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LUPUS ERYTHEMATOSUS Dr. Deepak K. Gupta

Lupus erythematosus

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  • LUPUS ERYTHEMATOSUS Dr. Deepak K. Gupta

  • LUPUS ERYTHEMATOSUS

    Lupus erythematosus (LE) may be seen in one of two well-recognized forms

    Systemic (acute) lupus erythematosus (SLE) profound impact it has on many organs

    Discoid (chronic) lupus erythematosus (DLE) chronic and localised skin lesions

    involving the bridge of nose and adjacent cheeks

    Without any systemic manifestations.

    Rarely, discoid form may develop into disseminated form.

  • SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

    Autoimmune disease characterized by autoantibodies, immune complex formation, and immune dysregulation resulting in damage to Essentially any organ, including the kidney,

    skin,

    blood cells,

    CNS

    Natural history of this illness is unpredictable

    Early diagnosis and careful treatment - improved the prognosis

  • Etiology

    Specific cause of SLE remain undefined Both cell mediated Immunity and Humoral

    mediated Many factors, including genetics, hormones, and

    the environment Tissue damage due to direct binding and/or

    immune complex deposition in tissues Autoantibodies against DNA, other nuclear

    antigens, ribosomes, platelets, erythrocytes, leukocytes, and other tissue-specific antigen result in widespread tissue damage

  • Clinical Features Serious cutaneous-systemic

    disorder Repeated remissions and

    exacerbations. Peak age of onset: 30 years in

    females but about 40 years in males

    Female predicted: 8 to 10 times more

    Erythematous patches: Butterfly Rash on the face which coalesce to

    form a roughly symmetrical pattern over the cheeks

    and across the bridge of the nose

  • Clinical Features

    neck, upper arms, shoulders and fingers may also be involved

    Itching or burning sensations

    Hyperpigmentation

    severity is intensified by exposure to sunlight

    Involvement of various organs, including the kidney and heart

  • Systemic Lupus Erythematosus (SLE)

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  • Clinical Features

    Kidney fibrinoid thickening of glomerular capillaries wire loops Progressive thickening may lead to renal insufficiency

    Heart: Libman-Sacks endocarditis Involves valves, as well as fibrinoid degeneration of the

    epicardium and myocardium

    Collagen diseases: widespread tissue involvement and the nature of the lesions have led to the inclusion of this disease Rheumatic fever, Rheumatoid arthritis, Polyarteritis nodosa, Scleroderma Dermatomyositis

  • SLE: Clinical Feature

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  • Oral Manifestations

    2050% of cases of DLE, slightly more frequently in SLE

    Oral mucosa may be involved prior to skin manifestation or later or even in its absence

    Oral Lesions of SLE and DLE are similar in nature except in SLE there is more severity of

    hyperemia, edema and extension of the lesions

    tendency for bleeding, petechiae and superficial ulcerations

  • Oral Manifestations

    It may be associated with oral candidiasis as well as xerostomia.

    Diagnosis should not be based on oral findings alone clinical findings frequently simulate other diseases, chiefly leukoplakia and lichen planus

    Lesions usually affect the palate, buccal mucosa, and gingivae

    Sometimes they appear as lichenoid reaction It may also involve vermilion zone of the lower lip

    lupus cheilitis Varying degrees of ulceration, pain, erythema, and

    hyperkeratosis may be present

  • Oral Manifestations

  • Chronic cutaneous lupus erythematosus

  • Histologic Features

    Histologic features of SLE and DLE: similar

    Just an increase of degree of certain of the findings in SLE occurs

    Common findings are hyperkeratosis with keratotic plugging,

    atrophy of the rete pegs,

    liquefaction, degeneration of the basal layer of cells,

    perivascular infiltration of lymphocytes

    Basophilic degeneration of collagen and elastic fibers,

    Hyalinization, edema and fibrinoid change, particularly prominent immediately beneath the epithelium

  • Histologic Features

    Low-power: hyperparakeratosis with interface mucositis and perivascular inflammation.

    High-power: interface mucositis

  • Histologic Features

    In SLE

    degenerative features and collagen disturbance are usually more prominent

    Inflammatory features less severe

    Hydropic degeneration and liquefaction necrosis of the basal cell layer

    subepithelial vesiculation or ulceration

  • Histologic Features: Direct Immunofluorescent Testing

    Confirm a suspected diagnosis of LE

    Detect the presence of immunoglobulins (IgG, IgM and IgA) at the epidermal-dermal junction or basement membrane zone of skin or oral mucosa of patient

    100 % systemic form and in nearly 75% with the discoid form

    Incidence of complement C3 and of fibrinogen

    Demonstrated in the uninvolved skin and mucosa of a significant percentage of patients with SLE

  • Laboratory Findings

    Raised ESR and CRP : elevated in inflammation from any cause.

    Serum protein electrophoresis : increased gammaglobulin and decreased albumin.

    Routine blood counts: anemia and low platelet and white cell counts.

    Routine blood chemistry which may reveal: Kidney involvement: increases in serum blood urea

    nitrogen and creatinine Liver function tests: AST, ALT, BUN, creatinine Muscle involvement: Increased muscle enzymes (such

    as CPK)

  • Laboratory Findings: Specific

    Commonly used blood tests in the diagnosis of SLE are Antinuclear antibody test (ANA): autoantibodies to

    cell nuclei are present in the blood Anti-DNA antibody test: antibodies to the genetic

    material in the cell Anti-Sm antibody test: antibodies to Sm

    (ribonucleoprotein) found in the cell nucleus Serum (blood) complement test: total level of a group

    of proteins which can be consumed in immune reactions

    Complement proteins C3 and C4: examine specific levels

  • Laboratory Findings: Specific

  • Treatment

    Careful and frequent clinical and laboratory evaluation Decide adequate medical regimen

    Provide prompt recognition and treatment of disease flare

    Lifelong illness, and patients must be monitored indefinitely

    high-risk disease: possibility of end-organ damage to any organ

    Decreased quality of life

  • Treatment Avoid excessive sunlight exposure

    DLE topical corticosteroids

    If resistant to topical therapy: systemic antimalarial drugs or low-dose thalidomide, sulfones

    SLE nonsteroidal antiinflammatory drugs (NSAIDs)

    combined with antimalarial drugs, such as hydroxychloroquine

    Severe, acute episodes: systemic corticosteroids combined with other immunosuppressive agents.

  • Prognosis SLE patient prognosis is variable

    5-year survival rate: approx. 82% to 90%;

    20 years survival rate: 63% to 75%

    Prognosis depends on which organs are affected and how frequently the disease is reactivated

    Common cause of death is renal failure

    Chronic immunosuppression also predisposes these patients to increased mortality

    Prognosis is worse for men than for women

    Transformation to SLE may be seen in approximately 5% of DLE patients 50% of DLE patients - eventually resolves after several

    years

  • Refrences

    Shafers, Oral Pathology 6th edition

    Regezi: Oral Pathology: Clinical Pathologic Correlations, 5th ed

    Essential of Oral Pathology and medicine 7th ed : Cawsons & odell

    Color atlas of Oral Pathology: Nevile

    Pathology of the Head and Neck: Antonio Cardesa, Pieter J. Slootweg

    Essential of Oral Pathology : Swapan Kumar Purkait

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