Diseases of skin 2011-12 /orthodontic courses by Indian dental academy

DISEASES
OF
THE SKIN
PEMPHIGUS
INDIAN DENTAL ACADEMY
LEADER IN CONTINUING DENTAL
EDUCATION
www.indiandentalacademy.com

LEARNING OBJECTIVES
At the end of the lecture student should beAt the end of the lecture student should be
able to understand theable to understand the
 Introduction, Clinical features, Oral manifestations,&
Histopathological features of Pemphigus & its
Variants

INTRODUCTION
 Dermatology = study of skin disorders
Importance of dermatology-
 Systemic diseases have cutaneous manifestations
 Also mucous membranes, including oral mucous
membrane
 Some dermatoses may be preceded by oral lesions
 Genodermatosis = Hereditary skin disorders
 Vesiculobullous diseases= skin disorders
characterized by presence of vesicles & bullae

 Most of the diseases under the heading of
vesicullobullous are caused by production of
antibodies by patient (auto antibodies).
 These are directed against various
constituents of molecular apparatus that
hold epithelial cells together or that bind
surface epithelium to the underlying
connective tissue.

 The ensuring damage produced by the
interaction of these auto antibodies with
the host tissue is clinically seen as a
disease process often termed as
 “Immunobullous disease”

Elevated blisters
containing clear fluid
that are under 1 cm
in diameter
Elevated blister like
lesions containing
clear fluid that are
over 1cm in diameter
Vesicle Bulla
Blister A fluid filled cavity formed within or beneath the epidermis

PEMPHIGUS
Pemphigus is a serious, chronic skin
disease characterized by appearance of
vesicles & bullae, small to large fluid
filled blisters on skin as well as mucous
membrane that develop in cycles.
Derived from Greek word
Pemphix bubble or blister.
Wichman in 1791.
It affects 0.1 to 0.5 patients per 100,000
population per year.

 Characterised
histologically by
intraepithelial clefts &
immunologically by
finding of circulating IgG
antibody directed against
the cell surfaces of
keratinocytes.

PEMPHIGUS VULGARIS
 It is an autoimmune, intraepithelial blistering disease
affecting skin & mucous membranes & is mediated by
circulating auto antibodies directed against keratinocyte
cell surface.
 These auto antibodies binds to keratinocyte desmosomes &
to desmosome free areas of keratinocyte cell membrane,
especially keratinocyte cell surface molecules
Desmoglein 1 & Desmoglein 3

 The binding of autoantibodies result in loss of
cell-cell adhesion.
 Antibody binding may activate complement with
release of inflammatory mediators & recruitment
of activated T-cell

PATHOPHYSIOLOGY
Hemidesmosomes
Desmosomes

 Desmosomes are adhesion proteins that function
both as an adhesive complex & as a cell –surface
attachment site for Keratin Intermediate
Filaments(KIFs) of the cytoskeleton
Plakoglobin
Desmoplakin
KIFs
Desmocolin
Desmoglein 1&3

IgGIgG

Other Factors
1. PV has some Genetic vulnerability.
2. Diet – Garlic
3. Drugs – Drugs like penicillamine and captopril &
rifampicin , diclofenac

4. Viruses – Herpes Simplex infection
5. Others – Pesticides, Estrogen
6. Association with Other diseases -
Rheumatoid Arthritis,
Myasthenia Gravis,
Lupus Erythematosus,
Pernicious anemia

CLINICAL FEATURES
 Affects all races with equal gender distribution.
 Age of onset - 50-60 yrs.
 Rapid appearance of vesicle & bullae varying in
diameter from few mm to several cm.
 Lesions contain thin, watery fluid shortly after
development but this may become purulent or
sanguineous. When bulla rupture they leave raw,
eroded surface.

Small intact vesicle on the
labial mucosa
Involvement of the eye, a few
days later with conjunctivitis
& conjunctival vesicles. There
is a small vesicle on the upper
eyelid

Large intact vesicles on
the skin
Erosive lesion of the posterior
buccal mucosa

Nikolsky’s sign
 The loss of epithelium occasioned by rubbing apparently
unaffected skin is termed Nikolsky’s sign. That is a bulla
can be induced on normal –appearing skin/mucosa if firm
lateral pressure is exerted.
 This is caused by prevesicular edema which disrupts dermal
epidermal junction.
 50% of patients have oral mucosal lesions before the onset of
cutaneous lesions by 1 yr & are very difficult to resolve with
therapy.
 “THE FIRST TO SHOW & LAST TO GO”

Blister formation on normal-appearing gingiva after the movement of
mirror handle under pressure indicative of positive Nikolsky sign.

 Virtually any oral mucosal locations may be
involved. Palate, labial mucosa, buccal mucosa,
ventral surface of tongue & gingival mucosa.
 Other sites being pharynx, larynx, oesophagus
conjunctiva, cervix, urethra & anal mucosa.
 Pemphigus can be associated with Thymoma,
Lymphoma, Kaposi’s sarcoma.

Multiple erosions affecting the
marginal gingiva.
Erosions on the dorsum of
the tongue.

Multiple erosions of the
left buccal mucosa.
Large, irregularly shaped
ulcerations involving the
floor of the mouth &
ventral tongue

Ocular lesions.

PEMPHIGUS VEGETANS
 An uncommon variant of Pemphigus vulgaris
 Occurs in 1 to 2% of Pemphigus vulgaris cases.
 Median age is 40-50yrs.
 Two clinical subtypes of pemphigus vegetans exists.
Flaccid bullae Pustules
& erosions

 Both subtypes develop into hyperpigmented
vegetative plaques with pustules &
hypertrophic granulation tissue at periphery.
 A characteristic feature of Pemphigus
vegetans is the Cerebriform tongue
characterized by pattern of sulci & gyri on
dorsum of tongue.

Vegetating lesions on
the buccal mucosa &
commissure.

ORAL MANIFESTATIONS
 Involves mucosa in 50-70% of patients. Intact
bullae are rare in mouth.
 Ill-defined, irregularly shaped, gingival,
buccal or palatine erosions which are painful
& slow to heal.
 Erosions extend peripherally with shedding
of epithelium. Erosions seen on any part of
oral cavity.

 Erosions may involve larynx with subsequent
hoarseness.
 Patient is unable to eat or drink adequately
because the lesions are so uncomfortable.
 Other mucosal surfaces may be involved,
including conjunctiva, esophagus, vagina,
cervix, penis, urethra & anus.

•An intraepithelial cleft
(Suprabasilar split) is
located just above the
basal cell layer.
•Rounded, acantholytic
epithelial cells sitting
within the intraepithelial
cleft. (Tzanck cells)Tzanck cells)
H
ISTO
PATH
O
LO
G
Y

Hyperchromatic Tzanck cellsHyperchromatic Tzanck cells

 Marked inflammatory
cell infiltration.
 Positive TzanckPositive Tzanck
test-test- Cytological test.

IMMUNOFLUORESCENCE
 Demonstrate presence of immunoglobulins,
predominantly IgG but sometimes in
combination with C3, IgA, IgM.
 Direct immunofluorescence – Frozen section of
patient’s tissue
 Indirect immunofluorescence – Patients serum

PARANEOPLASTIC PEMPHIGUS
 First described by Anhalt et al in 1990.
Diagnostic Criteria
1. Painful mucosal erosions, sometimes with a skin eruptions
that eventually resulting in blisters & erosions in the setting
of confirmed or occult malignancy
2. Acantholysis & keratinocyte necrosis

1. DIF typically reveals IgG & complement C3
within epidermal intercellular spaces as well as
at the epidermal basement membrane.
2. IDF reveals circulating antibodies specific for
stratified Squamous or transitional epithelium
3. Immunoprecipitation of a complex of proteins
with typical molecular weight.

 These are autoimmune response to intercellular adhesins
(Plakins).
 Mortality rate is 90 %
 Causes of death include –
1. Sepsis
2. Multiorgan failure
3. Respiratory failure

CLINICAL FEATURES
 Age - 60yrs (7-76yrs)
 M=F
 With little exception all patients had tumor (malignant)
(Non-Hodgkin Lymphomas 84 %)

Other associated malignancies include –
• Chronic lymphocytic leukemia (18 %)
• Castleman Tumor (18 %)
• Giant Cell Lymphoma (Reticulum Cell Sarcoma)
• Waldenstrom Macroglobulinemia (1.2 %)
• Thymoma (5.5 %)
• Poorly differentiated Sarcoma, SCC of Oral cavity
• Bronchogenic SCC
• Follicular dendritic Cell Sarcoma

 Conjunctival ulceration is a frequent features.
 Existence of neoplasm is recognized prior to
eruptions of lesions only in about 2/3rd
of cases

ORAL MANIFESTATIONS
 Painful oral lesions are accompanied by
generalized cutaneous eruption.
 Eruptions assumes form of morbilliform,
urticarial, bullous, papulosquamous or erythema
multiforme like lesions
 Pruritis, pain
 Erosions can occur on buccal, labial, lingual
mucosa & gingival.
 Erosions & crusting are similar to SJ syndrome
 Nose, pharynx, tonsils can be affected
 Nasal ulcers may cause epistaxis

Ocular involvement.
These diffuse oral ulcerations are
quite painful
Crusted, hemorrhagic lip Polymorphous cutaneous lesions.

HISTOPATHOLOGY
 Epidermal necrosis, Suprabasal acantholysis, dyskeratotic
keratinocytes, & lymphocytic infiltration.
 A distinctive feature is Dyskeratosis
Treatment
 Topical antibiotic ointment.

Paraneoplastic pemphigus. This low-power photomicrograph shows both
intraepithelial & subepithelial clefting.

PEMPHIGUS FOLIACEUS
 Cazenave in 1844.
 It is an autoimmune skin disorder characterized by
superficial blisters.
 Positive Nikolsky sign.
 Little or no involvement of mucous membrane.

It includes 6 subtypes
1. P. erythematosus
2. P. herpetiformis
3. Endemic Pemphigus foliaceus
4. Immunoglobulin A (IgA) Pemphigus foliaceus
5. Paraneoplastic Pemphigus foliaceus
6. Drug induced Pemphigus foliaceus.

PATHOPHYSIOLOGY
 Formation of autoantibodies directed against a
cell adhesion molecule, desmoglein1 expressed
mainly in the granular layer of the epidermis.

CLINICAL FEATURES
 Early bullous lesion which rapidly rupture & becomes
dry to leave masses of flakes or scales suggestive of
exfoliative dermatitis or eczema.
 It is relatively mild form of Pemphigus
 More common in older adults.
 Site – Rare in oral cavity
lower extremity,abdomen

Intact blisters, erosions & crusting

Brazillian pemphigus (Brazillian wildfire)
 Founds in tropical region particularly in Brazil,
Colombia, Bolivia, Venezuela, Tunisia.
 In children & frequently in family group.
 Oral lesions - Rare.
 Farmers, road constructers are affected
 Due to Arthopod borne
infective organism.
•Very severe scaling.
•Blisters are not apparent.

PEMPHIGOID
•Pemphigoid is a relatively
uncommon vesiculobullous lesion,
characterized histologically by the
subepithelial bullae formation in the
basement membrane zone.
•Pemphigoid Clinically similar to
Pemphigus (Oid=similar) BUT
histologic features & prognosis is
different.

PATHOGENESIS
 Formation of
autoantibodies in the body
against the antigenic
components of the
basement membrane zone
 Binding of autoantibodies
to hemi-desmosome
associated antigens in the
basement membrane.

Activation of complements
Synthesis of chemotactic factors
Release of proteolytic enzymes.
Destruction of hemidesmosomal junctions of
the basement membrane zone.
Formation of subepithelial vesicle or bullae.

 Unlike pemphigus, pemphigoid lesions usually
heal up by Scar formation (Cicatrication)
A.Cicatricial Pemphigoid
B.Bullous Pemphigoid
 Cicatricial Pemphigoid, in relation to the
mucous membrane, while Bullous
Pemphigoid occurs frequently in relation to
the skin.

CICATRICIAL PEMPHIGOID
(BENIGN MUCOUS MEMBRANE
PEMPHIGOID (BMMP)
CLINICAL FEATURES
 Frequently affects the middle-aged or
elderly females.
 The most common site of occurrence is the
OMM, followed by the conjunctiva.
 Sometimes the disease also involves the
nose, pharynx, larynx, esophagus and
genitalia, etc.

ORAL LESIONS
 Oral lesions begin as either vesicles or
bullae, most commonly involving gingiva.
 In severe cases, large vesicles or bullae
may develop on the palate, cheek, alveolar
mucosa or tongue, etc.

 Bullae are sometimes quite large in size and they
persist for several days.
 Gingival lesions often show erosion &
desquamation after rupture of bullae & leave raw
eroded, bleeding surface even for weeks or
months.
(Chronic Desquamative Gingivitis)

 The mucosal bullae are often tense and are
relatively tough because these are usually
covered by a full thickness epithelium, more-
over, these lesions remain intact for several
days
 Pain and bleeding are the common complaints
and Nikolsky's sign is positive

Adhesions (symblepharons) between
the bulbar & palpebral conjunctivae
Upper eyelid turns inward ( entropion)
resulting in eyelashes rubbing against
the eye (trichiasis)
Severe conjunctivitis, lower eyelashes
are removed because of trichiasis
Complete scarring between
conjunctival mucosa & eyelids
resulting in blindness)

Eye Lesions
 Conjunctivitis
 Blister formation in the eye
 Corneal ulceration-that often leads to blindness
 Swelling of the fornix and corneal opacity.
 Fibrosis and scarring of the lacrimal ducts, which
often leads to "dry eye".
 Scarring may cause adhesions between the bulbar
and the palpebral conjunctiva.

SKIN LESIONS
 Skin lesions occur in
about 5% cases and
mucosal lesions always
precede the skin lesions
 There are tense, fluid-
filled vesicles or bullae
which may appear over
the face, scalp or neck.

BULLOUS PEMPHIGOID
 Occurs more commonly on the skin, seldom affects
the oral mucosa.
CLINICAL FEATURES
 These lesions also follow the usual clinical patterns of
vesiculation, followed by ulceration and finally healing
(without scarring).
 Elderly people in the age group 70 to 80 years are
usually affected, and there is no gender predilection.

 The skin lesions mostly occur over the trunk
and limbs, and usually undergo spontaneous
regression.
 Skin lesions begin as red, eczematous plaques
which eventually progress to the formation of
bullae.

 Skin lesions always precede the mucosal
lesions and these mucosal lesions are
always smaller than that of the cicatricial
pemphigoid.
 Bullous pemphigoid having oral lesions
may be associated with internal
malignancy

HISTOPATHOLOGY
 Extracellular edema and vacuolation in the basement
membrane zone are the earliest histological changes
in cicatricial pemphigoid.
 Gradually there is formation of sub epithelial vesicles
or bullae.
 The sub epithelial bullae cause separation of the full
thickness epithelium from the underlying lamina
propria.

 Acantholysis and epithelial degenerative
changes are absent.
 Polymorphonuclear neutrophils (PMN) may be
found within the vesicular fluid.
 Blood vessels are often dilated and prominent
in the superficial part of the lamina propria.

 Subepithelial connective
tissue shows inflammatory
cell infiltration by
lymphocytes, macrophages &
eosinophils in the
perivascular areas.

ERYTHEMA MULTIFORME
•Erythema multiforme is an acute
inflammatory dermatological disorder
that frequently involves skin, mucous
membrane and sometimes the
internal organs.
•Characterized by presence of
Iris /Target lesions

 Two forms-
1. EM minor-localized eruption of skin with
mild or no mucosal involvement
2. EM Major ( Stevens-Johnson syndrome)-
more severe (potentially life threating)
skin & mucosal involvement

ETIOLOGY
1. Infections: Tuberculosis, Herpes simplex (I and II)
Infectious mononucleosis, Histoplasmosis,
1. Drug Hypersensitivity: Barbiturates,Sulfonamides,
Phenylbutazone, Salicylates, Oral pills, etc.
2. Hyperimmune Reactions
3. Miscellaneous Factor: Radiation therapy, Crohn's
disease, Vaccinations

CLINICAL FEATURES
 Frequently occurs between the age of 15 & 40
years
 Males > females.
 Rapidly developing erythematous macules,
papules, vesicles or bullae, often appear
symmetrically over the hands, arms, legs, feet,
face, neck
 The classic dermal lesions of erythema
multiforme which often appear on the extremities
are called "Target", "Iris" or "Bull's eye"

 Dermal lesions consist of
concentric erythematous
rings separated by rings of
near normal color on the
skin.
 Mucosal lesions of this
disease also consist of
macules, papules or
vesicles, etc. & mostly seen
on the tongue, palate,
buccal mucosa & gingiva.
Bulls eye lesion

 The vesicles of the
mucosal surfaces are
often short-lived and
they readily become
eroded or ulcerated &
bleed profusely.
 The ulcers are extremely
painful & are normally
covered by a slough;
they can be secondarily
infected in may cases
too.
Ulcerated lesions
Hemorrhagic crusts on lips

 Foul smell in the mouth & difficulty in eating &
swallowing
 The cutaneous & mucosal lesions may occur
either separately or simultaneously.
 Tracheo-bronchial ulcerations & pneumonia
 Uncommon among children & older individuals,
however, if it occurs in the older people, the
possibility of an internal carcinoma should not be
ruled out.

STEVENSJOHNSON
SYNDROME
It is a severe form of Erythema
Multiforme that simultaneously
involves the skin, eyes, oral mucosa &
genitalia.

CLINICAL FEATURES
 The clinical features of this condition are as
follows:
 Skin Lesions: Severe lesions of macules,
papules, vesicles & bullae, etc
Oral Mucosal Lesions: Large vesicles or bullae
which often rupture & leave painful ulcers.

 Pain & hemorrhage from the ulcers cause
difficulty in taking food.
 Ulceration & bloody encurstration are often
present on the lips.
 Mucosal lesions are self-limiting and complete
healing usually occurs in about 2 to 3 weeks.

 Eye Lesions :
 Photophobia
 Conjunctivitis
 Corneal ulceration
 Blindness due to secondary infection.
 Genital Lesions :
 Urethritis
 Balanitis
 Vaginal ulceration

HISTOPATHOLOGY
 Often nonspecific
 Acanthosis, intra or
intercellular edema &
necrosis of the epithelium
 Vesicles may form within the
epithelium or at the
epithelium-connective tissue
junction.

 Subepithelial connective
tissue shows edema &
perivascular infiltration of
lymphocytes and
macrophages.

HEREDITARY
ECTODERMAL DYSPLASIA
•Hereditary ectodermal dysplasia is an
inherited Xlinked recessive
disorder characterized by the
defective formation of ectodermal
structures of the body (e.g. skin, teeth,
nails, sweat glands, sebaceous glands
and hair follicles, etc.)

CLINICAL FEATURES
 The three most outstanding features are-
1. Hypohydrosis (lack of sweating),
2. Hypotrichosis (absence of hair) and
3. hypodontia (absence of teeth).
 Occurs more frequently among males than
females.

 Soft, dry and smooth skin
with little or no tendency
for sweating
 May have an unexplained
fever and they cannot
endure warm temperatures
 The hair over the scalp and
eyebrows are fine, scanty
and blond, and sebaceous
glands are also absent
(asteatosis)

 Complete or partial
anodontia occurs that
involves both deciduous as
well as the permanent
dentition (only the canines
are often present).
 The teeth which are
present, are often small
and conical in shape.
 Xerostomia is a constant
feature which occurs due to
the decreased salivary
secretion.

 The patient may also have rhinitis, sinusitis and
pharyngitis etc, with dysphagia and hoarseness
of voice
 Typical facial appearance characterized by
depressed nasal bridge, frontal bossing
and protuberant lips
 High palatal arch & cleft palate

TREATMENT
 The typical clinical manifestations always confirm
the diagnosis of this disease and there is no specific
treatment for it.
 Artificial dentures (with soft liners) are constructed
and are changed from time to time to cope up with
the growth of the jaws and artificial saliva is given to
keep the mouth moist.

PSORIASIS
Psoriasis is a selflimiting, chronic
inflammatory dermatological disease of
unknown etiology.
Some investigators believe that its
occurrence is genetically determined

Clinical Features
 Predominantly in the 2nd
& 3rd
decade of life.
 No sex predilection.
 Painless, dry, white scaly patches, that appear on
the skin over the elbows, knees, scalp, chest &
face, etc.
 The patches are well-circumscribed &
erythematous, few lesions can even produce sterile
pustules.

 The lesions enlarge at the periphery & the
disease often shows periods of remissions and
exacerbations.
 Mental anxiety or stress often increases the
severity of the disease.
 "Auspitz's sign“ If the deep scales on the
surface of the lesion are removed, one or two
tiny bleeding points are often disclosed.

 Oral lesions of psoriasis- Erythematous patches
with white scaly surfaces over the lips, palate,
gingiva & cheek, etc.
 Some lesions may appear as well-defined,
grayish-white or yellowish-white patches. In
some cases these oral lesions may resemble
'geographic tongue'.
 Sometimes psoriasis may be accompanied by
arthritis-Psoriatic Arthritis

HISTOPATHOLOGY
 The oral epithelium shows
atrophy with hyper -
parakeratosis, absence of
granular cell layer and
elongation or clubbing of the
rete pegs.
 Intraepithelial micro abscess
formation (abscess of
Monro) is an important
histological finding of
psoriasis.

 There is always an
increased mitotic activity
seen in the psoriatic skin
or mucous membrane.
 Thesubepithelial
connective tissue shows
mild lymphocytic or
histiocytic cell infiltrations.

EPIDERMOLYSIS BULLOSA
It is a generalized desquamating condition
of the skin & mucosa which is often
associated with scarring, contractures &
dental defects.
Separation of the epithelium from the
underlying connective tissue with
formation of large blisters, which often
heal with extensive & often immobilizing
scar formation.

Two broad categories:
 1. Hereditary epidermolysis bullosa include
epidermolysis bullosa simplex, epidermolysis
bullosa dystrophic and junctional epidermolysis
bullosa.
 2. Acquired epidermolysis bullosa. There is
only a single form of named as epidermolysis
bullosa aquisita.
 This acquired from of the disease may be
associated with multiple myeloma, diabetes
mellitus, tuberculosis, amyloidosis and crahn's
disease, etc.

CLINICAL FEATURES
 Characterized by the formation of multiple
vesicles or bullae on the pressure areas of
the skin (i.e. elbows and knees).
 Dystrophic typemost severe form & may
cause death secondary to septicemia.
 The lesions rupture & leave raw, painful ulcers,
which heal up with scarring.

 The healing of skin lesions cause scarring
with pigmentation or depigmentation of
the area.
 Nails often shed or exfoliate due to
formation of blisters in the nail beds.
 The hereditary form
 Very severe in nature & in infancy or
early childhood
o Acquired form
 Common during adulthood only.

 May also exhibit stunted
growth, mental retardation and
ectodermal dysplasia, etc.
 May have alopecia & claw-
like hands due to repeated
scarring and contractures.
 Many such patients die during
childhood.

ORAL MANIFESTATION
 Oral lesions are particularly common &
severe in relation to the hereditary forms
of the disease.
 Rapidly developing, multiple, fragile &
hemorrhagic blisters or bullae in the
areas of trauma (particularly in the
palate).

 The lesions rupture soon & leave painful
ulcers, which later on heal by
scarring.
 Repeated blistering & scarring around the
oral cavity result in decreased mouth
opening, ankyloglossia & loss of vestibular
sulci.

 May develop perioral & perinasal crusted &
hemorrhagic granular lesions.
 Sometimes oral lesions may transform into
squamous cell carcinoma.
 Hypoplastic pitted enamel of the molar
teeth
 Delayed eruption of tooth, increased caries
susceptibility & increased periodontal diseases.

HISTOPATHOLOGY
 Destruction of the basal or the
suprabasal layers of the oral
epithelium, resulting in the
formation of vesicles or bullae.
 Bullae formation may also be
seen within enamel organ of the
developing tooth germ.

 Disease type: Level at which bullae
forms.
1. Simplex type: intraepithelial bullae.
2. Junctional type: at the level of lamina lucida,
3. Dystrophic type: at the level of lamina densa,
 Treatment: Systemic steroid therapy.
Immunosuppressive drug therapy . Avoidance of
trauma

INCONTINENTIA PIGMENTI
It is a serious type of inherited
genodermatosis, which is transmitted
as a sex-linked dominant trait.

CLINICAL FEATURES
 Mostly during infancy & although it is more
common among females, it is often lethal among
males.
 Clinical manifestation begins to appear shortly
after birth & is characterized by slate- grey
pigmentation of the skin with formation of vesicles
or bullae over the trunk & limbs.

 Lichenoid, papillary or verrucous lesions also
develop in the skin.
 Oral mucosa exhibits patchy, plaque-like or
verrucous looking white lesions on the buccal
mucosa.
 Patients may also have epilepsy, strabismus
with nystagmus and partial anodontia, etc.

HISTOPATHOLOGY
 During the verrucous stage of the disease,
intra-epithelial vesicle formation is often
seen with accumulation of large number of
eosino-phils.
 Dermal or submucosal accumulations of
macrophages & melanin granules are also
seen.

 White areas display hyperorthokeratosis
or parakeratosis with acanthosis.
 Individual cell keratinization is also
sometimes seen.
Treatment
 No specific treatment is available.

SUMMARY
Introduction, Clinical features, Oral
manifestations,& Histopathological features of
 Hereditary ectodermal dysplasia
 Psoriasis
 Epidermolysis bullosa
 Incontinentia pigmenti

LUPUS
ERYTHEMATOSUS
Lupus erythematosus is an
autoimmune disorder, characterized
by the destruction of tissue due to
the deposition of auto antibodies &
immune complexes within it.

PATHOGENESIS
 Not clearly known.
 Believed that autoimmune reactions
cause changes within the basal cells of the
skin or mucous membrane along with the
collagen & vascular tissues.
 Auto antibodies to DNA and other nuclear
and ribonuclear protein antigens are
present in blood.

 The circulating auto antibodies cause cross
reactivity with antigenic determinants on
multiple tissues.
 The disease occurs in two basic forms.
1. Systemic lupus erythematosus (SLE)
2. Discoid lupus erythematosus (DLE)

SYSTEMIC LUPUS
ERYTHEMATOSUS (SLE)
 Lesions are in the skin & oral mucous
membrane, besides this also involves certain
body systems.
CLINICAL FEATURES
Skin lesions
 Occurs in about 0.1 to 0.4% individuals.
 Characterized by the development of fixed,
erythematous rashes, that often have a
"butterfly configuration" over the malar
region and across the bridge of the nose.

 Rashes may spread diffusely & have a wide area
of skin involvement.
 Skin rashes produce itching or burning
sensation, which is often aggravated by the
exposure to sunlight, often causes hyper
pigmentation of the skin.
 Females >five times more often than Males
 4th
decade
 Patchy or extensive loss of hair from the scalp-
very common.

Oral Lesions
 About 20 % of SLE,white, plaque-like &
resembles lichen planus.
 Erythematous lesions
 Formation of hemorrhagic macules in the oral
mucosa that becomes frequently ulcerated.
 Ulcerated lesions are often surrounded by a red
halo.
 Severe burning sensation & the affected area is
extremely tender to palpation.

Systemic Manifestations
 Fever, fatigue, malaise, vomiting, diarrhoea &
anorexia
 Dysphagia & depression
 Splenomegaly, lymphadenopathy .
 Leukopenia & hyperglobulinemia

 SLE may sometimes coexist with other
diseases like Sjogren's syndrome,
Raynod's phenomenon, Scleroderma,
Pemphigus, pemphigoid, Erythema
multiforme,etc.

HISTOPATHOLOGY
 Atrophy with hyperkeratinization of the
oral epithelium
 Liquefactive degeneration of the basal cell
layer .
 Edema of the sub epithelial connective
tissue, with vascular dilatations
 Lymphocytic infiltration & fibrinoid
degeneration of the collagen fibers.

DISCOID LUPUS ERYTHEMATOSUS (DLE)
 Occurs commonly among females
 The usual age is the 3rd
& 4th
decades of life.
 Commonly involves the skin over the back,
chest and extremities, OMM

ORAL LESIONS
 Multiple white plaques with central
atrophy and reddish purple erosions.
 Mucosal lesions involve cheek, lips,
gingiva & palate, etc.
 Small white dots are often present on a
slightly elevated border.

 Pain & burning sensations are common,
ulceration may occur in the plaque.
 Often these oral lesions resemble
leukoplakia.
 Erythema or ulcerations is surrounded by
a white keratotic border, which is also
sometimes having radiating striae.

SCLERODERMA
It is a complex Multisystem disease
Characterized by progressive diffuse
fibrosis (sclerosis) of the skin &
multiple internal organs, e.g. G I T,
lungs & kidney, etc.

PATHOGENESIS
 Blood circulation insufficiency in the tissue
because of the abnormalities in the arterioles
& blood capillaries.
 Cause replacement of the normal connective
tissue by the dense collagen bundles,results
in the fibrosis, or sclerosis of the tissue.

1)Localized or Morphea form-
Development of one or more, red or ivory
colored, smooth, hard patches on the skin.
Does not have any oral manifestations.
2) Generalized or Diffuse form (more
common type)-
Among children or young adults, frequently
affects the females. More prevalent in areas
where silicosis is a common environmental
hazard

CLINICAL FEATURES
 Begins with edema of the skin over the face,
hands or trunk.
 The affected skin often becomes tight & fixed
firmly to the underlying tissues e.g. muscles and
bones, etc.
 Results in progressive loss of mobility of hands &
joints as well as the movements of the other
internal & external organs.

 Arthritis & arthralgia, along with neuralgia &
paresthesia of the skin.
 With disease progression, the skin becomes
yellow, gray or ivory white in color & shows a
waxy appearance & gradually becomes atrophic,
with areas of pigmentation & calcium
deposition.

 Due to fixation or
induration of the skin,
"wrinkles" (lines of facial
expression) do not form
and as a result a "mask-
like" appearance of the
face and a "claw like"
appearance of the hands
develop.

 Restriction of movements occur in all the voluntary
& involuntary muscles as a result of fibrosis,&
patient becomes completely bed-ridden.
 Dyspnea, dysphagia, loss of vision & difficulty in
speech
 Finally, all the internal organs, e.g. GI tract, heart,
lungs, kidneys, etc. become affected by the fibrosis,
& these organs loose their respective function.

 CREST syndrome is often associated with
scleroderma & it includes the following features.
C- Calcinosis cutis
R- Raynaud's phenomenon
E- Esophageal dysfunction
S- Scleroductyly
T- Telangiectasia.
 Besides this, scleroderma may also coexsist with
some other systemic diseases like- Sjogren's
syndrome, Rheumatoid arthritis, Polymyositis &
Lupus erythematosus, etc.

ORAL MANIFESTATION
 Mostly involves the tongue, cheek, lips, soft
palate & the larynx, etc.
 Varying degrees of trismus & loss of salivary
secretion.
 Oral mucosa shows edema, followed by
atrophy & induration.
 Stiffness of the tongue muscles causes
restricted movements with difficulty in
speech & swallowing.

 Gingival tissue becomes pale and firm.
 Alteration of the fibrous components of gingiva
results in advanced periodontitis.
 Microstomia occurs due to the fixation & rigidity
of the lip muscles.
 Dysphasia due to esophageal strictures.
 Pain, clicking sounds & crepitations in the TMJ.

RADIOLOGICAL FEATURES
 Generalized widening of the periodontal
ligament space.
 Resorption of bone occurs in some areas of
the condyle or ramus of the mandible due to
persistent pressure from the contracting
lesions.

WIDENING OF PDL SPACE

HISTOPATHOLOGY
 Oral epithelium -atrophic with flattening of the
rete ridges.
 Areas of pigmentations
 Thickening & hyalinization of the collagen fibers
in the connective tissue.
 In the early stages, perivascular infiltrates of
mononuclear inflammatory cells are common.

 Blood vessels gradually become scanty & there is
narrowing of the lumen of the remaining vessels
due to the perivascular fibrosis.
 The thickness of the periodontal ligament is
increased due to the increased synthesis of
collagen and oxytalan fibers.
 Sweat glands, sebaceous glands & hair follicles
are often absent in the skin.

ORAL LICHEN
PLANUS
(LICHEN RUBER
PLANUS)
•Wilson in 1869
•Chronic mucocutaneous disease in
which the skin manifestations can
occur independently, concurrently or
sequentially

PATHOPHYSIOLOGY
 T cell mediated autoimmune disease
CD 8+ T-cells recognize antigen associated with
MHC class I on keratinocytes
Keratinocyte apoptosis
Release of cytokines
Attract additional lymphocytes

ETIOLOGY
 Expression of lichen planus antigen may be
induced by
1. Drugs (Lichenoid drug reaction)
2. Contact allergens in restorative material
3. Mechanical trauma( Koebner phenomenon)
4. Viral infection
5. Emotional stress/Anxiety
6. Malnutrition
7. Infection
8. Tobacco use
9. Grinspan’s syndrome-Lichen planus, Diabetes
mellitus,& vascular hypertension

CLINICAL FEATURES
 Prevalence in Indians-1.5%,highest (3.7%) with
habits & 0.3% in non-users of tobacco
 13.7% among who smoke & chewed tobacco
 Females > Males,1.4:1
 4th
to 5th
decade of life
 Pt complaints of burning sensation

 Skin lesions appear as
small, annular flat
topped papules few mm
in diameter
 Coalesce to form large
plaques, covered by
fine,glistening scale
 Early lesions are red
later reddish, purple or
voilaceous hue
 Later dirty brownish
color develops

Surface of the papule is covered by
fine grayish white lines called
Wickham’s Striae
Bilaterally symmetrical lesions on
flexor surfaces of wrist & forearms,
inner aspect of knees & thighs,&
trunk

ORAL MANIFESTATIONS
 May occur weeks or
months before
appearance of skin
lesions
 Radiating white, gray,
thread like papules in a
linear, annular or
retiform pattern
 Most common-Buccal
mucosa, may be on lips,
tongue, palate.

 Tiny white dot at the
intersection of white lines
known as striae of
Wickham
 When plaque like lesions
occur, striae are seen at the
periphery

TYPES
Bullous & Vesicular
Erosive
Atrophic
Hypertrophic

Hypertrophic
Hypertrophic
Erosive
Atrophic

HISTOPATHOLOGICAL FEATURES
 Hyperparakeratosis/
Hyperorthokeratosis
with thickening of
granular layer
 Saw tooth rete pegs
 Band like sub-
epithelial
inflammatory cell
infiltrate

 Liquefaction degeneration of basal cells
 Degenerating basal keratinocytes form Civatte,
hyaline, cytoid or colloid bodies, which appear as
homogeneous eosinophilic globules

 Max -Joseph spaces- Degeneration of basal
keratinocytes & disruption of anchoring element
of epithelial basement membrane & basal
keratinocytes( hemidesmosomes, filament fibrils)
weakens the epithelial –connective tissue
interface- Histological clefts-Max -Joseph
spaces
 Clinically blisters on oral mucosa-Bullous lichen
planus

DIFFERENTIAL DIAGNOSIS
 Lichenoid reactions
 Leukoplakia
 Candidiasis
 Pemphigus
 Erythema multiforme
 Recurrent aphthae
 Lupus erythematosus

TREATMENT & PROGNOSIS
 Corticosteroids
 Remedy for Etiological factors
 Malignant transformation rate of 0.3-3%
 Erosive & Atrophic forms- more prone

EHLERS-DANLOS
SYNDROME
•A group of hereditary disorders
characterized by defective collagen synthesis
in various body organs.
• Hyper mobility of the joints & increased
laxity of the skin, - "Rubber man"
•Excessive bruising tendency & defective
wound healing due to increased fragility of
the skin & blood vessels.

ORAL MANIFESTATIONS
 Increased fragility of the oral mucosa.
Retarded wound healing
 Bleeding from the gingiva & oral
mucosa ,Mobility of teeth.
 Enamel hypoplasia
 Loss of normal scalloping of the dentino-
enamel junctions of the tooth

 Large pulp stones in the teeth &
formation of irregular type of dentin
 Hypermobility of the
temporomandibular joint.
 Difficulty in suturing the oral
wounds.

Abnormal ability to elevate right toe
Dorsiflexion of all the fingers
Joint hypermobility
Dorsiflexion of all the fingersLuxation of TMJLuxation of TMJBilateral bleeding on cheeks

Differential Diagnosis
 Hereditary benign intraepithelial
dyskeratosis.
 Lichen planus.
 Hyperplastic candidiasis.
 Leukoplakia.
 Verrucous carcinoma.

Histopathology
 White sponge nevus microscopically presents mild to
moderate hyper-parakeratosis of the epithelium
with acanthosis and intercellular edema.
 There may be presence of some "vacuolated" cells in
the spinus cell layer having pyknotic nuclei.

 The underlying connective tissue shows mild
inflammatory cell infiltration.Parallel striae of
condensed parakeratin traverse the surface
layers in oblique planes.
 Individual cell keratinization may be seen in the
spinus layer.

FOCAL DERMAL HYPOPLASIA
SYNDROME (GOLTZS SYNDROME)
 Genetic disorder characterized by distinctive skin
abnormalities and variety of defects affecting
eyes , teeth, skeletal , urinary , gstrointestinal ,
cardiovascular and central nervous system.
 The skin lesions appear to evoke as
accumulations of fat rather than hypoplasia of
the dermis.

 Mnemoic FOCAL
 Female sex
 Osteopsthica striatia
 Coloboma
 Lobster claw deformity
 Absent ectodermis, mespdermis, neurodermis
elements.
 Affected individuals are recognized at birth or
prenatally.
 Also known as GOLTZ syndrome.

Etiology:
 X-linked dominant inheritance pattren,
common findings of syndactyly, oligodactyly
and polydactyly.
 Clinical features:
 Characterized by relative focal absence of
dermis associated with herniation of
subcutaneous fat into the defects
 Skin atropy, streaky pigmentation &
telangiectasia
 Multiple papillomas of the mucosa.

 Anomalies of the extremities including
syndactyly, polydactyly and adactyly
 Asymmetrical face with pointed chain and
notched nasal alae, asymmetical ears
 Sunken ears
 Sparse eyes brows and scalp hair
 Eye anomalies
 Mental retardation

ORAL MANIFESTATIONS
 Papillomas of the lips is a striking feature.
 Teeth are commonly defective in size, shape or
structure
 Microdontia is a common finding.
 Cleft lip or palate may also be present.

HISTOLOGIC FEATURES:
 Attenuation of dermal collagen fibres with
partial to complete absence of significant portions
of dermis,
 Change in appearance of adipose in the cells in
the dermis.
 If the accumulation of adipocytes is pronounced it
may cause the apparent herniation of
subcutaneous through the thinned skin.

KERATOSIS
FOLLICULARIS
It is a hereditary disorder of the skin &
is characterized by the formation of
multiple crusted, greasy lesions that
often produce foul odor

CLINICAL FEATURES:
 Face & neck of the younger individuals.
 Small white papules on the gingiva,
tongue, palate & cheek, etc.
 Hyperkeratotic papules over the skin-
about 2 to 3 mm in diameter & become
grayish-brown with age.
 Smaller lesions coalase together &
gradually enlarge with time.

INVOLVING LEG AND PALATE

HISTOPATHOLOGY
 Multiple clefts & lacunae within the
epithelium.
 These clefts or lacunae contain two types of
cells. The grain-like keratinized epithelial
cells named "corps grains" & esoinophilic
cells named "corps ronds".

 Intra-epithelial bullae may appear & in
such cases the lesions resemble
pemphigus vulgaris.
 The epithelium -hyperplastic with
presence of chronic inflammatory cell
infiltration in the lamina propria.
TREATMENT
 By high doses of vitamin A therapy or
steroids.

DYSKERATOSIS
CONGENITA
It is a rare hereditary disorder of skin,
which is inherited as a recessive trait.

CLINICAL FEATURES
 Commonly occurs during early childhood & definite male
predominance.
 Nails exhibit dystrophic changes and they gradually shed
in few days time.
 Skin shows grayish-brown pigmentations over the trunk,
neck and thighs.
 Facial skin appears red due to atrophic or telangiectatic
changes.
 Mental retardation, dysphagia, deafness and
hyperhydrosis of the palms and soles, etc.

INVOLVING NAILS AND TONGUE

 Vesicles and ulcerations over the oral mucosa is
often seen and these are followed by the
appearance of white patches of necrotic epithelium.
 Oral mucosa may also have red, macular lesions or
erythroplakic patches with superadded candidal
infections.

 Later on leukoplakia develops in the oral
mucosa which may turn into squamous
cell carcinoma.
 Severe periodontal tissue destruction and
loss of alveolar bone is often seen.

HISTOPATHOLOGY
 Oral mucosa exhibits hyperortho or hyper-
parakeratosis and acanthosis.
 In many cases there is presence of
epithelial dysplasia.
 Skin lesions show increased vasculitis and
increased number of melanin containing
chromatophores.

WHITE SPONGE
NEVUS
A hereditary disease characterized by
the occurrence of white, thickned,
corrugated mucosal lesions in the oral
cavity that often affects several
members of the same family.

CLINICAL FEATURES
 Occurs during childhood & no sex predilection.
 Cheek, palate, tongue, gingiva & floor of the
mouth- common intraoral sites
 Clinically exhibit asymptomatic, white, folded
areas in the mucosa.
 Sometimes the lesions may develop in the nasal,
rectal or genital mucosa.

 The oral lesions are soft & spongy & have a
peculiar opalescent hue. These lesions often
develop bilaterally & are either diffuse or well
localized.
 The surface of the lesion sometimes exhibit
areas of desquamation.

THE FOLDED SPONGY TEXTURE OF THE BUCCAL
MUCOSA

HISTOPATHOLOGY
 Verrucous stage of the disease- intra-
epithelial vesicle formation with
accumulation of large number of
eosinophils.
 Dermal or submucosal accumulations of
macrophages & melanin granules
 White areas display hyperorthokeratosis
or parakeratosis with acanthosis.
 Individual cell keratinization -sometimes

ACANTHOSIS
NIGRICANS
Acanthosis nigricans is a rare
cutaneous disease, which usually
affects the flexural surfaces of skin
and it has an oral mucosal
component.

3 TYPES:
1.Benign type-
 Present at birth or during puberty.
 Inherited as autosomal dominant trait
 Never associated with internal
malignancy.
2.Malignant type-
 Usually develops after the age of 40 years
 Invariably associated with internal
malignancies like adenocarcinomas or
lymphomas of the GI tract.

3.Pseudoacanthosis type-
 Most common form of acanthosis nigrican
 Lesions develop around body creases as a
result of obesity.

CLINICAL FEATURES
 Pigmented, papillary or verrucous growths
over the axilla, palms, soles & face, etc.
 Dorsum of the tongue exhibits hypertrophy of
the filiform papilla with development of a
shaggy appearance.
 Tongue also presents some areas of
papillomatous growth.

 Lip is grossely enlarged (mainly the upper lip)
and its surface is dotted with small papillo-
matous nodules especially at the commissure.
 Givgiva also exhibits hyperplastic changes and
the lesions are clinically similar to that of the
fibromatosis gingivae.

Buccal mucosa and palate show a
velvety white appearance with
papillary projections in some areas.

HISTOPATHOLOGY
 Thickening of the epithelium with marked
acanthosis.
 Hyperkeratinization of the surface
epithelium.
 Malignant form of the disease shows
marked epithelial hyperplasia and
acanthosis.

Diseases of skin 2011-12 /orthodontic courses by Indian dental academy

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