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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
• 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
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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