This document summarizes key information about systemic lupus erythematosus (SLE). It describes SLE as an autoimmune disease characterized by the formation of autoantibodies and immune complexes that can damage multiple organs like the kidneys, skin, blood cells, and central nervous system. Genetics, hormonal imbalances, and environmental factors can lead to immune dysregulation and the production of autoantibodies against nuclear and other antigens. SLE predominantly affects women aged 30-40 and can present with characteristic rashes and oral ulcers. Complications involve organ damage like glomerulonephritis. Diagnosis involves identifying clinical features and serum autoantibodies.

1. • Autoimmune disease with formation autoantibodies,
immune-complexes and immune dysregulation resulting in
damage to kidney, skin, blood cells, CNS, etc
• Etiology: Genetics, Hormonal imbalances, Environmental
causes (Sunlight, Drugs, etc) leading to immune
dysregulation
• Autoantibodies and immune-complexes are produced
against DNA, other nuclear antigens, Ribosomes, Platelets,
Erythrocytes, Leukocytes and other tissue-specific antigens
Systemic Lupus Erythematosus

2. • Female : Male = 4:1
• Around 30-40yrs of age
• Erythematous patches on face with symmetrical pattern over
the cheeks and across the bridge of nose in a Butterfly
Distribution
• Neck, upper arms, shoulders and fingers may be affected
• Lesions present itching or burning sensations as well as areas
of hyperpigmentation
• Severity is intensified by exposure to sunlight
Clinical features

4. • Kidney: Fibrinoid thickening of glomerular capillaries
producing “Wire-loop” which result in kidney insufficiency
• Heart: Atypical endocarditis involving the valves as well as
fibrinoid degeneration of epicardium and myocardium
• Due to widespread tissue involvement , this disease has been
included into the “Collagen Vascular Diseases”

5. • Most common on Buccal mucosa, Palate and Tongue
• Erythematous areas with white spots which are usually
depressed and non-indurated
• Superficial painful ulceration may occur with crusting or
bleeding but no actual scale formation
• Margins of the lesion are not sharply demarcated but
frequently show formation of a narrow zone of keratinization
with white striae radiating out of the margins
• Central healing may result in depressed scarring
Oral Manifestations

6. • Hyperemia, edema and extension of lesions is more
pronounced
• Greater tendency to bleed, petechiae and superficial
ulcerations which are surrounded by red halo as a result of
local telangiectasis
• Superimposed oral candidiasis as well as xerostomia can be
seen
Oral Manifestations

7. • there is NO KERATINIZATION in SLE
• Liquefaction degeneration of basal cell layer with thickening
of basement membrane
• Perivascular infiltration of lymphocytes along with
infiltration around dermal appendages,
• Basophilic degeneration of collagen and elastic fibers with
hyalinization,
• Edema and fibrinoid change juxta-epithelially
Histologic Features

8. • Laboratory findings: Serum immunologic findings specific for
SLE
– Antidouble stranded DNA antibodies
– Anti- Sm antibodies
Histologic Features

9. • A characterisitic of lupus erythematosus
• Formation of LE cells on incubation of normal leukocytes
with the Serum from affected patients at 37°C.
• LE cells are Mature neutrophils arranged in a rosette-
pattern around a homogeneous-appearing basophilic
inclusion derived from nuclear material of degenerating
leukocytes and coated with antinuclear antibody
LE Test / LE phenomenon

11. • Chronic, Scarring, Atrophy producing, Photosensitive
Dermatosis
• May occur in pts with SLE or some patients with DLE may
progress to SLE
• Etiology: Genetic
• Heat-shock protein is induced in keratinocyte following UV
ray exposure or Stress and this protein is the target for the
T-cell mediated epidermal damage
Discoid Lupus Erythematosus

12. • 3rd and 4th decades of life
• Females more common
• Face, Oral mucous membrane, Chest, Back, Extremities, etc
• Elevated red / purple macules which are often covered by
gray or yellow adherent scales
• Forceful removal of scales reveals numerous “Carpet-Track”
extensions into enlarged pilosebaceous canals
• Periphery of lesion appears pink / red while centre exhibits
scarring or atrophy
Clinical features

15. • Most common on Buccal mucosa, Palate and Tongue
• Erythematous areas with white spots which are usually
depressed and non-indurated
• Superficial painful ulceration may occur with crusting or
bleeding but no actual scale formation
• Margins of the lesion are not sharply demarcated but
frequently show formation of a narrow zone of
keratinization with white striae radiating out of the margins
• Central healing may result in depressed scarring
Oral Manifestations

17. • Tongue: Atrophy of papillae and severe fissuring seen
• Vermilion border of lip: Atrophic plaques surrounded by
keratotic borders
• Malignant transformation of lip lesions may occur with some
frequency

19. • Hyperkeratosis with keratin plugging down into spinous
layer alternating with areas of Atrophic retepegs
• Liquefaction degeneration of basal cell layer with thickening
of basement membrane
• Perivascular infiltration of lymphocytes along with
infiltration around dermal appendages,
• Basophilic degeneration of collagen and elastic fibers with
hyalinization,
• Edema and fibrinoid change juxta-epithelially
Histologic Features

21. Epidermolysis Bullosa

22. • Group of inherited bullous diseases characterized by blister
formation in response to mechanical trauma
• Classified into 4 major categories:
1. Epidermolysis Bullosa Simplex (Intraepidermal skin
separation)
2. Junctional Epidermolysis Bullosa (Skin separation in
central basement membrane zone)
3. Dystrophic Epidermolysis Bullosa (sub-lamina densa
basement membrane zone separation)
4. Hemidesmosomal Epidermolysis Bullosa (Skin
separation in superior aspect of basement membrane
zone)
Epidermolysis Bullosa

23. • Epidermolysis Bullosa Simplex Is caused by mutation of
genes coding for Keratin 5 & Keratin 14
• Junctional Epidermolysis Bullosa shows blistering in the
Lamina Lucida along with various hemidesmosomal
abnormalities.
– Nonsense mutation LAMB3 gene
• Dystrophic Epidermolysis Bullosa is caused by mutations in
gene coding for type VII collagen (COL7A1)
– Anchoring fibrils are affected
Etiology

24. • Autosomal dominant and manifests at birth
• Vesicles & Bullae chiefly on hands and feet at sites of friction
or trauma
• Blisters heal in 2-10days without any scarring or permanent
pigmentation
• Localized form “Weber-Cockayne Syndrome” where bullae
develop only on hands and feet and tend to exacerbate in hot
weather. No scarring seen
Epidermolysis Bullosa Simplex

26. • Oral manifestations: Rare bullae seen in oral cavity
• Histologic features: Intra-epithelial Vesicles and bullae
develop as a result of destruction of basal and suprabasal
cells
• Individual cells become edematous and show dissolution of
organelles and tonofibrils with displacement of nucleus to
the upper end of the cell

28. • Extremely severe form
• 3 criteria established for diagnosis of this form:
1. Onset at birth
2. Absence of scarring , milia or pigmentation
3. Death within 3 months of age
• Bullae develop spontaneously and sheets of skin may be
shed
Junctional Epidermolysis Bullosa

29. • Oral manifestations: frequently very extensive bullae
– Cause feeding problems due to extreme fragility
– Similar lesions seen in upper respiratory tract and
bronchioles and eosophagus
– Severe disturbances in enamel and dentin formation of
deciduous teeth

31. • Onset at infancy and blisters develop on ankles, knees,
elbows, feet and head
• Healing results in scarring which could be keloidal in type
• Nails are thick and dystrophic along with palmoplantar
keratoderma and milia are commonly present
Epidermolysis Bullosa Dystrophic
Dominant

32. • Oral manifestations: Bullae occur in 20% of the cases and
teeth are unaffected
• Histologic features: Sub-epithelial split at the basement
membrane level. Basal cell layer is at the roof of the
vesicle/bullae and the connective tissue shows absence of
elastic and oxytalan fibers

33. • Classic form of the disease
• Onset at birth and blisters develop on sites of Trauma,
Friction or Pressure
• Feet , buttock, scapulae, elbows, fingers, etc
• Nikolsky’s sign is positive and the Bullae rupture to leave a
raw, painful surface
Epidermolysis Bullosa Dystrophic
Recessive

34. • The healing occurs by scar, milia or pigmentation and the
scarring may result in afunctional club-like fists (Mitten
Hands & Sock-Feet)
• Oral Manifestations: White spots / patches / or areas of
localized inflammation may precede the development of
bullae
• The bullae may be initiated by even a simple dental
operative procedure and large areas of denudation of oral
mucous membrane can occur without proper precautions

36. • Bullae are painful and scarring results in obliteration of
vestibule, or restriction of tongue
• Hoarseness or dyaphagia may occur as a result of bullae of
larynx or pharynx
• Dental Defects: Rudimentary teeth, Congenitally absent
teeth, Hypoplastic teeth, Absence of enamel on crowns
• Histologic features: Sub-epithelial split beneath the
basement membrane. Basal cell layer is normal and the
connective tissue shows increase in pre-elastic and oxytalan
fibers

39. Scleroderma
Systemic Sclerosis
Hidebound Disease

40. • Collagen Vascular Disease characterized by:
– Vasomotor disturbances
– Fibrosis with subsequent atrophy of skin, subcutaneous
tissue, muscles, internal organs
– Associated immunologic disturbances
• Etiology: Autoimmune disease with involvement of Genetic,
Environmental & Vascular factors
– MHC Antigens such as HLA-B8, HLA-DR5, HLA-DR3,
etc
– Apoptosis and generation of free radicals may be involved

41. • 30 – 50 yrs of age
• Female : Male = 3-6:1
• Begins on face , hands and trunk
• Increased collagen deposition with ultimate induration of the
skin and fixation of the epidermis to the deeper
subcutaneous tissues is seen.
• Early lesions start as indurated edema of the skin, neuralgia,
parasthesia as well as arthritis
Clinical features

42. • As the disease progresses the skin becomes yellow – grey – or
ivory-white waxy in appearance
• Calcium deposition or brown pigmentation can be seen
sometimes
• Skin becomes hardened, atrophic, firmly fixed to the deeper
connective tissue and cannot be “picked-up”
• This firm contracture of skin gives an appearance of
“MASK-LIKE” face and “CLAW-LIKE” hands
• Progressive disease involves internal organs by fibrosis, loss
of smooth muscle and loss of visceral function

45. • CREST syndrome: C = Calcinosis Cutis
R = Raynaud’s Phenomenon
E = Esophageal Dysfunction
S = Sclerodactyly
T = Telangiectasia
• Circumscribed scleroderma / Morphea: Appears as well-
defined, white-yellow, slightly elevated or depressed patches
which are surrounded by Violaceous halo
• Coup-de-sabre: Linear bands on forehaed made by a furrow
with an elevated ridge on one side resembling mark made by
blow of a tiger

48. • Early mild edema followed by atrophy & induration of
mucosal and muscular tissues
• Tongue , lips, soft palate and larynx are usually involved
• Tongue: Stiff and board-like causing difficulty in eating and
speech
• Lips: Thin, Rigid and partially fixed producing microstomia
• Dysphagia, inability to open and close mouth and difficulty in
breathing
Oral Manifestations

49. • Reduced mouth opening is due to involvement of
peritemporomandibular joint tissues
• Pathologic changes in minor salivary glands similar to
Sjögrens syndrome
• Radiographic Features: Extreme widening of PDL space, 2-4
times the normal thickness
• Bilateral Bone resorption of the angle of mandibular ramus
• Partial or complete resorption of condyles or coronoid
processes of mandible

50. Normal Scleroderma

51. • Thickening and hyalinization of collagen fibers in skin along
with loss of dermal appendages
• Atrophy of epithelium with Loss of retepegs and increased
melanin pigmentation
• Subcutaneous fat disappears and walls of blood vessels
become sclerotic
• PDL : Increase in collagen and oxytalan fibers along with
hyalinization and sclerosis with reduction in number of cells
Histologic Features

53. Ehlers – Danlos Syndrome
Rubber Man disease
Tenascin-X deficiency
Lysyl Hydroxylase deficiency

54. • Group of more than 10 different inherited diseases involving
genetic defect in collagen and connective tissue synthesis and
structure
• Affects Skin, Joints and Blood
• Type IV EDS is associated with arterial rupture and visceral
perforation which can be fatal

55. • Hyperelasticity of skin Circus Rubber Man
• Hyperextensibility of joint appearance
• Fragility of skin and blood vessels resulting in excessive
bruising as well as defective healing of skin wounds
• Face: Hypertelorism, Wide nasal bridge , Epicanthic folds ,
Protruding ears, Frontal bossing
Clinical features

59. • Extremely fragile OMM which bruised easily and could NOT
hold sutures
• Hypermobility of TMJ resulting in repeated Jaw dislocations
• Teeth : DEJ scalloping ABSENT, Passage of Dentinal tubules
into enamel with formation of Irregular dentin and increased
pulp stones
• Teeth with Hypoplastic enamel which are fragile and
fractured easily
• Sometimes Extensive periodontal destruction
Oral Manifestations