lupus erythematosus

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LUPUS ERYTHEMATOSUS Dr. Deepak K. Gupta

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Page 1: Lupus erythematosus

LUPUS ERYTHEMATOSUS Dr. Deepak K. Gupta

Page 2: Lupus erythematosus

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.

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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

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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

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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

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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

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Systemic Lupus Erythematosus (SLE)

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Page 9: Lupus erythematosus

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

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SLE: Clinical Feature

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Oral Manifestations

• 20–50% 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

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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

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Oral Manifestations

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Chronic cutaneous lupus erythematosus

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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

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Histologic Features

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

High-power: interface mucositis

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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

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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

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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)

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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

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Laboratory Findings: Specific

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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

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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.

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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

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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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