3. Autoimmune disease affecting multiple organ
systems
Common in females
Lupus erythematosus (LE) may be seen in one of
two well-recognized forms
1. Systemic lupus erythematosus (SLE)
2. Discoid lupus erythematosus (DLE)
4. Systemic lupus erythematosus (SLE)
Autoimmune disease characterized by autoantibodies, immune
complex formation, and immune dysregulation resulting in damage to
– Essential organs including
kidney, skin, blood cells, CNS
Etiology- genetics, hormones, and the environment(sunlight,drugs)
Pathogenesis-• 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
• The plasma cells are producing antibodies that are specific for self
proteins, namely ds-DNA Overactive B-cells Suppressed regulatory
function in T-cells Lack of T-cells Activation of the Complement
system
5. CLINICAL FEATURES- Serious cutaneous-systemic disorder • 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
• 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
• Kidney – fibrinoid thickening of glomerular capillaries – wire loops – Progressive
thickening may lead to renal insufficiency
• Heart-endocarditis – Involves valves, as well as fibrinoid degeneration of the
epicardium and myocardium
• Rheumatic fever, • Rheumatoid arthritis, • Polyarteritis nodosa, • Scleroderma
• Dermatomyositis
6. Oral Manifestations-• 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
• It may be associated with oral candidiasis as well as xerostomia.
• Lesions usually affect the palate, buccal mucosa, and gingivae • It may also
involve vermilion zone of the lower lip lupus cheilitis
• Varying degrees of ulceration, pain, erythema, and hyperkeratosis may be
present
Blister formation
7. DISCOID LUPUS ERYTHEMATOSUS
DLE is an uncommon skin rash, usually made worse by exposure to sunlight.
‘Discoid’ lupus erythematosus is confined to the skin and is not associated
with symptoms from other organs.
Etiology- DLE probably occurs in genetically predisposed individuals.
CLINICAL FEATURES-Although any skin area may be involved by the discoid
form of lupus erythematosus, the most common sites are the face, oral
mucous membranes, chest, back and extremities.
The typical cutaneous lesions are slightly elevated red or purple macules that
are often covered by gray or yellow adherent scales. The periphery of the
lesion appears pink or red, while the center exhibits an atrophic, scarred
appearance indicative of the long-standing nature of the disease with
characteristic central healing. The discoid form of the disease may also
assume a typical ‘butterfly’ distribution on the malar regions and across the
bridge of the nose
8. Oral Manifestations-Oral mucous
membrane involvement is reported in 20–
50% of cases of discoid lupus
erythematosus, The oral lesions in the
discoid form begin as erythematous areas,
sometimes slightly elevated but more often
depressed, usually without induration and
typically with white spots. Occasionally,
superficial, painful ulceration may occur
with crusting or bleeding but no actual
scale formation as is seen on the skin
9. 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
11. Ehlers-Danlos syndrome (EDS) is the name given to a group of more than 10
different inherited disorders; all involving a genetic defect in collagen and
connective-tissue synthesis and structure.
EDS can affect the skin, joints, and blood vessels.
One type, type IV, is associated with arterial rupture and visceral perforation, with
possible life-threatening consequences.
Clinical Features.
RUBBER MAN
The characteristic clinical features of this disease are the hyperelasticity of skin,
hyperextensibility of the joints, and fragility of the skin and blood vessels resulting in
excessive bruising as well as defective healing of skin wounds
Oral Manifestations.
The gingival tissues appeared fragile and bled after toothbrushing, gingival
hyperplasia and fibrous nodules were also noted
Hypermobility of the temporomandibular joint, resulting in repeated dislocations of
the jaw
the oral mucosa is of normal color but excessively fragile and bruised easily
13. Relatively uncommon condition of the oral mucosa described by Cannon in
1935.
Clinical Features-
The oral lesions may be widespread, often involving the cheeks, palate, gingiva,
floor of the mouth and portions of the tongue
The mucosa appears thickened and folded or corrugated with a soft or spongy
texture. There is sometimes a minimal amount of folding present. Ragged white
areas may also be present which can be removed sometimes by gentle rubbing
without any ensuing bleeding.
Histologic Features-
The epithelium is generally thickened, showing both hyperparakeratosis and
acanthosis, and the basal layer is intact. The cells of the entire spinous layer,
continuing to the very surface, exhibit intracellular edema.
In addition, parakeratin plugs running deep into the spinous layer are typically
found. The submucosa may show a mild inflammatory cell infiltration, but this is
not consistent.
