Desquamative gingivitis

Tanushree Bera
Department of Periodontics
Army College of Dental Sciences
 CONTENTS
 Introduction
 History
 Classification
 Disorders associated with desquamative gingivitis
• Lichen planus
• Pemphigoid
• Pemphigus
• Lupus erythematosus
• Erythema multiforme
• Miscellaneous
 Conditions mimicking desquamative gingivitis
 Diagnostic pathways
 Clinical significance and implications
 Conclusion
 References
 INTRODUCTION
• The term “desquamation” is derived from the Latin word
‘Desquamare’, which means scraping fish flakes. As a word,
desquamation means ‘loss of epithelial elements in small and large
amounts, peeling of skin, and exfoliation’

• Desquamative gingivitis is the clinical term given to the gingival

manifestation of mucocutaneous diseases.

• Desquamative gingivitis (DG) is characterized by erythematous

gingiva, desquamation and erosion of the gingival epithelium, and
blister formation.
• This condition is non-plaque associated and may be localized to the
marginal gingiva, the attached gingiva or both.

• It is not a specific diagnosis, but rather a sign of the underlying

disease.

• It is more common in middle-aged to elderly females.

• Its clinical appearance is not significantly altered by traditional oral

hygiene measures or conventional periodontal therapy alone.
 HISTORY
• Chronic desquamative gingivitis was first described by Tomes and
Tomes in 1894.
• The term “Desquamative gingivitis” was coined by Prinz in 1932. It
is a descriptive term synonymous with the presence of erythema,
desquamation, erosion, and blistering of the attached and marginal
gingiva.
• Schour and Massler (1947) used the term ‘Gingivosis’ to describe
this condition in a group of malnourished Italian children.
• Initially, it was thought to be a specific degenerative disease of
gingival tissue with unknown etiology.
• In 1960 McCarthy and colleagues suggested that DG was not a
specific entity, but a gingival response associated with a variety of
conditions.
• This was further concluded by Glickman and Smulow in 1964 -
mild, moderate and severe forms.
• Nisengard and Levine (1995) cited the following as the standard
for the clinical appearance of DG:
• 1) Gingival erythema not resulting from plaque,
• 2) Gingival desquamation,
• 3) Other intraoral and sometimes extraoral lesions, and
• 4) Complaint of sore mouth, particularly after eating spicy foods.

• Nikolsky's sign often shows a positive reaction in patients with DG

(Fig. 1). This sign involves the application of a shearing force on
normal-appearing gingiva, producing epithelial desquamation.
A. Mild form B. Moderate form C. Severe form
Diffuse erythema. •Patchy distribution of •Scattered, irregularly
Condition is bright red and gray shaped areas -striking red
painless Age: 17 – areas. appearance.
23 years common •Surface is smooth and •areas is grayish blue giving an
in females. shiny and soft in overall speckled appearance.
consistency. •Surface epithelium - shredded
•Slight pitting on and friable and can be peeled
pressure. off in small patches.
•Nicolsky’s sign +ve •Areas of involvement seem to
•Remainder of the shift to different locations on
mucosa is also extremely the gingiva.
smooth and shiny. •Patient cannot tolerate
Age: 30 – 40 years. coarse food or temperature
changes.
C/o of burning sensation •Constant dry and burning
and sensitivity to sensation throughout the
thermal changes. oral cavity,
Diseases presenting clinically as desquamative gingivitis

Dermatological/ Mucocutaneous lesions

•lichen planus
•pemphigoid
•pemphigus vulgaris
•chronic ulcerative stomatitis
•linear iga disease (linear iga dermatosis)
•dermatitis herpetiformis
•lupus erythematosus
•erythema multiforme

Drug induced oral reactions

Allergic reactions

Endocrine disturbances

Ageing

Chronic infections

Other causes
Chronic desquamative gingivitis

Describe a peculiar condition characterized by intense erythema, desquamation,

and ulceration of the free and attached gingiva.

Desquamative gingivitis was not a specific disease entity, but a

gingival response associated with a variety o f conditions.

General information:
•Patients may be asymptomatic.

•when symptomatic range from a mild burning sensation to an intense

pain.

•Approximately 50% of desquamative gingivitis cases are localized to the

gingiva, although involvement of the gingiva plus other intraoral and even
extraoral sites is common.

•Most cases were diagnosed in women in the fourth to fifth decades

of life
ETIOPATHOGENESIS
Other conditions - Rare
Diagnosis of desquamative gingivitis
Clinical History.

A thorough clinical history is mandatory to begin the assessment of

desquamative gingivitis.

Data regarding the symptomatology associated with this condition, as well as

its historical aspects (i.e., when did the lesion start, has it gotten worse, is
there a habit that exacerbates the condition, etc.).

Previous historyof treatment

Clinical Examination
Recognition of the pattern of distribution of the lesions (i.e., focal or
multifocal, with or without confinement to the gingival tissues).

Nikolsky's sign offers insight into the plausibility of the presence of a

vesiculobullous disorder.

Biopsy
 General clinical features
• Predominantly affects women
(Prinz 1932, Meritt 1993)

• Occurs in 4th – 5th decade

• Red, swollen & glossy gingiva

• Multiple vesicle
and superficial
denuded areas

• Difficult to eat hot, spicy food

• Nikolsky’s sign may be

positive.
 HISTOPATHOLOGY
• Microscopically, dg often
appears as a bullous lesion or
lichenoid lesion.
• Occasionally, there will be thin
atrophic epithelium with little or
no keratin at the surface and a
dense diffused infiltration of
chronic inflammatory cells in the
connective tissue.
• Histochemical and
ultrastructural studies revealed
separation of collagen fibrils and
a decrease in the no. of
anchoring fibrils
•Differential diagnosis

•Lichen planus
•pemphigoid
•pemphigus vulgaris
•chronic ulcerative stomatitis
•linear iga disease (linear iga dermatosis)
•dermatitis herpetiformis
•lupus erythematosus
•erythema multiforme
Lichen Planus

•Lichen planus is a chronic autoimmune, mucocutaneous disorder, characterized

by the presence of cutaneous papules that may coalesce to form plaques.

•The current evidence suggests that lichen planus is an immunologically

mediated mucocutaneous disorder where host T lymphocytes play a central role.
Ishii et al, 1987

• Female : Male – 2:1

•Oral lichen planus lesions follow a chronic course and have alternating,
unpredictable periods of quiescence and flares.

Types of Lichen planus:

•Reticular
•Atrophic.
•Erosive
•Bullous.
• Small, angular, flat-topped papules only a few millimeters in
diameter

• Wickham’s striae – fine, grayish-white lines covering the papules.

• Lesions-bilaterally symmetrical pattern, commonly involving buccal

mucosa, gingivae and dorsum of the tongue.

• Lichen planus may also occur concurrently or independently in the

skin and the genital, anal, esophageal, nasal and laryngeal mucosae.

• Female 40-49years, male – 50-59 years

• Commonly involved sites (skin)-

▫ Flexor surfaces of wrist
▫ Inner aspect of knees & thighs
▫ Trunk
 Etiology
Malnutrition
and infection
Traumatism Autoimmune

Stress Hereditary

Idiopathic
Lichen Grinspans
planus syndrome
Skin lesions of lichen planus. Papules with delicate white striations.
Four types of gingival lesions:

1. Keratotic lesions. These raised white lesions may present as groups of

individual papules, linear or reticulate lesions, or plaque like configurations.

2. Erosive or ulcerative lesions. These extensive erythematous areas with

a patchy distribution may present as focal or diffuse hemorrhagic areas. These
lesions are exacerbated by slight trauma.

3. Vesicular or bullous lesions. These raised, fluid-filled lesions

are uncommon and short lived on the gingiva, quickly rupturing and leaving
an ulceration.

4. Atrophic lesions. Atrophy of the gingival tissues with ensuing epithelial

thinning results in erythema confined to the gingiva.
A variety of clinical appearances is characteristic of OLP. These
include:

• Papular.
• Reticular.
• Plaque-like .
• Atrophic.
• Ulcerative.
• Bullous.
Erosive lichen planus
Papular lesion of right buccal
mucosa.
 Reticular lesion of lower lip
mucosa. The white striations
are denoted Wickham striae.
Reticular lesion
Plaque-type lesion of
maxillary
gingiva.
Atrophic lesions of facial maxillary and mandibular gingiva.
 Atrophic and
reticular lesion of
lower left canine
region.
Bullous/reticular lesion of left
palatal mucosa.
Histopathology Direct immunofluorescence

Indirect immunofluorescence
Treatment for lichen planus:

The keratotic lesions of oral lichen planus are asymptomatic and do

not require treatment once the microscopic diagnosis is established.

Follow-up of the patient every 6 to 12 months is warranted to

monitor clinical changes and the emergence of an erosive
component.

In contrast, the erosive, bullous, or ulcerative lesions of oral lichen

planus are treated with high-potency topical steroid such as:
• 0.05% fluocinonide ointment (Lidex, three times daily).
•Lidex can also be mixed 1:1 with carboxymethyl cellulose (Orabase)
paste or other adhesive ointment.
•Intralesional injections of triamcinolone acetonide (10 to 20 mg) .
• Topical calcineurin inhibitors (TCIs) represent an additional topical
treatment for DG. TCIs can be delivered via gel, cream, or as a
suspension to swish and spit, created by dissolving a 1-mg capsule
into a liter of water.24 TCIs have demonstrated efficacy in patients
with OLP, with comparable results to topical corticosteroids.

• Tacrolimus(0.1% Protopic ointment, twice daily), is an

immunosuppressant - effective for controlling the lesions of erosive
lichen planus.

• Short-term regimens of 40 mg prednisone daily for 5 days followed

by 10 to 20 mg daily for an additional 2 weeks have also been
employed in more severe cases.
Pemphigoid
PEMPHIGOID
The term pemphigoid applies to a number of cutaneous, immune-mediated,
subepithelial bullous diseases that are characterized by a separation of the
basement membrane.

Bullous pemphigoid, mucous membrane pemphigoid and pemphigoid.

Among these conditions, bullous pemphigoid and mucous membrane
pemphigoid (also known as benign mucous membrane pemphigoid and
cicatricial pemphigoid)

Bullous pemphigoid
is preferred when the disease is nonscarring and mainly affects the skin.

Cicatricial pemphigoid is favored when scarring occurs and the disease

is mainly confined to mucous membranes
Bullous Pemphigoid

Oral lesions.

There is an erosive or desquamative gingivitis presentation and

occasional vesicular or bullous lesions.

Oral involvement – 1/3 of the patients

Histologically,
• No acantholysis
• Subepithelial vesicles
• Epithelium separation at th BMZ
• Two major antigenic determinants

180-kDa collagen-
230- kDa protein
like transmembrane
plaque BP1
protein BP2
• Immunofluoroscence
• immunoglobulin G (IgG) and complement 3 (C3) immune deposits
along the epithelial basement membranes and circulating IgG
antibodies to the epithelial basement membrane.

• Direct IF – 90-100% positive

• Indirect IF – 40-70% positive
• Affects males and females in equal numbers
• Mostly affects the elderly

Treatment:
• Control its signs and symptoms.
• The primary treatment is a moderate dose of systemic prednisone.
• Steroid-sparing strategies (i.e., prednisone plus other
immunomodulatory drugs) are used when high doses of steroids are
needed or when the steroid alone fails to control the disease
• Localized lesions of bullous pemphigoid, high-potency topical
steroids or tetracycline with or without nicotinamide can be
effective .
Mucous Membrane Pemphigoid (Cicatricial Pemphigoid).

Effects women in 50s, and young children.

Ocular lesions.
In cases presenting first to the dentist (mainly desquamative gingivitis), the eyes
are affected in approximately 25% of the patients (Syblephharon)

Oral lesions. The most characteristic feature of oral involvement is the presence
of desquamative gingivitis with typical areas of erythema, desquamation,
ulceration
 Cicatricial
pemphigoid
BP 1, BP2,
laminin 5 and 6, Production of Complement
uncein, autoantibodies activation
α6β4integrin

Sequestration of
Cytokine release
leukocytes

Release of
proteases,
Blister formation
collagenases,
elastases
Unilateral conjunctivitis that becomes bilateral within 2 years.
Subsequently, adhesions of the eyelid to the eyeball (i.e. symblepharon)
may form.
Adhesions at the edges of the eyelids (i.e., ankyloblepharon) may
lead to a narrowing of the palpebral fissure.
Small vesicular lesions may develop on the conjunctiva, which can
eventually produce scarring, corneal damage, and blindness.
 Desquamative gingivitis, typically with
areas of erythema, desquamation,
ulceration, and vesiculation of the
attached gingiva.

Vesiculobullous lesions can occur

elsewhere in the mouth. The bullae
tend to have a relatively thick roof
and rupture in 2 to 3 days after
formation, leaving irregularly
shaped areas of ulceration

Healing of these lesions can take 3

weeks or more
 Histopathology
• subepithelial vesiculation
• intact basal layer
• Separation of the epithelium
and the connective tissue
occurs at the basement
membrane zone.
• Electron microscopic studies
demonstrate a split in the
basal lamina.
• A mixed inflammatory
infiltrate (i.e., lymphocytes,
plasma cells, neutrophils, and
scarce eosinophils) is
observed in the underlying
fibrous connective tissue.
Treatment

• Topical steroids are the mainstay of treatment for mucous membrane

pemphigoid. when localized lesions are present.

•Fluocinonide (0.05%) and clobetasol propionate (0.05%) in an adhesive vehicle

can be used three times a day for up to 6 months.

•topical corticosteroids are effectively delivered with vacuum-formed custom

trays or veneers

•Optimal oral hygiene is essential because local irritants on the tooth surface
result in an exaggerated gingival inflammatory response.

•Gingival irritation from any dental prosthesis should also be minimized.

•Ocular involvement exists, systemic corticosteroids are indicated.

•When lesions do not respond to steroids, systemic Dapsone (4-

4'diaminodiphenylsulfone) has proven to be effective

•Systemic steroids can be combined with azathioprine or cyclophosphamide

 Pemphigus Vulgaris

The pemphigus diseases are a group of autoimmune bullous disorders that

produce cutaneous and/or mucous membranes blisters. (Intraepithelial bulla)

Oral Lesions

Oral lesions of pemphigus range from small vesicles to large bullae. When the
bullae rupture, they leave extensive areas of ulceration

The soft palate is more often involved (80%).

Followed by the buccal mucosa (46%)
ventral aspect or dorsum of tongue (20%)
and lower labial mucosa (10%).
Histopathology.

Lesions of pemphigus demonstrate a characteristic intraepithelial separation,

which occurs above the basal cell layer.

The intraepithelial vesiculation begins as a microscopic alteration.

Oral lesions of pemphigus vulgaris

•Erosive lichen planus,

•Pemphigoid linear IgA
•chronic ulcerative stomatitis
Treatment of pemphigus vulgaris:

•Is systemic corticosteroid therapy with or without the addition of other

immunosuppressive agents.

•They consist of combinations of steroids plus other medications such as

azathioprine, cyclophosphamide, cyclosporine, dapsone, gold, methotrexate,
photoplasmaphoresis, and plasmaphoresis.
Chronic ulcerative stomatitis

Clinically, this condition presents with chronic oral ulcerations and has a
predilection for women in their fourth decade of life.

The erosions and ulcerations present predominantly in the oral cavity, with only
few cases exhibiting cutaneous lesions.

Oral Lesions.
Painful, solitary small blisters and erosions with surrounding erythema are
present mainly on the gingiva and the lateral border of the tongue.

The hard palate may also present similar lesions.

Treatment.
For mild cases, topical steroids (fluocinonide, clobetasol propionate) and topical
tetracycline may produce clinical improvement; however, recurrences are common.

For severe cases, a high dose of systemic corticosteroids is needed to achieve

remission.
Linear IgA disease (LAD)
Predilection in women.
Drug induced – ACE inhibitors (Femiano et al 1995)
Mucosal involvement – 50-100%
Pruritic vesiculobullous rash.
Mimics lichen planus.
Immunofluroscence assay required
IgA autoantbodies against BMZ antigens BP180 and BP230, LAD 285 and
collagen VII

Oral Lesions.

•Vesicles, painful ulcerations or erosions, and erosive gingivitis cheilitis.

•The hard and soft palates are more commonl affected.

•Tonsillar pillars, buccal mucosa, tongue, and gingiva follow in frequency.

Characteristic skin lesions –
‘string of pearls’ sign
• Histopathology: similar to erosive lichen planus
• Direct immunofluorescence
• Management:
• General – wound care, prevent secondary
infection
• Mild : Topical steroids
• Moderate-severe : Dapsone (0.5mg/kg -
1mg/kg) c
• Erythromycin (in hildren)
• Other drugs : tetracycline, colchicine,
sulfamethoxypyridazine, prednisolone,
azathioprine, mycophenolate mofetil

• D/D: erosive lichen planus, chronic

ulcerative stomatitis, pemphigus vulgaris,
bullous, pemphigoid, and lupus
erythematosus.
Lupus Erythematosus.
Systemic ,Chronic cutaneous, and subacute cutaneous.

Systemic Lupus Erythematosus.

Females 10:1

Vital organs : kidneys heart, skin and mucosa.

Extra-oral symptoms and signs:

•Fever.
•weight loss.
• Arthritis.
•Rash on the malar area with a butterfly distribution.
• Oral lesions
• Similar to lichen planus
• Ulcerations 36%
• Hyperkeratotic plaque 4%

Antinuclear antibodies (ANA) are present in more than 95% of cases

Histopathology
• Immunofluorescence:
• Direct immunofluorescence of the perilesional and normal tissue
reveals immunoglobulins and C3 deposits at the dermal-epidermal
interface.
Treatment.
Cutaneous rashes are treated with topical steroids, sunscreens, and
hydroxychloroquinine.

Arthritis and mild pleuritis, - nonsteroidal anti-inflamatory agents

Severe cases of SLE – prednisone

Immunosuppressive drugs (e.g., cytotoxic agents such as

cyclophosphamide and azathioprine) and plasmapheresis alone or with
steroids

Rituximab
Erythema Multiforme

Genesis of ulcerative lesions affecting the skin

immune complex vasculitis.

complement fixation

leukocytoclastic destruction of vascular walls and small

vessels occlusion

ischemic necrosis of the epithelium and

underlying connective tissue
The three most common etiologic factors:
(1) herpes simplex infection,
(2) mycoplasma infection
(3) drug reactions.

Drugs : sulfonamides,
penicillins, quinolones, chlormezanone, barbiturates, oxicam
nonsteroidal antiinflammatory drugs, anticonvulsant drugs,
protease inhibitors, and allopurinol

Erythema multiforme

Erythema multiforme
Erythema multiforme
major (Stevens-
minor (Mild)
Johnson syndrome)
Target or "iris" lesions with central
clearing, are the "hallmark“ of
erythema multiforme
Oral Lesions.
•Multiple,large, shallow, painful ulcers with an erythematous border.
•Painful - chewing and swallowing are impaired.
•The buccal mucosa and tongue are the most commonly affected sites, followed by
the labial mucosa.
•Less commonly affected are the floor of the mouth, hard and soft palate, and the
gingiva.
•Rare -lesions confined exclusively to the gingival tissues - clinical diagnosis of
desquamative gingivitis.
.
Histopathology.
Treatment.

•No specific treatment - may resolve spontaneously.

•Patients exhibiting bullous or ulcerative lesions require intervention.

•For mild symptoms, systemic and local antihistamines coupled to topical

anesthetics and debridement of lesions with an oxygenating agent is adequate.

• In patients with severe symptoms, corticosteroids are considered the drug of

choice although, its use is controversial and not completely accepted.
DRUG REACTIONS

•Stomatitis medicamentosa.

•Stomatitis venenata or contact stomatitis.

Lichenoid contact lesion of left buccal mucosa due
to type IV hypersensitivity to mercury.
Diffuse gingivitis and cheilitis due to
contact allergy to flavor additive in
dentifrice.
•Pyrophosphates and flavoring agents.

•Oral reactions to cinnamon compounds - intense erythema of the attached gingival

tissues characteristic of plasma cell gingivitis.

•A thorough clinical history.

•Elimination of the offending agent (e.g., tartar control toothpaste) leads to

resolution of the gingival lesions within a week.

•Challenge with the offending agent leads to recurrence of the oral lesions.
Miscellaneous

•Factitious lesions.
•Candidiasis.
•Graft vs. host disease.
•Wegener's granulomatosis.
•Foreign body gingivitis.
•Kindler syndrome.
•Squamous cell carcinoma.