Etiology
Genetic predisposition: Higher incidence of auto-antibodies is
seen in blood relations of patient.
Viral infection
Hormones, i.e. increase estrogen level in pregnancy.
Autoimmune: Antibodies are develops towards one’s own body
cells.
Pathogenesis
Antibodies are produced in reaction to exposure of normally
unexposed self antigens. Dysregulation of immune system leads
to excessive production of antibodies against DNA, ribosomes,
other nuclear antigens, platelet, erythrocytes, leucocytes and
various tissue specific antigens which causes tissue damage.
Clinical Features
♦ It occurs during 30 years of age in females and 40 years
of age in males.
♦ Female predilection is seen. Female to male ratio is 2:1
♦ Most common sites affected are face, neck, upper arm,
shoulders. Disease is characterized by repeated remission
and exacerbations over these sites.
♦ Patient complaints of pain and fever in joints and muscles.
Itching or burning sensation is also present along with the
areas of hyperpigmentation. Symptoms aggravates under
exposure to sunlight.
♦ The characteristic sign of the disease is presence of erythematous patches over the face which coalesce to form roughly
symmetrical pattern over the cheeks and across the bridge
of nose, this is known as butterfly distribution. In the kidney
fibrinoid thickening of glomerular capillaries produces characteristic wire loops, this leads to renal insufficiency.
♦ In the heart there is presence of typical endocarditis involving valves along with fibrinoid degeneration of epicardium
and myocardium.
Oral Manifestations
♦ Buccal mucosa, lip and palate are most commonly affected.
♦ Patient complaints of presence of burning sensation in the
mouth. Xerostomia is also seen.
♦ Lesions have very much similarity to lesions of discoid
lupus except that they are hyperemic, edematous and
extension of lesion is pronounced. Tendency for bleeding
and petechiae is more as well as superficial ulcerations
surrounded by red halo are also present.
♦ Intraoral lesion consists of central depressed red atrophic
area which is surrounded by 2 to 3 mm elevated keratotic
zone which merge in white lines.
Histopathology
♦ In systemic lupus erythematosus, areas of epithelial atrophy are present with absence of keratinization.
♦ There is presence of liquefactive degeneration of basal
cell layer.
♦ There is presence of edema of subepithelial connective
tissue with dilatation of vessels.
♦ In systemic lupus erythematosus degenerative areas and
collagen disturbances are more prominent.
♦ Inflammatory features are less common.
For diagram refer to Ans 13 of same chapter.
Laboratory Findings
♦ LE cell inclusion phenomenon is used in which there is
addition of blood serum from a person who is suspected
to the buffy coat of normal blood. If patient is suffering
from systemic lupus erythematosus, typical LE cells will
appear. The test consists of rosette of neutrophils surrounding pale nuclear mass.
♦ There is also presence of anemia, leucopenia, thrombocytopenia and elevated ESR and serum gamma globulin
level with positive Coombs test.
♦ Lupus band test is positive, i.e. there is deposition of IgG,
IgM or complement component at epidermal – dermal
junction or basement membrane zone of skin.
Treatment
♦ Exposure to sunlight should be avoided.
♦ Patient should be kept on systemic corticosteroid therapy.
♦ NSAIDs should also be given to combat the symptoms.
