P2-PERIO CHAPTER 18

When was Desquamative Gingivitis first recognized and reported?

1894

What are the clinical characteristics of chronic desquamative gingivitis?

Intense erythema, desquamation, ulceration of free and attached gingiva

What symptoms may occur in chronic desquamative gingivitis?

Mild burning sensation, intense pain

Why was hormonal derangement initially suspected

Most cases diagnosed in women in 4th–5th decades

Name t other autoimmune conditions presenting as DG.

Bullous pemphigoid,

Pemphigus vulgaris

Linear IgA disease

Lichen planus pemphigoides

Dermatitis herpetiformis

Linear IgA disease, Lichen planus pemphigoides, Dermatitis herpetiformis

• Medications, mouthwashes, chewing gum, foreign body gingivitis

What is the use of Nikolsky sign in DG exam?

To assess likelihood of vesiculobullous disorder

What is the best biopsy method for DG?

Incisional biopsy

How should DG biopsy specimens be handled?

Bisected for H&E and immunofluorescence

What fixative is used for H&E?

10% buffered formalin

What fixative is used for immunofluorescence?

Michel’s buffer

What type of disorder is lichen planus?

Inflammatory mucocutaneous disorder

Name three mucosal surfaces affected by lichen planus.

Oral cavity, genital tract, other mucosae

Name two skin sites affected by lichen planus

Scalp and nails

What mediates the pathogenesis of lichen planus?

Host T lymphocytes (immunologically mediated)

: Who is most commonly affected by lichen planus?

Middle-aged or older women (female-to-male ratio 2:1)

Is lichen planus common in children?

Rarely affected

What is the classic presentation of lichen planus in buccal mucosa?

Bilateral, white striae (reticular subtype)

Is the reticular subtype usually symptomatic?

No, it is asymptomatic

Which subtypes are associated with pain and burning sensation?

Atrophic and erosive forms

Can OLP cases may develop into squamous cell carcinoma?

Yes

What is a valuable diagnostic tool for lichen planus?

Direct immunofluorescence (DIF)

What is the common DIF finding in OLP?

Fibrinogen with a shaggy configuration along basement membrane

Name the six clinical forms of OLP

Reticular, Papular, Patch, Atrophic, Erosive, Bullous

What is the most common form of OLP?

Reticular

How does reticular OLP usually present?

Interlacing white lines (Wickham’s striae), bilateral

Where is reticular OLP most common?

Posterior buccal mucosa

Name three other possible sites for reticular OLP.

Tongue, hard palate, alveolar ridge/gingiva

What may cause erythematous background in OLP?

Candidiasis co-infection

What is the clinical course of OLP?

Chronic, with periods of quiescence and unpredictable flare-ups

Which subtype of OLP is often painful?

Erosive

What does erosive OLP look like?

erythematous, ulcerated with radiating striations

Name the four gingival patterns in OLP.

Keratotic, Erosive/ulcerative, Vesicular/bullous, Atrophic

What do keratotic lesions look like?

Raised, white (papules, linear/reticular, plaque-like)

What do erosive/ulcerative gingival lesions look like?

Extensive erythematous areas, patchy hemorrhages

What exacerbates erosive gingival lesions?

Slight trauma like toothbrushing

What do vesicular/bullous gingival lesions look like?

Raised, fluid-filled but rare and short-lived (rupture → ulcerations

What happens in atrophic gingival lesions?

Epithelial thinning and erythema confined to gingiva

Name three classic microscopic features of OLP.

Hyperkeratosis/parakeratosis

hydropic degeneration of basal layer

dense bandlike lymphocytic infiltrate

What configuration may rete ridges show

Sawtooth

What are colloid bodies (Civatte bodies)?

Eosinophilic apoptotic keratinocytes at epithelium-connective tissue interface

Which lesions are best for biopsy?

Keratotic lesions

What does electron microscopy reveal in OLP?

Separation of basal lamina from basal cells

What do lesional/perilesional biopsies show on DIF in OLP?

Linear fibrillar deposits of fibrin at basement membrane

What do immunoglobulin-staining cytoid bodies show IN OLP?

Scattered deposits in upper lamina propria

What condition most commonly mimics OLP?

Lichenoid mucositis

what is the main treatment for erosive/bullous/ulcerative OLP?

High-potency topical corticosteroids

Example of topical corticosteroid for OLP?

0.05% fluocinonide gel (Lidex)

clobetasol propionate

What intralesional steroid is used in severe OLP?

Triamcinolone acetonide (10–20 mg)

What oral systemic steroid may be prescribed?

Prednisone 40 mg daily × 5 days → taper

Name three other systemic treatment options for OLP.

Retinoids, Hydroxychloroquine, Cyclosporine

What immunosuppressants are used in severe OLP?

Azathioprine, Cyclophosphamide

What topical agent is used for recalcitrant erosive OLP?

Tacrolimus 0.1% ointment

Why is tacrolimus useful in diabetic patients?

Avoids hyperglycemia caused by corticosteroids

What is pemphigoid/

A group of cutaneous, immune-mediated, subepithelial bullous diseases involving separation at the basement membrane zone.

Name three conditions included under pemphigoid.

Bullous pemphigoid, Mucous Membrane Pemphigoid (MMP), Pemphigoid gestationis

What is the key feature of all pemphigoid types?

Autoimmune subepithelial blistering

What complicates diagnosis of pemphigoid subtypes?

Histologic and immunopathologic overlap between bullous pemphigoid and MMP

Which condition primarily affects the skin and is nonscarring?

Bullous pemphigoid

Which condition mainly involves mucosa and may cause scarring?

Mucous Membrane Pemphigoid (cicatricial pemphigoid)

What type of disease is bullous pemphigoid?

Chronic, autoimmune, subepidermal bullous disease

How does BP present clinically?

Tense cutaneous bullae → rupture → flaccid lesions

What drugs may induce BP?

Checkpoint inhibitors, DPP4-inhibitors

BP skin lesions resemble which other disease?

Pemphigus (but differ histologically)

What is absent in BP histopathology (unlike pemphigus)?

Acantholysis

Where do vesicles form in BP

Subepithelially at basement membrane

What characterizes immunofluorescence findings in BP?

IgG and C3 immune deposits at basement membrane

What are the common oral manifestations of BP?

Erosive or desquamative gingivitis, occasional bullous lesions

What is the main therapy for BP?

Moderate-dose systemic prednisone

What is MMP?

A chronic vesiculobullous autoimmune disorder of unknown cause

Who is primarily affected by MMP

Women in their 50s; rare in children

Name five sites affected by CICATRICIAL PEMPHIGOID.

Oral cavity, conjunctiva, nose, vagina, rectum, esophagus, urethra (skin in ~20%)

At what level does the split occur in MMP?

Lamina lucida of basement membrane

What are the main antigenic targets in MMP?

BP2, BP1, epiligrin (laminin 5), β4 integrins

What integrin is recognized in ocular pemphigoid?

β4 integrin

What integrin is recognized in oral pemphigoid?

α6 integrin

What is a key complication of ocular MMP?

Symblepharon (eyelid–eyeball adhesion

Name three differential diagnoses of MMP.

Bullous pemphigoid, bullous lichen planus, dermatitis herpetiformis

What is the main treatment for localized MMP disease?

Topical corticosteroids (fluocinonide, clobetasol)

What drug may be used if MMP is resistant to steroids?

Dapsone (monitor for hemolysis/methemoglobinemia in G6PD deficiency)

Name two immunosuppressants combined with steroids in severe MMP.

Azathioprine, Cyclophosphamide

hat are pemphigus diseases?

Autoimmune bullous disorders producing mucocutaneous blisters

Name three other forms of pemphigus.

Pemphigus foliaceus, vegetans, erythematosus

Who is most often affected by PV?

178. Women after the 4th decade (but can occur in children/newborns)

How do PV blisters form?

Damage to keratinocyte adhesion by circulating autoantibodies binding desmosomal glycoproteins

Which glycoproteins are targeted in PV?

Desmogleins (DSG)

Which autoantibody correlates with oral disease severity in PV?

DSG3 autoantibodies

Which autoantibody correlates with cutaneous disease severity in PV?

DSG1 autoantibodies

Name two drugs that can induce pemphigus.

Penicillamine, Captopri

Name two non-drug triggers for pemphigus.

UV radiation, Hepatitis B

Name the most common site for PV oral lesions.

Soft palate (80%)

Name three other oral sites often affected in PV.

Buccal mucosa (46%), tongue (20%), lower labial mucosa (10%)

Are gingival lesions common in PV?

Less frequent, may appear as erosive or desquamative gingivitis

What is the main therapy for PV?

Systemic corticosteroids ± immunosuppressants

Name four steroid-sparing therapies for PV.

Azathioprine, Cyclophosphamide, Cyclosporine, Dapsone

Who is most often affected by CUS?

Women in the fourth decade of life

What sites are most commonly involved in CUS?

Mostly oral, rarely cutaneous

What autoantibodies are found in CUS?

Circulating IgG autoantibodies to ΔNp63α

What is ΔNp63α?

Epithelial nuclear transcription factor that modulates epithelial growth

What do oral lesions of CUS look like?

Painful, solitary, small blisters and erosions with erythema