LUPUS ERYTHEMATOSUS

MADE BY: MUSKAN SRIVASTAV

ROLL NO:49
 INTRODUCTION

Lupus erythematous (LE) is a immunologically mediated condition

or can be called as an autoimmune disorder.
It is the most common “collagen vascular” or “connective tissue
disorder”.
The name “LUPUS” came from latin meaning wolf and refers to
erythematous red ulcerations on the face.
 ETIOLOGY
1. AUTOIMMUNE
Immunocomplexes consisting chiefly of nucleic acid and antibody.
1. GENETIC FACTORS
Genes that increase the risk of SLE are HLA-DR 2 and HLA-DR 3.
1. INFECTIONS
Epstein barr virus
Cytomegalovirus
Varicella zoster virus
 ETIOLOGY
4. ENVIRONMENTAL FACTORS
Exposure to pollutants
Hormonal factors
Ultraviolet light
Smoking
Possibly diet
5. ENDOCRINE FACTORS
A hormonal component to SLE is suggested by its high incidence in
women.
 In the presence of appropriate self antigens

Stimulation of autoantibodies

Formation of soluble immune complexes, ie IgG and IgM with antigen

Antibody complexes are deposited in various tissues such as skin,

lungs, kidneys or joints

Causes complement activation(type iii hypersensitivity)

Causes tissue damage and multiple organ involvement

 TYPES OF LUPUS ERYTHEMATOSUS

1. SYSTEMIC LUPUS ERYTHEMATOSUS: If multi organ involvement

occurs.
2. DISCOID LUPUS ERYTHEMATOSUS: It is also called chronic
cutaneous lupus erythematosus. It is confined to skin and
mucosa.
3. SUBACUTE CUTANEOUS ERYTHEMATOSUS: It present features of
systemic as well as discoid type.
4. DRUG INDUCED LUPUS ERYTHEMATOSUS: It characteristically
develops in people who have no history of systemic rheumatic
disease.
 DISCOID LUPUS ERYTHEMATOSUS
CLINICAL FEATURES
Age and sex distribution: It occurs in 3rd and 4th decades, female predilection
in the ratio of 5:1
Location: Most common sites are face, oral mucosa, chest, back and extremities
Appearance: It is a circumscribed, slightly elevated, white patch that may be
surrounded by red telangiectatic halo.
Cutaneous lesions: These are slightly elevated, red or purple macula’s, that are
often covered by gray or yellow adherent scales.
Carpet track extension: Forceful removal of scale results in ‘carpet
track extension’, which has dipped into enlarged pilosebaceous canals.
—The lesion increases in size by peripheral growth. Periphery of the lesion
appears pink or red, while the centre exhibits an atrophic scarred
appearance.
—Butterfly distribution on macular region and across the bridge of the nose
is present.
 DISCOID LUPUS ERYTHEMATOSUS
ORAL MANIFESTATIONS
Location: Most common sites are buccal mucosa, tongue, palate and
vermilion border of lip.
Appearance: It begins as erythematous area, sometimes slightly
elevated, but more often depressed, usually with induration and typically
with white spots.
Signs: Occasionally, superficial painful ulceration may occur with crusting
or bleeding, but no actual scale formation.
Symptoms: There may be burning and tenderness which may be
intermittent or disappear if the lesion becomes inactive.
—The margins of the lesion are not sharply demarcated. Fine white striae
radiate out from the margins. Central healing may result in depression.
Erythematous, atropic plaque, surrounded by keratotic border may involve
the entire lip.
SYSTEMIC LUPUS ERYTHEMATOSUS
CLINICAL FEATURES
Age and sex distribution: It occurs in 3rd decade in females and in
4th decade in males and has female predilection of 8:1.
Location: It is characterised by repeated remission and exacerbations
with common sites being face, neck, upper arm, shoulders and fingers.
Symptoms: It is manifested by symptoms of fever and pain in the
muscle and joints. Its may be present as itching or burning sensation
as well as area of hyperpigmentation.Severely intensifies after
exposure to sunlight.
Cutaneous lesion: The cutaneous lesion consists of erythematous
patches on the face, which coalesce to form roughly symmetrical
pattern over the cheeks and across the bridge of the nose, in a so
called butterfly distribution.
—Skin lesions are widespread, bilateral with signs of acute inflammation.
This finding helps to differentiate between skin lesions of DLE and SLE.
SYSTEMIC LUPUS ERYTHEMATOSUS
CLINICAL FEATURES

Other manifestation: There is involvement of various organs

including kidneys and heart. In kidney, fibrinoid thickening of
glomerular capillaries produce the characteristic ‘wire loop’ which
may be sufficient to result in renal insufficiency.
== Heart may suffer a Libman-sacks endocarditis involving valves
as well as fibrinoid degeneration of epicardium and myocardium.
SYSTEMIC LUPUS ERYTHEMATOSUS
ORAL MANIFESTATIONS
Location: The most common sites are buccal mucosa, lip and palate.
Patient complains of burning sensation, xerostomia or soreness of mouth
Appearance: The intraoral lesion is composed of a central depressed
red atrophic area surrounded by 2 to 4mm elevated keratotic zone that
dissolves into small white lines.
== Lesions similar to DLE, except that hyperaemia, edema and extension
of lesion is more pronounced
Signs: There is greater tendency to bleed and petechiae, suspected
ulcerations surrounded by red halo.
Lupus chelitis: The lip lesions appear with central atrophic area with
small white dots surrounded by keratinised border which is composed of
small radiating white striae.There is occasional ulceration of central area.
DRUG INDUCED LUPUS ERYTHEMATOSUS
It characteristically develops in people who have no history of
systemic rheumatic disease.
By far, the drugs with the highest risk are Procainamide and
Hydralazine, with the incidences of approximately 20% for
procainamide and 5-8% for hydralazine.
The risk for developing lupus like disease with other drugs is much
lower: quinidine can be considered a moderate-risk drug, whereas
sulfasalazine, chlorpromazine, penicillin, methydopa,
carbemazepine, acebutalol, isoniazid, captopril, propylthiuracil and
mincycline are relatively low risk drugs.
 SUBACUTE CUTANEOUS LUPUS
ERYTHEMATOSUS

Subacute cutaneous lupus erythematosus is a clinically distinct

subset of cases of that is most often present in white women
lupus erythematosus

aged 15 to 40, consisting of that are and evolve as poly-cyclic skin lesions scaly

annular lesions or plaques similar to those of plaque . psoriasis

Characteristically the lesions appear in sun-exposed areas such as

the vee of the or the , but not the . It may be brought on by
neckline forearms face

, but is usually associated with

sun-sensitizing medications such as and autoimmune disorders rheumatoid arthritis

.
Sjögren's syndrome

Therapy generally involves sun avoidance and protection and

topical . Sometimes systemic drug treatment is necessary.
corticosteroids [3]

Besides corticosteroids other such as are also used. immunosuppressants methotrexate [4]
 LABORATORY FINDINGS OF LE
Anaemia, leucopenia, thrombocytopenia
LE cell phenomenon-Positive
- It is rosette of neutrophils surrounding a pale nuclear mass apparently derived
from a lymphocyte.
-occurs as a result of antibody combining with DNA from nuclei of damaged
cells.
Coomb’s test-positive
Serum gammaglobulin increased
Autoantibodies found in patients with SLE are:
-Antinuclear antibody
-Antibody to double stranded DNA
-Anti- Smith antibody
-Anti-Ro antibody
-Antiphospholipid antibody
 ORAL MANIFESTATION
Patients with SLE are affected by a variety of orofacial disorders
including characteristic oral lesions, nonspecific ulcerations, salivary
gland disease and temporomandibular disorders.
Mucosal lesions:
- presence of desquamative gingivitis, marginal gingivitis or erosive
mucosal lesions.
- appear clinically identical to reticular or erosive lichen planes.
- the lesions are characterised by erythematous central zone,
surrounded by white radiating striae giving a brush border
appearance or hyperkeratotic plaques on the buccal mucosa, palate
and tongue.
 ORAL MANIFESTATION
- Unlike lichen planus, the distribution of lesions is usually
asymmetric, peripheral striae are faint and oral lesions rarely occur in
the absence of skin lesions.
Xerostomia- leads to caries and candidiasis.
Glossodynia, dysgeusia, dysphagia.
Root canal calcification, delayed primary and permanent tooth
eruption and twisted root formation.
Angular chelitis, ANUG( Acute necrotising ulcerative gingivitis)
TMJ disorders : Arthralgia, arthritis.
 LABORATORY FINDINGS
LE cell inclusion phenomenon with surrounding pale nuclear mass
apparently devoid of lymphocytes is present.
It is characterised by presence of abnormal serum antibodies and
immune complexes.
There is also anemia, leukopenia and thrombocytopenia with
sedimentation rate increased.
Serum gamma globulin increased and Coomb’s test is positive.
HISTOPATHOLOGICAL FINDINGS
Hyperorthokeratinization, hyperparakeratinization with keratotic
plugging, atrophy of the rate pegs and liquefaction degeneration of
basal layer is present.
There is perivascular infiltration of lymphocytes and their collection
about dermal appendages, basophilic degeneration of collagen and
elastic filers with hyalinisation, edema and fibrinoid change.
Skin lesions show hyperkeratosis, with keratin packed into the
opening of hair follicles called follicular plugging. Degeneration of
the basal cell layers is seen common in both skin and oral lesions.
The underlying connective tissue stroma shows patchy to dense
aggregates of chronic inflammatory infiltrate.
 TREATMENT
MECHANISM DOS
E
1. Provide symptomatic relief for arthralgia, Ibuprofen-400mg 4-6 hrly, should not exceed
NSAID fever 2.4g/day
S

TOPICAL:
Triamcinolone acetonide-0.1%
2.Corticosteroid To decrease inflammation and Clobetasol propionate-0.05%
s as Betamethasone dipropionate-0.05% twice a day
Immunosuppressant application
For 2 weeks followed by a 2 week rest period can
minimise
The risk of local complications
SYSTEMIC:
Prednisolone-20-40 mg/day
Methyl prednisolone-1g/day IV for 3 days

3.Immuno- To suppress immunity Methotrexate:7.5-25 mg for 1 week

Suppressive Cyclophosphamide:500-750 mg
IV every month
Azathioprine:1.5-2 mg/kg/day for 6-8 weeks,
increase
By 0.5 mg/kg until response is seen.