IMMUNOPATHOLOGIC DISEASES PART II€¦ · • Clinical: Deep red gingival enlargement, generalized • Microscopic: Diffuse sheets of plasma cells in the submucosal gingival connective

IMMUNOPATHOLOGICDISEASES

PART II

IMMUNOGLOBULIN MEDIATED DISEASES

Plasma Cell Gingivitis

• Hypersensitivity to flavoring agents or resins within chewing gum

• Clinical: Deep red gingival enlargement, generalized

• Microscopic: Diffuse sheets of plasma cells in the submucosal gingival connective tissues

• Tx: Withdraw allergen, topical steroid

Plasma Cell Gingivitis

Immediate HypersensitivityAllergic Stomatitis

• An IgE mediated reaction to foods and drugs• Red lesions and vesicular eruptions, may have

cutaneous lesions as well• Painful, burning• Microscopic: nonspecific inflammation, may

have eosinophils• Common Allergens:

– Penicillin, other antibiotics– Sulfa drugs (erythema multiforme-like lesions)– Shell fish

• Tx: Antihistamines

Allergic Stomatitis

Shellfish allergens

Angio(neurotic) Edema• Antibody Mediated: IgG or IgE complexed with

antigen, facial connective tissues>histamine release, diffuse facial and prominent soft lip swelling– Managed with steroid and antihistamine therapy

• Genetic defect in Complement C1 esterase inhibitor with resultant urticaria of skin and facial edema, ofter precipitated by surgery or trauma

Angioedema

Mucous Membrane Pemphigoid• Differs from Bullous Pemphigoid• Marked Female>Male• Midlife – Elderly• Gingiva• Ig Mediated• Basement Membrane Antigens• DIF – basement Membrane Igs• Sub-basilar Clefting

Mucous Membrane Pemphigoid

Mucous Membrane Pemphigoid

symblepheron

Mucous Membrane PemphigoidHistopathology IgG anti-BM, DIF

SalivaryAnti-BM

Pemphigus Vulgaris

• Midlife-Elderly• Oral lesions precede skin• Can be fatal• Soft Palate/Fauces• Ig Mediated• Desmoglein 3 Target Antigen• DIF – perikeratinocyte membrane

Igs• Suprabasilar Cleft

Pemphigus VulgarisOral Lesions Cutaneous

Pemphigus

Pemphigus vulgaris

Suprabasilar split

Pemphigus VulgarisHistopathology IgG Antibodies - DIF

Erythema Multiforme

• Teens/Young Adults• Hemorrhagic Lip Involvement• Immune Complex Mediated

– Drugs (sulfa)– Postherpetic

• DIF – perivascular complement• Keratinocyte Necrosis

Erythema MultiformeLip Involvement Cutaneous Lesions

EM – Herpetiform Ulcers

Erythema MultiformePerivascular C3, DIF

Erythema Multiforme

EM 1 week post Prednisone

Epidermolysis Bullosa

• Mutation type VII Collagen• Enamel Hypoplasia• Disfigurement• Fatal Outcome

Epidermolysis Bullosa Acquisita

• Skin Lesions• Oral Lesions very rare• DIF – Basement Membrane• Subepithelial Clefting• Target Antigen – type IV collagen

Epidermolysis Bullosa

Epidermolysis BullosaDental Defects Histopathology

Paraneoplastic Pemphigus

• Lymphoma, Castleman’s Disease• Other malignancies• Lesions resemble Erythema

Multiforme• DIF – perikeratinocyte and BM• Antigen – Multiple plaque proteins,

desmoplakins

Paraneoplastic Pemphigus

Paraneoplastic Pemphigus

Perikeratinocyte, BM Igs

Pyostomatitis Vegetans

• Ulcerative Colitis, Crohn’s Disease• Serpentine Erosions• Intraepithelial Eosinophilic Abscesses• DIF – perikeratinocyte membrane Igs• Pyodermatitis Vegetans

Pyostomatitis Vegetans

Pyostomatitis VegetansHistopathology Perikeratinocyte Igs

Systemic Sclerosis (Scleroderma)• Collagen-Immune disease characterized by

progressive fibrosis• Females>Males• Mask-like facies• Limited opening• Dysgeusia (esophageal fibrosis)• CREST syndrome

– Calcinosis, Raynaud’s phenomenon, esophageal stricture, sclerodactyly, Telangiectasia

• Radiographic Features– Widening of the periodontal ligament spaces– Pregonial notching of the mandible

Radiologic Manifestations of SclerodermaAntegonial Notch

Scleroderma, wide PDL

Systemic Sclerosis (Scleroderma)

CREST (calcinosis, raynauds, esophageal stricture, telangiectasia

Orofacial Granulomatosis• Noncaseating granulomatous inflammation• Lesions are of unknown etiology and may be

localized or generalized• Some orofacial granulomas are a component of

systemic disease• Subtypes:

– Sarcoidosis– Crohn’s Disease– Melkerson Rosenthal Syndrome– Cheilitis Granulomatosa

Melkerson Rosenthal Syndrome

Cheilitis granulomatosa

Fissured tongue Oral granulomas

Cheilitis Granulomatosa

Crohn’s Disease• Regional enteritis• Young Adult to Mid Life onset• Ilium/Colonic lesions, segmental, chronic

inflammatory foci in gut wall, some with granulomatous type inflammation

• Diarrhea alternating with constipation, abdominal cramping

• Oral Lesions: Nodular submucosal masses comprised of noncaseating granulomas

Crohn’s Disease

Wegener’s Granulomatosis

• Vasculitis, Fibrinoid Necrosis• Palatal perforation• Gingival “strawberry gums”• Lung, Renal Lesions• ANCA (antineutrophil cytoplasmic Abs)• Potentially Fatal• Chemotherapy (Cytoxan, prednisone)

Wegener’s Granulomatosis

Ulceration“Strawberry Gums”

Nasal Mass

Treatment of Bullous Lesions• Systemic Steroids

– 40-60 mg Prednisone, 7 days, taper for 3– Medrol Dosepak

• Topical Steroid Mouth Rinse– Dexamethasone elixer (0.1mg/ml)

• Topical Steroid Gels.05% Fluocinonide (Lidex gel).05% Clobetasol (Temovate gel).05% Halobetasol (Ultravate gel)

Systemic Steroids

• Medrol Dosepak –recommend two, take both, double doses

• Prednisone 10 mg40-60 mg/day for 7 days20-40 mg/day for 2 days10 mg/day for 2 days