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

• 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)

• 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

• 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

• 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

• 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

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

• 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-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• 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

• 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