Sjögren syndrome: xerostomia Lupus erythematosus: white, erosive lesions of the oral mucosa Pemphigus vulgaris: mucosal ulceration, bullae Benign mucous membrane pemphigoid: mucosal ulceration, desquamative gingivitis Behçet syndrome: aphthous ulcers Pernicious anemia: mucosal atrophy, mucosal ulceration, loss of filiform and fungiform papillae
Sjögren syndrome (1 of 2)
Autoimmune disease that affects salivary and lacrimal glands, resulting in decreased saliva and tears. This is combo is sometimes called sicca syndrome. Eye damage is called keratoconjunctivitis sicca. Other lubrication glands can be affected. Both types of immunity are involved. 50% with Sjögren syndrome have another autoimmune disease. Lacrimal/salivary gland involvement without other autoimmune = primary. With another autoimmune = secondary Oral manifestations: dry mouth, mucosa becomes red
Sjögren syndrome (2 of 2)
Histologic appearance: gland replacement by lymphocytes, presence of epithelial islands called epimyoepithelial islands. Minor glands show lymphocyte aggregates around ducts. Xeropthalmia... burning, itching of eyes. Raynaud phenomenon. Laboratory: positive reaction to rheumatoid factor (antibody to IgG). Diagnosis: when 2 of 3 present (xerostomia, keratoconjunctivitis, rheumatoid arthritis/autoimmune) Tx=NSAIDs. Corticosteroids/immunosuppressives for extreme
Systemic Lupus Erythematosus (SLE) (1 of 2)
Acute/chronic inflammatory autoimmune disease of unknown cause. Women 8x more than men. Wide spectrum of activity, multiple organ involvement. Both cellular and humoral immunity are impaired. Antigen/antibody complexes are deposited in various organs. Autoantibodies for DNA in serum. Skin lesions, arthritis, problems with CNS, lungs, heart, kidneys. Oral lesions: erythematous plaques, resemble lichen planus.
Systemic Lupus Erythematosus (SLE) (2 of 2)
Diagnosis: antinuclear antibodies in serum, histologically: like lichen planus, microscopically: destruction of basal cells (like lichen planus). Inflammatory infiltrate is distributed around blood vessels. Deposits of immunoglobulins around basement membrane. Tx depends on disease activity. Treated with NSAIDs, hydroxychlorquinone (an antimalarial), and corticosteroids.
Pemphigus Vulgaris (1 of 2)
Severe progressive autoimmune disease that affects skin and mucous membranes. Characterized by breakdown of cellular adhesion between epithelial cells: acontholysis. Patient has antibodies active against cell attachment. Often seen in Ashkenazi jews. First signs are in oral cavity. Oral lesions: shallow ulcers to fragile vesicles or bullae. Bullae are fragile and rupture. Nikolsky's sign.
Pemphigus Vulgaris (2 of 2)
Microscopic exam: intact basal layer of epithelium. Detached cells are closer to surface and are rounded, called acontholytic cells or Tzanck cells Diagnosis: made by biopsy, microscopic exam, direct/indirect immunofluorescence. Direct immunofluorescence shows fluorescence surround cells of prickle layer. Indirect immunofluorescence shows presence of circulating autoantibodies. Tx= High doses of corticosteroids/other immunosuppressive drugs like methotrexate.
Chronic autoimmune disease that affects the oral mucosa, conjunctiva, genital mucosa, and skin. Not as severe as pemphigus. Heals with scarring. Lesions occur as epithelium detaches from connective tissue (no basal layer). Most common site is gingiva: desquamative gingivitis. Ranges from erythema to ulceration. Nikolsky's sign.
Diagnosis: by biopsy/histologic exam. Direct immunofluorescence shows linear fluorescence at basement membrane. Tx= Mild cases: Topical corticosteroid. Severe cases: Systemic corticosteroids.
Eighty percent of patients are older than 60. Circulating autoantibodies are usually detectable, but do not relate to disease activity. Oral lesions are less common. Microscopic appearance shows epithelium separating from connective tissue at the basement membrane. Tx= high doses of corticosteroids/NSAIDs.
Chronic, recurrent, autoimmune disease, primarily of oral ulcers, genital ulcers, and ocular inflammation. Onset is at 30 years of age. Incereased in those of Mediterranean, Assian descent. Markers HLA B5 and HLA B51. Oral ulcers are similar to aphthous ulcers, genital lesions are small, ocular lesions begin with photophobia. Diagnosis requires 2 of the 3 manifestations (oral/genital/ocular). Pustular lesion develops after needle puncture. Tx=topical corticosteriods/other immunosuppressives.
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