zoon’s balanitis plasmacellularis
– Band-like infiltrate of plasma cells
• Treatment: Clobetasol propionate bid,
Griseofulvin 500mg qd.
Plasma Cell
Cheilitis
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
• Mackie reports 7 Patients with Ulcers of lower lip.
Dc oral meds and resolved.
• May be confused with ulcers of DLE or SCC
• Offending agents:
– Phenylbutazone, Chlorpromazine, Phenobarbital,
Methyldopa, Thiazide diuretics.
• Fixed-Drug/Photoreaction ?
Other forms of Cheilitis
•
•
•
•
Lichen Planus
SLE
Psoriasis
Lip Biting
Oral Crohn’s Disease
• 10-20% of Crohn’s patients. Assoc with active
esophageal and anal involvement.
• 90% have granulomas on biopsy
• Presentation: Inflammatory hyperplasia of oral
mucosa, cobblestoning, fissuring, metallic
dysgeusia, gingival bleeding.
• Metastatic Crohn’s – non-caseating granulomatous
skin lesions in patients with Crohn’s.
Oral Crohn’s Disease
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 or other
inflammatory bowel disease.
• Presentation: 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 may occur and favor: axilla, groin, and
scalp.
– Appear as crusted erythematous papulopustules that coalesce
into asymmetrical annular plaques.
Pyostomatitis Vegetans
Red – edema
Black – perivascular infiltrate
Blue – abscess formation with
eosinophils
TX – Systemic Steroids
Cheilitis Granulomatosa
• Sudden onset of lip swelling that progresses
to permanent lip enlargement.
• Upper lip usually swells first.
• Cause unknown
• Histology shows tuberculoid granulomas
and an inflammatory reaction pattern
• TX- IL steroids and surgical repair.
Pathology – tuberculoid granulomas with
epithelioid and Langerhan’s giant cells
Melkersson-Rosenthal Syndrome
•
Classic Triad (starts in adolescence)
1. Edema with lip enlargement (other areas may swell too)
2. Scrotal Tongue
3. Recurring facial paralysis (transient or permanent)
•
•
•
Pathology similar to Cheilitis Granulomatosa
R/O Ascher Syndrome – lip swelling, edema of
eyelids (blepharochalasis)
Treatment: IL Steroids, Surgical nerve
decompression, cosmetic surgery for lip reduction,
Clofazimine, Thalidomide
MelkerssonRosenthal
Syndrome
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.
Stomatitis Nicotina
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 (very
common), Pachyonychia Congenita,
Pemphigus Vegetans, Cowden’s Syndrome.
• Treatment: None required. Keep clean.
Scrotal Tongue
Geographic Tongue
• Usually isolated finding. May be
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
Geographic Tongue
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
• Etiology: smoking, oral antibiotics, Candida
• Histology: elongated and stratified filaments
composed of ortho and parakeratotic cells.
• TX: toothbrush, tretinoin, 40% urea, stop
predisposing factors.
Black Hairy Tongue
Black Hairy Tongue
Benign hyperplasia
of the filiform
papillae
Moller’s Glossitis
• Intensely red, well defined
irregular patches in which the
filiform papillae are absent and
thinned and the fungiform papillae
are swollen.
• Tip and lateral tongue. Tongue
may become smooth and glazed.
• Painful, chronic and makes eating
difficult
• Check for macrocytic
anemia, if present then
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
• Presentation: Shiny, oval or diamond shaped
elevation, midline, directly in front of the
circumvallate papillae.
• Candida species may be present. No assoc. with
cancer.
• Histology: Chronic inflammation with fibrosis
with occasional hyphae in areas of parakeratosis
• Itraconazole helpful.
Median Rhomboid Glossitis
Eosinophilic Ulcer of the Tongue
• Ulcer with elevated borders usually covered by a
pseudomembrane.
– Most common on posterior aspect of tongue
– Rapid onset, spontaneously resolves in a few weeks.
• Benign, self-limited.
• Etiology: Likely Trauma.
• Histology: Predominantly eosinophilic infiltrate
with histiocytes and neutrophils
• If multifocal and recurrent, CD30 +
lymphoproliferative disease may be present.
Eosinophilic
Ulcer of the
Tongue
Caviar Tongue
• Small round purplish
capillary telangiectasias
• Commonly found on
underside of tongue
after age 50
• Etiology: elastic tissue
deterioration
Dental Sinus
• Tooth abscess forms a sinus
tract that opens on the skin
as an inflamed nodule. May
palpate a cord-like tract
beneath the lesion
• Chin or jawline.
• Dental X-Ray diagnostic
• DDX: SCC, Actinomycosis,
osteomyelitis, deep fungal,
foreign body
Dental Sinus
Leukoplakia
• Once regarded as
pre-cancerous
• 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.
• Most common 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
Erythroplakia
Proliferative Verrucous
Leukoplakia
• Flat white areas on
mucous membranes that
thicken and become
exophytic
• 70% become SCC
• F > M is 4:1
• Assoc with HPV 16
• Aggressive early
therapy is best.
Squamous Cell Carcinoma
• 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
due to late discovery.
Melanocytic Oral Lesions
• Melanocytic nevi frequency by type: Intramucosal
> 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%.
• Oral Melanoma - Rare, mostly in elderly patients.
Bleeds easily, irregular shape, periphery of
erythema or satellite lesions may be present.
LABIAL MELANOTIC MACULE
Melanoacanthoma
Variant of pigmented
SK. Melanocytes not
restricted to basal layer
Oral Melanosis
•
•
Most common in African Americans
Oral melanosis is associated with:
1. McCune Albright Syndrome (dimple over 4th knuckle,
Coast of Maine border, unilateral café au lait macule
with bony abnormalities below it)
2. Peutz-Jeghers (polyposis, colon cancer)
3. Addison’s Disease
•
Other causes of oral melanosis: Tar, Heavy metal
poisoning, dental amalgams. Cis-platinum can
causes a 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
• Probably 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
• Assoc
Pregnancy.
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
• Most common 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 2nd to
biting.
• Soft rounded
translucent
projection often
with a bluish tint.
• TX: excision.
Acute Necrotizing Ulcerative Gingivostomatitis
(Trench Mouth, Vincent’s Disease)
• “Punched out” ulcerations
on interdental papillae and
marginal gingivae. Rapid
onset with pain and foul,
fetid odor. Erodes
gingivae.
• Bacteroides fusiformis &
Borrelia vincentii.
• TX: PCN, 3% H2O2
mouthwash, debridement
• R/O herpes infection – not
primarily gingivae, no
necrosis.
NOMA
• Means “to devour”
• Gangrenous. Starts in the
mouth as a benign oral lesion
and rapidly destroys tissues of
the mouth and face. < 6 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”
• Rare -Autosomal Recessive. 1 in 100,000 in
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
• Pathology: Decrease of circulating
neutrophils
– Every 21 days neutropenia, mouth ulcerations,
fever, malaise, arthralgias.
• Ulcers irregularly outlined and covered with
grayish white slough on mucosal surfaces.
• Cause is unknown.
• TX: Recombinant Colony Stimulating
Factor, Cyclosporine, Antibiotics for
infections, good dental hygiene.
Recurrent Aphthous Stomatitis
Approach to Recurrent Apthous
Stomatitis
• GI symptoms or surgeries ? – UC, Crohn’s, Celiac Dz,
Malabsorption (B1, B2, B6)
• Genital or Ocular lesions? – Behcet’s, or Reiter’s
syndromes.
• Evaluate risk factors for HIV, AIDS.
• Laboratory:
– CBC for anemia, B-12, Folate, Iron, Neutropenia.
– Tzanck to R/O Herpes, RPR to R/O syphillis
– Biopsy to rule out pemphigus, LP
Recurrent Aphthous Stomatitis
Treatment
Symptomatic treatment:
–
–
–
–
–
–
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)
Prevention:
– 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.
• Palate is most common. (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 Aphthous Ulcer)
• Sutton’s Dz
• Sharply circumscribed
ulcer with deeply
punched out and
depressed crater
• Heals with scar.
• Freq.assoc with HIV.
• Tx: IL steroids. If
frequent then colchicine
or dapsone.
Behcet’s Syndrome
(Oculo-Oral-Genital 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
• Oral:
– Ulcers are 2-10mm, sharply circumscribed, with a dirty
grayish base and a surrounding bright red halo.
• Genital lesions:
– Similar to oral lesions. Appear on genitals, perineum, anus
or rectum.
• Ocular lesions:
– Start with intense periorbital pain and photophobia,
conjunctivitis. Retinal vasculitis may lead to blindness.
Also glaucoma and cataracts.
• CNS:
– Multiple sclerosis-like.
• GI symptoms: due to intestinal ulcerations.
• Others: thrombophlebitis, vasculitis,Polyarthralgia
Behcet’s Disease
• Course: Starts with oral ulcerations and
over time (often years) others symptoms
develop.
• Incidence: Relatively high prev in Far East
and Mediterranean. Less common in U.S.
• Pathology: LCV
Pathergy test – Inject 0.1 ml of NS or just prick the skin. Pustule forms
24 hours 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 vennules. There
is fibrin deposition in the blood vessel walls, transmural
migration of PMNs, extravasated RBCs and nuclear dust
FIBRIN
C-3
IgM
Behcet’s
Disease
Behcet’s Treatment
Treatment of ulcers:
•
•
Ulcerations usually heal spontaneously
Oral hygiene – mild toothpastes, limited
toothbrushing.
Prevention of attacks:
•
•
•
•
•
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