Oral Lichen Planus: Reticular and Erosive

Dermatologic Diseases Overview

• Dermatologic diseases can manifest in the mouth and are often autoimmune related.
• Important dermatologic diseases to study include lichen planus, pemphigus and pemphigoid.
• Understand histo details.

Lichen Planus

• Presents as a chronic, immunological mucocutaneous disorder.
• Middle-aged female adults are more commonly affected.
• Skin lesions appear as purple, pruritic, polygonal papules.
• Fine, lace-like network of white lines known as Wickham's striae is a characteristic feature.
• Can also appear extraorally on the vulva, glans penis, and nails.
• There are two forms: reticular and erosive.

Reticular Lichen Planus

• May present as papules and is usually asymptomatic.
• Waxes and wanes over weeks or months.
• Dorsal tongue has keratotic plaques.
• More common than the erosive form.
• Posterior buccal mucosa is often affected bilaterally.
• No treatment needed.

Erosive Lichen Planus

• Symptomatic.
• Characterized by atrophic, erythematous areas with central ulceration.
• Atrophy or ulceration confined to the gingiva is known as desquamative gingivitis.
• Has a low potential of becoming SCC.
• Histologically, biopsy reveals perilesional biopsy.

Histopathologic Features of Lichen Planus

• Hyper-ortho and parakeratosis noted.
• Thickness of the spinous layer varies.
• Rete ridges are either absent or hyperplastic, pointed or "saw-toothed."
• Destruction of the basal layer (hydropic degeneration).
• Intense, band-like infiltrate of lymphocytes is located subjacent to the epithelium.
• Degenerating keratinocytes (Civatte bodies) are present.

Diagnosis and Treatment of Lichen Planus

• Reticular form: Diagnose clinically, particularly if classic lesions are present bilaterally on the buccal mucosa.
• Erosive form: More difficult to diagnose clinically.
• Direct immunofluorescent test: Fibrinogen positivity confirms diagnosis
• Reticular form requires no treatment.
• Erosive form is treated with corticosteroids.

Lichenoid Lesions

• Graft-Versus-Host Disease
• Recipients of allogeneic bone marrow transplants are at risk.
• HLA match is not always exact.
• Acute form occurs within first few weeks after bone marrow transplantation.
• 50% of bone marrow patients are affected, with 33-75% oral involvement.
• Chronic form: Continues from the acute phase or develops >100 days post-transplant.
• May appear several years after procedure, >80% of cases have oral lesions.
• Presents as a reticular network of white striae resembling lichen planus.

Clinical Features of Graft-Versus-Host Disease

• Tongue and labial/buccal mucosa is affected.
• Burning sensation present - rule out candidiasis.
• Xerostomia due to immune response to salivary gland tissue.
• Treatment involves careful histocompatibility matching and immunosuppressant drugs.
• Topical corticosteroids may be used.

Lupus Erythematosus Systemic

• Multisystem disease.
• Genetic factors play a role in pathogenesis.
• Chronic Cutaneous Lupus Erythematosus primarily affects the skin and oral cavity.
• More common in females than males; average age is 31.
• Symptoms include fever, weight loss, arthritis, and general malaise.
• 40-50% develop a butterfly rash over the malar area and nose.
• Sunlight exposure often makes lesions worse.
• Kidneys and heart are frequently affected (40-50%), leading to serious sequelae.
• Interface deposition of IgM at the basement membrane.

Oral Manifestations of Lupus Erythematous

• Oral lesions occur in 5-25% of cases, sometimes as high as 40%.
• Palate, buccal mucosa, lips, and gingiva are affected.
• May appear as lichenoid areas.
• Ulceration, pain, erythema, and hyperkeratosis present.

Diagnosis and Treatment of Lupus Erythematosus

• Diagnosis involves clinical, microscopic, and immunologic studies.
• Direct immunofluorescence shows IgM, IgG, or C3 in a shaggy or granular band at the basement membrane zone.
• 95% of patients have antinuclear antibodies (ANAs).
• Acute episodes are treated with systemic corticosteroids, possibly combined with other immunosuppressive agents.

Pemphigus Vulgaris

• It is the most common of pemphigus disorders (Vulgaris is "common" in Latin).
• Oral lesions are often the first sign.
• Blistering occurs due to autoantibodies against desmosomes.
• Immunologic attack causes intra-epithelial split.
• Untreated pemphigus vulgaris often results in death.

Clinical Features of Pemphigus Vulgaris

• 60-70% of initial lesions occur in the oral cavity.
• Adults average age at diagnosis of 50.
• Superficial, ragged ulcerations noted.
• Palate, labial/buccal mucosa, and gingiva are affected.
• Flaccid vesicles/bullae rupture quickly, leaving erythematous, denuded surfaces.
• Bulla can be induced by firm lateral pressure on normal-appearing skin or mucosa (+ve Nikolsky sign).

Histopathologic Features of Pemphigus Vulgaris

• Intraepithelial separation just above the basal cell layer.
• Spinous cells fall apart (acantholysis) and become rounded "Tzanck cells”.
• Biopsy of ulcerated mucosa is often inconclusive due to separation and lack of intact epithelium.
• Confirmed by direct immunofluorescence.
• Perilesional tissue is placed in Michel's solution.
• Antibodies between involved epithelial cells are IgG and C3.
• Indirect immunofluorescence: positive in 80-90% of cases, circulating autoantibodies in serum.

Treatment and Prognosis of Pemphigus Vulgaris

• Systemic corticosteroids are a common treatment, often combined with "steroid-sparing" agents.
• Success is measured by titers of circulating autoantibodies (indirect immunofluorescence).
• Mortality rate is 5-10%, usually due to complications from long-term systemic corticosteroid use.

Mucous Membrane Pemphigoid

• It is a chronic, blistering, mucocutaneous autoimmune disease (cicatrical pemphigoid, meaning "scar").
• Tissue-bound autoantibodies are present against components of the basement membrane (hemidesmosomes).
• Oral lesions do not scar.
• Similar to pemphigus, but differs in prognosis and microscopic features.
• Gingival involvement leads to desquamative gingivitis.
• Desquamative gingivitis is seen in other conditions like erosive lichen planus but concentrated on the gingiva, or pemphigus vulgaris.

Clinical Features of Mucous Membrane Pemphigoid

• Older adults are typically affected, with an average age of 60.
• Females are more affected than males at a 2:1 ratio.
• Oral lesions are present in most patients.
• Blistering can occur in the conjunctiva, nose, esophagus, larynx, vagina, and skin.
• Oral lesions begin as vesicles or bullae.
• Blisters form in the subepithelial location; thicker, stronger roof than pemphigus.
• Blisters rupture, causing large, superficial, ulcerated mucosa.
• Ulcers are painful and persist for weeks to months.

Clinical Complication Features of Mucous Membrane Pemphigoid

• Ocular involvement is the most significant complication (14-25% of cases).
• The conjunctiva becomes inflamed and eroded.
• Scarring develops between the bulbar and palpebral conjunctivae.
• Adhesions called symblepharons result.
• Blindness occurs if not treated.

Histopathologic Features Mucous Membrane Pemphigoid

• Biopsy of perilesional mucosa shows a split between the epithelium and connective tissue.
• Direct immunofluorescence is used to confirm the diagnosis.
• Immunoglobulins (IgG & C3) are seen at the basement membrane zone.
• Indirect immunofluorescence is not helpful.

Treatment and Prognosis of Mucous Membrane Pemphigoid

• Referral recommended to an ophthalmologist.
• Potent topical corticosteroids for oral lesions only.
• Gingival lesions respond to oral hygiene measures.
• If topical steroids fail, use systemic steroids or other immunosuppressive agents.
• Aggressive therapy indicated with advancing ocular disease.

Erythema Migrans (Geographic Tongue)

• Common condition primarily affecting tongue.
• Increased frequency in atopic individuals and patients with psoriasis.
• Usually asymptomatic, but may burn with spicy foods.
• If symptomatic, topical corticosteroids can be used.
• If fissured, antifungals may be used.
• Well-demarcated zones of atrophy of filiform papillae.
• Atrophic areas surrounded by white serpentine borders.
• Lesions heal in a few days or weeks and then develop in another area.
• Infrequently occurs on other sites (ectopic geographic tongue.)

Systemic Sclerosis (Scleroderma)

• Immunologically mediated condition.
• Dense collagen deposited in large amounts.
• Women are affected three times more than men.
• First sign is Raynaud's phenomenon (vasoconstriction).
• Vascular events and abnormal collagen deposition produce ulcers on fingertips.
• Flexion contractures result in claw-like fingers.

CREST Features

• Furrows radiating from mouth - "purse-string" appearance.
• Tongue stiff, board-like
• Esophagus deposition of collagen in , difficulty in swallowing
• Microstomia from collagen deposition in perioral tissues
• Mask like facies subcutaneous collagen deposition in facial skin.
• The autoantibody Anti-Scl-70 (Anti-Topoisomerase I) can be tested for.

Features

• Mild variant of systemic sclerosis.
• Anti-Centromere Antibody (ACA)
• Calcinosis cutis
• Raynaud's phenomenon
• Esophageal dysfunction
• Sclerodactyly
• Telangiectasia
• Small superficially dilated blood vessels

Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Disease)

• It is an autosomal dominant disorder.
• Presents with multiple vascular defects (telangiectasias).
• A presenting symptom is epistaxis (begins in childhood).
• Also presents as Bleeds of the nose.
• Has persistent anemia.
• Displays Cutaneous and oral lesions is Mostly on tongue, lips.

Epidermolysis Bullosa

Genetic,structural proteins, of hemidesmosomes, fibrils

• Attachment defect Epithelial
• 23 EB forms
• Blisters minor trauma
• Epidermolysis Bullosa Autoimmune

Oral Manifestation

• highly variable EB type.
• Increase fragility
• ulceration Lips, mucosa, cheeks, Tongue, palate
• vestibule Obliteration
• Rampant caries, Enamel hypoplasia

Overview of dermatologic diseases manifesting in the mouth, focusing on lichen planus's reticular and erosive forms. Includes symptoms such as Wickham's striae and keratotic plaques. Discusses prevalence, affected areas like the buccal mucosa and tongue, and treatment needs.