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DISEASES
OF THE
SKIN
#
ECTODERMAL DYSPLASIA
(hereditary ectodermal dysplasia)
• It represents a group of the inherited disorder in
which two or more ectodermally derived anatomic
structures fail to develop, thus hyperplasia or aplasia
develops.
example-skin, teeth, nails, hair.
• The most common syndrome associated within this
group include are hypohidrotic ED and hidrotic
ED.
#
Clinical features
• EDS is most common in males,
females are carrier
• Dental, nail and hair anomalies are
evident during childhood and
infancy.
• Such patient are intolerant to heat to
the decreased sweat glands. Hence
cannot regulate body temperature.
#
• Salivary gland are developed ectodermally
hence shows marked xerostomia.
• Decreased no of mucous salivary gland.
• Decreased lacrimal gland secretion.
• Deficient hearing and vision.
• Nails are brittle or deformed
• Hair are sparse or there is complete absence
• Tooth shows abnormal morphology.
• Lack of breast development.
• Saddle shaped nose.
#
• Hypohidrotic(anhidrotic)ED-
(CHRIST-SIEMENS-TOURAINE SYNDROME )
It is the most common.
It is usually inherited as an X-linked
recessive trait.
It is characterized by-
Frontal bossing
Sunken cheeks
Thick everted lips
Wrinkled , hyper pigmented skin
around the eyes.
#
Dental manifestation-
1. Conical and pegged
teeth.
2. Hypodontia or
complete
anodontia.
3. Delayed eruption of
permanent teeth.
#
• Presence of eczema
• Short stature
• decreased flow of tears
• photophobia
#
Oral manifestation
• Absence or rudimentary salivary gland.
Hence xerostomia is there.
• Teeth shows abnormal morphology. Shape of
the teeth may be truncate or conical.
• High arch palate , cleft palate.
Protuberant lips.
#
Hidrotic ED (Clouston syndrome)
Nail dystrophy.
Hair defect and palmoplantar
dyskeratosis.
Paronychial infection.
Scalp hair is very thin, fine and
brittle.
Eyebrows are thinned or absent
These patient have normal facies
normal sweating and no specific
dental defect.
#
Histological finding-
There is absence of
• Sweat gland
• Hair follicles
• Salivary gland ,
• Sebaceous gland
• Lacrimal gland
• Sparse or complete absence of hair
#
Treatment-
Dental defects or abnormality can be treated
by proving prosthesis such as complete
denture ,RPD etc
#
Oral lichen planus
(lichen rubber planus)
• It is relatively common chronic dermatological
disease that often effects oral mucosa.
• This condition mainly affects the skin and the
oral mucosa.
• It causes bilateral white striation ,papules and
plaque on tongue ,buccal mucosa and
gingivae.
#
etiology-
1. drug induced(lichenoid drug
reaction)
2. Contact allergen in restorative
material or toothpaste.
3.mechinical trauma.
4.viral infection
5. in nervousness
This disease seldom occur in carefree
people.
#
• Grins pan's syndrome-
it is an syndrome associated
with oral lichen planus which is an triad of
lichen planus, diabetes mellitus and vascular
hypertension.
#
Clinical features-
• Female to male ratio is 1.4:1
• It mainly occur in adults older than 40 years.
• Skin lesions of lichen planus appears as
 Small ,angular, flat topped papules
only few cm to mm in diameter.
These may be discrete earlier and later
on coalesce to form larger plaque.
These are covered by fine glistening
scale.
They are demarcated from skin.
#
 the lesion may appear as reddish ,
purple ,or violaceous hue. Later on dirty
brownish color develops.
Center of the papule is umblicated.
The surface of the lesion is covered with
very fine grayish-white lines called as
Wickham's striae.
#
Location-
Any where on the skin in the bilaterally
symmetrical pattern most often on the-
a) Flexor surface of the wrist and
forearm.
b) Inner aspect of knees, thighs and
trunk.
#
Oral manifestation
characterized by-
 radiating grey or white, velvety,
threadlike papules
Arrangement-
 In linear ,annular or retiform
arrangement forming lacy ,reticular
patches, rings or streak

Location-
 over buccal mucosa and to a
lesser extent to lips ,tongue and
palate.
#
#
• A tiny white elevated dot
present at the intersection of
white lines is known as
striae of Wickham.
• % of distribution of oral
lesions-
85% -buccal mucosa
65% -tongue
20% -gingivae ,floor of the
mouth and palate.
#
Types of lichen planus
• Bullous form of lichen planus
• Erosive formof lichen planus-
 These lesions are more
symptomatic.
Clinically they are atrophic
erythematous area with clinical
ulceration of varying degree.
• Atrophic form of lichen planus-
Appear clinically as smooth red
poorly defined area often but not
always with peripheral striae.
#
• Hypertrophicform-
• it may also occur on the oral mucosa, as a well
circumscribed, elevated white lesion resembling
leukoplakia.
In this case biopsy is necessary to establish
the diagnosis.
• The oral manifestation of lichen planus may occur
weeks or months before the appearance of the skin
lesion.
• Other mucous membrane affected are of penis ,
vagina and epiglottis.
#
• There are variety of drugs that may cause
lesion similar to lichen planus they are termed
as lichenoid lesion.
• Causes-
inflammatory drugs
Sulfonylurea's
 anti malarial
 beta blockers
ACE inhibitors.
idiopathic
• In rare cases it may occur after dental
restoration is performed or when patient start
wearing denture.
#
Histopathological finding
• Typical findings are-
Hyperparakeratosis or hyperorthokeratosis with
thickening of granular layer
Acanthosis with intercellular edema of spinous
cell
 “SAW tooth” shaped rete pegs.
Degenerating basal keratinocytes that form
colloid bodies.
Formation of MAX –JOSEPH SPACES
Subepithelial collection of chronic inflammatory
infiltration is seen in a band like manner.
#
#
• Differential diagnosis
1. Leukoplakia
2. Lichenoid reaction
3. Candidasis
4. Pemphigius
5. Erythema multiforme
6. Recurrant apthae
7. Lupus erythematous
#
Psoriasis
• It is an non-contagious skin disorder that
most commonly appear as inflamed,
edematous skin lesion covered with silvery
white scales
• Most common type of psoriasis is Plaque
psoriasis $ it is characterized by patches on
scalp , trunk and limbs.
• Nails may be pitted or thickened.
#
• Etiology-
Cause is unknown, but do have genetic
prediposition.
It is an autoimmune d/s
Psoriasis is associated with increased activity
of T cell in underlying skin.
 2.5 % persons with HIV develop psoriasis
during the course of the disease
 Stress can cause exacerbation of psoriasis
#
Pathogenesis-
 It is due to the increased turn over rate of
the dermal cells, from the normal turnover
from duration of 23 days to 3-5 days in the
affected area.
Also increase in mitotic index of psoriatic
skin
#
#
Clinical features
• Characterized by-
By occurrence of small ,shapely
delineated, dry papules each covered by
delicate slivery scale which resembles to
that of mica.
If these deep dry scales are removed
bleeding points are disclosed, it is an
characteristic feature of psoriasis and is
called as Auspitz”s sign.
After the removal of scale skin is red
and dusky in appearance.
#
• Cutaneous lesion are painless and seldom
pruritic,they may be few in no.
• Papules enlarge at the periphery and tend to
elevate whereas smaller lesion coalesce to
form larger plaque of irregular outline
Location-
1.On the extensor surface of the
extremities especially elbow ,knee, scalp ,
back, chest , face and abdomen.
• Diseases commences with the appearance of
small papules that gradually increases in
size.
#
• New lesion arises over a period of time .
• The disease may remain static over a period
of time, thus involving more of the skin
surface.
• The disease is more severe in winter and less
in summers due to the increased exposure to
the UV rays.
• Mental anxiety increases the chances of the
diseases.
• Arthritis is complication of psoriasis. It most
frequently increases in 2 to 3 decade of life.
• Psoriasis is more common in females.
#
Oral manifestation
• Lesions are mainly reported on lips , buccal
mucosa, palate, gingiva and floor of the
mouth.
• These lesions are clinically described as gray
or yellowish-white plaque, as silvery white
scaly lesion with erythematous base.
#
Histological features
1. Uniform parakeratosis
2. Absence of stratum granulosum
3. Elongated and clubbing of rete pegs
4. Epithelium over the connective tissue is
thinned and from these points bleeding
occurs when the scales are peeled.
5. .Monor’s abscesses are common.
6. mild lymphocytic and histiocytic infiltration of
connective tissue is common
#
Treatment-
• It include UV-A light.
• Psoralen plus UV-A light .
• Retinoid.
• Methotrexate ,cyclosporine.
#
• It is an ulcerative mucocutaneous
disease of uncertain pathogenesis.
• In erythema multiforme there is mild
skin eruption and mucosal involvement
whereas in erythema multiforme and in
Stevens Johnson's syndrome there is
severe skin and mucosal involvement.
Erythema multiforme
(Stevens-Jhonsons syndrome)
#
• Differentiation b/w Stevens
Johnson's syndrome can only be
made by there lesions
• In EM the lesions is raised
atypical target and mucosal
erosion
• Whereas in SJS there is mucosal
erosion with flat atypical target
cells
#
• Etiology-
1.It is caused by drugs such as sulpha drugs.
2.herpes simplex virus.
3.mycoplasm infection.
Clinical feature-
• It mainly occur in adults.
• It may occur at any age.
• It is most common in 2 and 3 decade.
• It is more common in males than females.
#
• It is characterized by-
Occurrence of
asymptomatic,vividly
erythematous discrete
macules,papule,vesicle
,or bullae in symmetrical
pattern over hand arms
feet legs face and neck.
Lesion may vary from few
cm to mm in diameter.
#
A concentric ring like appearance of
lesion
-resulting from varying shade of erythema
and this gives rise to the term target , iris
or bull’s eye.
They are most common on hands, wrist
, and ankles.
Mucous membrane involvement,
including the oral cavity is common.
Lesions appears within few days or two
and persist for several days to few
weeks and gradually disappears.
#
Oral mucous membrane-
•Lesions are usually not so
significant except the pain and
the discomfort it may cause.
•Hyperemic papule ,macules ,or
vesicles may erode and bleed
profusely.
•Tongue , palate , gingiva are
commonly involved
#
Stevens Johnsons syndrome-
• it is an severe form of erythema
multiforme with widespread
involvement of skin , oral cavity
, eye and genital.
Oral mucous membrane lesion-
1.difficulty in chewing and
mastication
2.severe bleeding and crusting
of the lips
#
Eye lesion-
• Conjunctivitis
• Corneal ulceration.
• Keratoconjunctivitis
• Blindness may occur.
Genital lesions-
• Urethritis
• Vaginal ulcers.
• Balanitis.
#
Histological features
• Cutaneous and mucosal lesion usually exhibit
intercellular edema.
• Edema of superficial CT tissue.
• Zone of severe liquefaction degeneration I
the upper layer of the epithelium.
• Interepithelium vesicle formation and thinning
or absence of basement membrane.
• Dilatation of superficial capilaries.
• Varying degree of inflammatory cell infiltrate.
#
Differential diagnosis-
1. Apthous stomatitis.
2. Contact dermatitis.
3. Stomatitis.
4. Acute necrotizing gingivitis.
5. Pemphigus
6. Dermatitis
7. Bullous lichen planus.
8. Herpes zoster.
9. Chicken pox.
10.Toxic epidermal necrolysis.
#
Toxic epidermal necrolysis
• It is an serious, often fatal
,Bullous drug eruption.
• It is so severe that large
sheet of skin peel off, giving
the appearance of
widespread scalding burn.
• It is considered form of
Stevens Johnson's
syndrome.
#
• Toxic epidermal necrolysis must be
differentiated from Staphylococcal
scalded skin syndrome, which
appear clinically similar even though
the latter is milder diseases with a
better prognosis.
#
• Treatment-
• If the drug is suspected withdraw the drug.
• Infection should be treated after culture.
• Oral antihistamines.
• Analgesics.
• Local skin care.
• Soothing mouth wash.
• Topical steroid.
• oral antacids are helpful in oral ulcers,
• liquid antiseptics such as 0.05 chlorhexidine during
bathing.
• systemic corticosteroids.
#
Mucocutaneous lymph node
syndrome(kawasaki disease)
• It is an acute self limiting febrile illness.
• Mucocutaneous lymph node syndrome ,
Kawasaki syndrome or KD , is an systemic
vasculitis of unknown etiology.
• Hallmark of KD are-
Fever of unknown origin for more than
five days.
 Generalized erythema and
desquamation of skin.
 cervical nonsuppurative
#
 Lymphadenopathy. Swelling of hand and feet.
Etiology-
1.Abnormal inflammatory response triggered by
a neoantigen.
2.Epstein-barr virus.
3.Retrovirus.
4.Streptococcus species.
5.Streptococcus viridians.
6.Chlamydia and pseudomonas species.
#
Clinical features-
• Majority of cases occur b/w three
month and 12 yrs of age.
• KD more often occur in boys than
girls, ratio of about 1.4:1
• SYMPTOMES-
Fever for 5 or more days, with
no response to antibiotics.
Bilat. Congestion of ocular
conjunctivitis.
Reddening of oral and
pharyngeal mucosa.
#
 Changes in lip and mouth
including dryness , redness,
and swelling of tongue
papillae.
 In durative edema and
erythema of palms and
soles.
 Polymorphous exanthema
of torso.
 Acute , non purulent
swelling of cervical lymph
node.
#
• Differential diagnosis-
• No lab test is present for the diagnosis of the
diseases.
• So diagnosis is based on clinical
manifestation.
• It should be distinguished from-
1.Scarlet fever.
2.Erythema multiforme or Stevens-
johnson syndrome.
#
treatment-
• Reduction of the fever. And inflammation.
• Intravenous administration of gamma
globulin.
#
Keratosis follicularis
(Darier diseases
• Also known as Darier-White
disease, or Keratosis follicularis,
Darier's disease.
• It is a rare inherited disease of
keratinization characterized by
scaly, crusted papules typically
occurring on seborrheic areas.
Mucous membrane and nail
changes also occur.
#
• ETIOLOGY & PATHOGENESIS-
• Darier's disease is inherited as an autosomal
dominant trait with high penetrance.
• The Darier's disease gene is located on
chromosome 12 and encodes a adenosine
triphosphatase calcium pump (SERCA 2).
• Mutations of the gene results in abnormality in
epidermal cell-cell adhesion and premature and
faulty keratinization leading to intraepidermal
clefts and dyskeratosis cells.
• Precipitating factors include heat and humidity,
mechanical trauma like friction, sunlight, and
secondary bacterial infections.
#
CLINICAL FEATURES-
• Darier's disease occurs in all races.
• Both sexes are equally affected.
• The onset of disease is during the first
or second decade.
• Initial lesions are skin colored papules
that develop scales or crust on its
surface which, on removal may reveal
a depression. The lesions gradually
darken to take a brownish or blackish
hue.
#
• The discrete, warty papules may
coalesce to become crusted plaques.
On intertriginous areas, vegetative
lesions may become malodorous.
• The distribution is typically on the
seborrheic areas: chest, back, back of
ear, forehead, scalp, neck, nasolabial
folds, groin, and axillae. In extensive
disease, any area of the body may be
involved. The affection is symmetrical.
• The scalp may show greasy scales.
• Localized, unilateral disease along
Blaschko's lines may occur due to
somatic mutation.
#
• Oral mucosa: whitish papules
with central umbilications on
gingiva, palate. Pebbly white
plaques (cobble stoning) may
also be found. Involvement of
oropharynx, trachea and
esophagus has also been
described.
• Nails: Thinning of plate,
hyperkeratosis, longitudinal
ridging, alternate,
longitudinal white and red bands.
V-shaped, triangular scalloping of
distal nail plate is characteristic.
#
DIFFERENTIAL DIAGNOSIS-
• Seborrheic dermatitis
• Hailey-Hailey disease
• Pemphigius vegetans
• Pemphigius foliaceus
• Grover's disease (transient acantholytic
dermatosis)
• Localized form: epidermal nevus
• Histologic D/D: Epidermal nevus, warty
dyskeratoma, actinic Keratosis
•
#
DIAGNOSIS-
• Family history of similar affection in
parents/siblings.
• Typical clinical features of dirty,
Keratosis papules at sites of predilection
• Histology-
• Hyperkeratosis, parakeratosis,
Acanthosis
Suprabasal intraepidermal clefts
Acantholysis
Dyskeratosis cells in spinous
layer ( corps ronds) and keratin layer
(grains)
#
COURSE AND PROGNOSIS-
• Darier's disease runs a life-long course.
• Seasonal variations in severity occurs.
TREATMENT-
• Avoidance of frictions/mechanical irritations.
• Use of sunscreens.
• Topical retinoid: tazarotene, adapalene
• Systemic retinoid: isotretinoin or acitretin
may induce temporary remission.
#
• Systemic retinoid: isotretinoin or acitretin
may induce temporary remission.
• Topical and systemic antibiotics to treat
secondary bacterial infections.
• Hypertrophic lesions may be treated with
dermabrasion, excision and grafting, or laser
excision.
#
White sponge nevus
familial white folded dysplasia of mucous mem, cannons
disease, white folded gingivostomtitis
• It is an uncommon condition
of oral mucosa.
• Clinical features-
• This mucosal abnormality is
congenital in many instances.
• It does not appear until
infancy, childhood, or even
adolescence, by this time it
reaches its full extent.
#
Oral lesions-
The lesions involve the
• cheek ,
• palate,
• gingiva,
• floor of the mouth
• and portions of the mouth.
 The mucosa appear
thickened and folded or
corrugated with a soft or
spongy texture and a
peculiar white opalescent
hue.
#
• Sometimes a minimal
amount of folding is
present.
• Ragged white area can
be scrapped out without
ensuing bleeding.
Histological features-
• Epithelium is thickened.
• Hyperkeratosis and
Acanthosis.
• Basal layer is intact.
• Intercellular edema.
#
• Vacuolated cells may
show pyknotic nuclei.
• Para keratin plugs
running deep into the
spinous layer.
• Sub mucosa may show
mild inflammation.
• Treatment and prognosis-
• There is no t/t.
• Its benign, the prognosis
is excellent.
• Has no complication.
The microscopic features of
white sponge nevus are
distinctive. There are two
characteristic cell types:
dense keratinized cells and
pale vacuolated ones. There
is no evidence of dysplasia
or anaplasia in this
condition
#
Pemphigus
• It is an serious chronic disease characterized
by the appearance of vesicles and bullae with
small or large fluid filled blisters that develop
in cycles.
• Pemphigus includes a group of autoimmune
blistering disease of the skin and the mucous
mem.
• Characterized histologically by intradermal
blisters and immunologically by the finding of
the circulating IgG antibodies.
#
– There are primary three
types of Pemphigus:-
1. Pemphigus
vulgaris (PV)
2. Pemphigus
foliaceus
3. Pemphigus
paraneoplastic.
• Pemphigus vulgaris
accounts for approx 70%
of Pemphigus.
#
Pemphigus vulgaris
• Pemphigus vulgaris is an
auto immune disease of
the skin and the mucous
mem.
• Blister in PV is associated
with the binding of IgG
auto antibodies to
keratinocytes cell surface.
• Pemphigus antibodies
bind to keratinocytes cell
surface molecule
desmoglein 1 and 3.
#
Clinical features-
• Pemphigus vulgaris affects all
races, with equal gender
distribution among males and
females.
• Mean age of onset is approx 50-
60yrs.
• It is characterized by rapid
appearance of vesicles and
bullae, varying in dia. from few cm
to mm , in such large skin surface
is covered
• Lesions are fluid filled soon after
development and soon become.
#
-purulent, and soon the
skin become eroded
when the bullae
rupture.
• The characteristic
feature of Pemphigus is
NIKOLSKY’S SIGNS,
in which there is loss of
epithelium by rubbing
the unaffected skin.
• It is caused by the peri
vesicular edema which
disrupts the dermal
epidermal junction.
#
• Nikolsky's sign is a
condition caused by a
staphylococcal infection
• In which the superficial
layers of skin slip free
from the lower layers
with a slight rubbing
pressure. Large areas of
the skin will blister and
peel away leaving wet,
red and painful areas
#
• Nikolsky's sign is either positive or
negative. A positive result may be
present in several different medical
conditions. Persons with a positive sign
have loose skin that slips free from the
underlying layers when rubbed. The
area beneath is pink and moist and may
be very tender.
• Typically, your health care provider will
test for this sign simply by twisting a
pencil eraser against your skin. If
positive, a blister will form in the area,
usually within minutes
#
• Pemphigus vegetans is an uncommon variant of
the Pemphigus vulgaris.
• It occur in 1-2% of the case of the PV.
• The median age of onset of the Pemphigus
vegetans is 40-50yrs.
• Its two subtypes are-
1. Neumann (flaccid bullae and erosions)
2. Pustules(Hallopeau)
• Both the subtypes develops into hyper pigmented
vegetative plaque with pustules and hypertrophic
granulation at the periphery.
• Its characteristic feature is the cerebriform tongue
,characterized by the presence of sulci and gyri on
the dorsum of the tongue.
#
Oral manifestation-
• Mucous mem is typically
affected in PV. The disease
typically the mucous mem in
50-70%.
• Patients typically have ill
defined, irregularly shaped
gingival buccal or palatine
erosions which are painful
and slow to heal.
• The erosion extend
peripherally with the shedding
of the epithelium.
• Erosion may be seen on any
part of the oral cavity.
#
• Erosions may spread
to involve the larynx
with subsequent
hoarseness.
• The patient is often has
difficulty in eating and
drinking because of the
lesion.
• Other mucosal surface
involve conjunctiva,
esophagus, labia ,
vagina , cervix, penis ,
urethra and anus.
#
Histological finding-
• Pemphigus is
characterized by the
presence of the
formation of vesicle or
bullae entirely
intraepithelially and
just above the basal
layer producing the
distinctive
“suprabasilar split”
•Suprabasal separation of
epithelium
•Intraepithelial vesicle
#
• Perivascular edema
appears to weaken this
junction, and the
intercellular bridges
between the epithelium
cells disappear.
• This results in the loss of
cohesiveness or
Acantholysis
Acantholyis
#
• The clumps of the
epithelial cells are
found lying free with in
the vesicular space.
these have been called
as “Tzank cells” and
are characterized
particularly by
degenerative changes
which include swelling
of the nuclei and hyper
chromatic staining. Acantholysis - Tzank cells
(epithelial cells which are
smaller, rounder)
#
• The fluid in most vesicle ,particularly those
more than a day or two old,
Contains-
• Polymorhonuclear leukocytes
• Lymphocytes
• Scarce inflammatory cell infiltrate, in
both the vesicular fluid and in the
connective tissue at the base of the
vesicles or bullae is suggestive of
Pemphigus.
#
• Immunoflurescent test
has proven to be of
great value in estb the
diagnosis of
Pemphigus.
• Direct
immunofluorescence-
Is used to demonstrate
the presence of
immunoglobuluns,pred
ominantly IgG, and
sometimes C3, IgA and
IgM.
Direct immunofluorescence:
IgG deposition in intercellular
areas
#
• A biopsy of a skin sample is treated in the
laboratory with a chemical compound to find the
abnormal desmoglein antibodies that attack the
skin. The specific type of antibodies that form
may indicate what type of Pemphigus exists.
• Indirect immunofluorescence-.
• Sometimes called an antibody titer test, a
sample of blood is tested to measure
Pemphigus antibody levels in the blood and help
determine the severity of the disease. Once
treatment begins, this blood test may also be
used to find out if treatment is working
#
• Indirect
immunofluorescence has
also been highly beneficial.
this is accomplished by
incubating normal animal
or human mucosa with
serum from the patient
suspected of having the
diseases and adding
fluorescein conjugated
human antiglobulin.
• Positive reaction indicates
presence of circulating
immunoglobulin
antibodies.
Direct
immunofluorescence:
IgG deposition in
intercellular areas
#
• Differential diagnosis-
1. Dermatitis herpetiformis.
2. Erythema multiforme
bullosum
3. Bullous lichen plan us
4. Epidermolysis bullosa
5. Bullous pemphigoid
6. Cicatricial pemphigoid.
#
• Treatment-
• T/t for Pemphigus vulgaris involves using one or
more drugs.
• High-dose oral corticosteroids, such as prednisone
or prednisolone, are the main treatment for
Pemphigus. These are anti-inflammatory medicines
that suppress the immune system.
• High doses are often required to bring Pemphigus
under control. To minimize the side effects patients
may experience, once the disease begins to
subside the corticosteroid levels are reduced slowly
to the lowest level required to prevent new blisters
or sores from appearing.
#
• Many patients will go into complete
remission with treatment, although this may
take a number of years. Other patients will
need to continue to take small doses of
medication to keep the disease under control
• Prednisone is usually taken by mouth, but
can also be injected into a vein, muscle, or
directly into a blister. The route depends on
the type and severity of disease. Usually, a
corticosteroid cream will be used directly on
the blisters.
#
• To keep the levels of corticosteroid use to a
minimum, immunosuppressive drugs are
often added to a patient’s treatment. These
are drugs that stop or slow down the immune
system’s response to what it sees as an
attack on the body. They include:
• Myco-phenolate mofetil (CellCept)
• Azathioprine (Imuran)
• Cyclophosphamide (Cytoxan)
• Methotrexate.
• Other drugs that may be used include:
• Dapsone (DDS)
• Antibiotics such as tetracycline.
#
Pemphigus foliaceus
• Pemphigus foliaceus is a benign
condition of pemphigus.
• It is an autoimmune skin disorder
characterized by the loss of
intercellular adhesion of
keratinocytes in the upper part of
the
epidermis(acantholysis),resulting in
the formation of superficial blister.
• It is characterized by the
involvement of healthy appearing
skin that blisters when
rubbed(Nikolsky’s sign).
#
• PF is
characterized by
a chronic course,
with little or no
involvement of
mucous mem.
• Superficial
blisters are
induced by IgG
autoantibobies
directed against a
cell adhesion
molecule
,desmoglein 1
#
• Subtypes of pemphigus foliaceus
are-
1. Pemphigus erythematosus.
2. Pemphigus herpetiformis.
3. Endemic pemphigus foliaceus
4. Immunoglobulin pemphigus foliaceus.
5. Drug induced pemphigus foliaceus.
• Clinical features-
• PF is characterized by early bullous
lesions which rapidly rupture and
dry to leave masses of flakes or
scales suggestive of an exfoliative
dermatitis or eczema.
• It is a mild form of pemphigus and
common in older adults.
#
• Histological finding-
• Acantholysis of upper
epidermis.
• It usually detaches
without bullae
formation. more estb
lesions have
acanthosis and mild to
moderate
papillomatosis.
• Hyperkeratosis and
parakeratosis, with
dyskeratosis cell within
the granular layer
#
Treatment-
• Therapy for PF is usually less aggressive that
of PV ,because of low mortality and morbidity
rate.
• Non steroidal t/t is necessary.
• Mestinon is used to slow down the
progression of the disease.
#
Paraneoplastic
pemphigus (PNP)
demonstrating an
erosive cheilitis. This
is a frequent feature
in PNP and often
responds very poorly
to treatment.
Paraneoplastic pemphigus is the most
fatal type of the pemphigus.
It may even cause death due to the
multi organ failure and respiratory
failure, thus causing direct effect on the
respiratory epithelium.
The of diagnosis of paraneoplastic
pemphigus involves-
Paraneoplastic pemphigus
1. Painful mucosal erosion , sometimes
with a skin eruption that results in
blister and erosions.
2. Histopathological changes of
acantholysis ,keratinocytes , necrosis
and dermatitis.
3. Direct immunofluorescence revels IgG
and complement C3 .
#
4. Indirect immunofluorescence revels circulating
antibodies specific for stratified squamous
epithelium.
Etiology-
• Tumor antigen are believed to evoke an
immune response that led to the
development of autoimmune response to
intercellular adhesins.
• Autoantibobies leads to blistering in the
mucosa and other epithelium.
#
Clinical features-
• Mean age of onset is 60 yrs.
• Male and females are equally affected.
• All the patient with paraneoplastic pemphigus have
tumor, most of which are malignant.
• Most common malignancy associated are-
1. Non Hodgkin's lymphoma
2. Chronic lymphocytic leukemia.
3. Castel man tumor
4. Giant cell lymphoma.
5. Macroglobulinemia.
6. thymoma,.
7. Poorly differentiated sarcoma.
8. Bronchogenic squamous cell carcinoma.
#
Oral manifestation-
• Patient has oral ulceration and
erosions.
• Patient with oral erosions often has
cutaneous eruptions.
• Erosions has wide variety of
patterns.
• Erosions can occur anywhere in
the mouth.
• Erosions and subsequent crusting
of the vermillion border of the lips
are typical and is similar to Stevens
Johnson's syndrome.
#
Histological finding-
• Vesicular lesions express the most characteristic
histopathologic features. Oral and cutaneous
lesions show variable epidermal necrosis,
suprabasal acantholysis, dyskeratotic keratinocytes,
vacuolar interface dermatitis, and lymphocytic
exocytosis. Substantial inflammation can be
present, even in early lesions
• Oral mucosal lesions show the greatest
acantholysis, while some skin lesions may not have
any acantholysis.
• When present, suprabasal acantholysis can result
in clefts and tomb stoning, which is the appearance
of the basal cell layer below the cleft, and it can be
indistinguishable from pemphigus vulgaris.
#
• A distinctive feature of paraneoplastic
pemphigus is dyskeratosis.
• The presence of dyskeratosis in a person with a
suprabasal acantholytic bullous disorder is a
clue to the presence of paraneoplastic
pemphigus.
• Interface dermatitis is a frequent finding in
persons with paraneoplastic pemphigus, and it
can be found both with and without acantholysis.
• Exocytosis of inflammatory cells into the
epidermis is common.
• Dermal changes include a superficial
perivascular infiltrate of variable intensity, which
is mostly composed of lymphocytes.
#
• The inflammation can sometimes be lichenoid,
leading to a misdiagnosis of lichen planus.
Papillary dermal edema is present in early lesions,
whereas older lesions may exhibit mild dermal
fibrosis.
Treatment-
1. Warm compresses.
2. Non adherent dressing.
3. Topical antibiotics ointments.
4. Potent immunosuppressive.
In general-
skin lesions are more responsive than that of
mucosal leions.
#
• Other therapeutic options include-
plasmapheresis
immunophoresis.
• For solid neoplasm-
curative resection.
• The prognosis for paraneoplastic pemphigus
is poor.
#
Familial benign pemphigus
• Familial benign pemphigus originally was
described by the Hailey brothers in 1939.1 It
is a chronic autosomal dominant disorder with
incomplete penetrance. Approximately two
thirds of patients have a family history of the
disorder. A history of multiple relapses and
remissions is characteristic. Decreased
numbers of desmosomes have been
implicated in the pathogenesis of benign
familial pemphigus. Therapeutic options are
limited
#
Clinical features-
• The disease first manifest itself during
adolescence or young adult life.
• There is no predilection in either gender.
• Lesions develops as small vesicles appearing on
normal or erythematous skin, which soon rupture
to leave eroded skin.
• Lesion appear too enlarge on periphery and heal
at center.
• Heat and sweating increases its outbreak,
spontaneous remission occur in cold weather.
• Lesion develop in the area of excessive friction.
#
• Tender and enlarged lymph node are also present.
• It is been seen that bacterial infection ppt the
appearance of the lesion, mostly by Candida
albicans.
Oral manifestation-
• Oral lesions rarely occur in such patients.
• The lesions develop as crop of vesicles and rupture
rapidly leaving raw eroded area.
histological features-
• It bears close resemblance to PV, Keratosis
follicularis and Darier's disease.
• It as extensive acantholysis than PV.
• less damage to acantholytic cells.
#
• Characteristic feature of this disease is that
intercellular bridges persist so that adjacent
epithelial cell still adhere to each other and
are not entirely acantholytic.
• This appearance is called as “dilapidated
brick wall "effect.
• Treatment-
1. Soothing compress followed by mild
use of corticosteroid prep
2. topical antibiotics.
3. Erythromycin and tetracycline.
4. Bacterial culture and sensitivity test to
help guide appropriate therapy.
#
Cicatricial pemphigoid
(benign mucous membrane
pemphigoid, ocular pemphigus)
• Cicatricial pemphigoid is
an auto immune blistering
disease that affects the
mucous mem including
the mouth , oropharynx ,
conjunctiva ,nares and the
genitalia.
• Patient with cutaneous
involvement present with
tense blister and erosion,
often on head and neck.
Cicatricial pemphigoid may
affect skin as well as the
mucous membranes, usually on
the head and neck (termed
the Brunsting Perry type).
#
Etiology-
• Cicatricial pemphigoid (CP) is due to antibodies
that target several potential antigens.
Clinical features-
• the disease occur twice as frequently in females
as that in males
• Peak age of involvement is 40-50yrs.
• Vesicobullous lesion occur on the oral mucous
mem and conjunctiva.
• Lesion also occur on skin particularly around
genitalia.
• Lesion heal by scar formation, particularly around
conjunctiva.
#
• Other mucous mem surfaces to
be involved are nose , larynx ,
pharynx , esophagus.
• Ocular involvement is the most
serious complication. following
initial conjunctivitis, adhesion
develops between the palpebral
and bulbar conjunctiva resulting
in obliteration of palpebral
fissure c/a adhesions symblephoron
formation-closes of the opening of lachrymal
gland –blindness and scar formation.
• with opacity and thus complete
blindness
#
Oral manifestation-
• Gingivitis in a patient with Cicatricial
pemphigoid. This pattern of
periodontal, chronic, red, velvety
gingivitis is very suggestive of this
disorder but may occur in other
blistering diseases.
• the mucosal lesion are
vesiculobullous in nature but
appear to be relatively thick walled
so that it persist for 24-48hr before
rupturing.
• Ruptured surface do not leave raw
eroded bleeding surface.
#
• Cicatricial pemphigoid
affecting the tongue. The
lesions are rather annular,
and may be mistaken for
migratory glossitis
(geographic tongue)unless
there are other associated
features.
• Involvement of the palate in
Cicatricial pemphigoid,
manifest as purpuric spots.
Intraoral lesions of
dermatitis herpetiformis may
also produce similar
purpuric lesions..
#
Histological finding-
• In the histological finding of the CP , the
vesicles and bullae are sub epidermal rather
than suprabasilar and there is no evidence of
acantholysis.
• The basement structure appear to detach
with the epithelium from underlying CT.
• There is chronic inflammatory cell infiltrate in
CT , chiefly lymphocytes ,plasma cells and
eosinophils.
• Immunofluorescence studies reveled the
presence of tissue bound basement mem
zone antibodies,as well as circulating
antibasement mem zone antibodies in serum
#
• Immunofluorescence pattern of CP was
indistinguishable from the pattern observed in
bullous pemphigoid.
Differential diagnosis-
1. Pemphigus vulgaris
2. Bullous pemphigoid.
3. Erosive lichen planus.
4. Bullous erythema multiforme.
Treatment-
• The goal of treatment is to suppress the blister
formation and to promote the healing and to
prevent scarring.
#
• The lowest dose of medication to suppress
disease activity and to minimize risk.
• It has severe complication that causes visual
loss or blindness , airways stenosis ,
esophageal stricture, cutaneous blistering
and milia formation.
#
Bullous pemphigoid
• BP is a chronic ,
autoimmune ,sub
epidermal , blistering
skin disease that
rarely involves
mucous mem.
• It is characterized by
the presence of IgG
auto antibodies.
#
What Is Pemphigoid, and How Is It Different From
Pemphigus?
• Pemphigoid is also a blistering disorder caused
by autoimmune problems that result in an attack
on the skin cells by a person’s own antibodies.
Pemphigoid produces a split in the cells where
the epidermis and dermis, the layer below the
epidermis, meet, causing deep, tense blisters that
do not break easily. Pemphigus, on the other
hand, causes a separation within the epidermis,
and the blisters are soft, limp, and easily broken.
Pemphigoid is seen most often in the elderly and
may be fatal. Usually, both pemphigus and
pemphigoid are treated with similar medications.
Severe cases may require different treatment
#
COMPARISON BETWEEN BULLOUS
PEMPHIGOID AND PEMPHIGUS
VULGARIS
Characteristic Bullous pemphigoid Pemphigus
Frequency Uncommon Rare
Age group Most > 70 years Often 50-60 years
Blister morphology Tense, clear fluid content
May be pompholyx changes
on palms or soles
Fragile, may just be
blister remnants at
the margin of
erosions
Adjacent skin May be urticated or
eczematous
Normal, but may be
able to induce
blister by shearing
force (Nikolsky's
sign)
Mucous membrane
involvement
About 25%, usually minor Present in 80%,
typically precedes
skin involvement by
several months
Direct
immunofluorescence
Positive at basement
membrane zone
Positive epidermal
intercellular
Response to treatment Usually good Often refractory
#
Pathogenesis-
• IgG autoantibodies
• Skin basement membrane
• Complement and inflammatory mediators
• Inflammatory cells to the basement mem.
• Proteases which degrades hemidesmosomal
proteins.
leads to blister formation
Bind to
activates
attracts
release
#
Clinical features-
• It is basically a disease of elderly
people.
• Mainly occur at the age of 60 yrs
to above.
• It can even occur earlier I life.
• There is no gender predilection.
Cutaneous lesions-
• Generalized nonspecific rash,
commonly on limbs, which
appears urticarial or eczematous
and may persist for several
weeks or months before its
ultimate appearance
#
• Vesicles and bullae are relatively thick walled and
may remain intact for some days.
• Rupture does not always occur, although when it
occur it leaves raw eroded area which heals
rapidly.
Oral manifestation-
• Frequency of oral lesion are very less.
• Oral lesion BP are described as vesicles and
area of erosions and ulceration.
• Its important feature is its similarity to the gingiva
lesions to those of the Cicatricial pemphigoid.
• Gingival lesions involve the gingival mucosa and
it is extremely painful.
#
• The gingival tissue appear extremely
erythematous and may even desquamate as a
result of minor friction.
• Hence vesicles form and erosion may occur.
• Lesion may also occur on buccal mucosa ,
palate, floor of the mouth and tongue.
Histological features-
• Vesicle and bullae in it are sub epidermal and
non specific.
• There is no evidence of acantholysis of
epithelial ; in fact the epithelium is normal.
• Vesicles contain fibrinous exudates admixed
with inflammatory cells
#
• Electron microscope studies revel-
basement mem remain attached to the CT
• Ultra structural studies revel-
 primary changes in bullous pemphigoid
appear to occur in the CT where the blood
vessels shows alteration in their penetrability.
Basement mem shows thickening with
interruption in continuity.
• Indirect immunofluorescence studies revel-
circulating basement mem zone antibodies
are found.
• Direct immunofluorescence studies revel-
tissue bound antibasement mem zone
antibodies
#
Treatment-
1. To decrease blister formation.
2. To promote healing of blisters and erosions.
3. Therapy must be individualized for each
patient.
4. Treatment for most of the patients require 6-
60 months.
#
Systemic lupus erythematosus
• Systemic lupus
erythematosus is an
autoimmune disease
characterized by the
autoantibodies, immune
complex formation, and
immune dysregulation
resulting in damage to
essentially any organ,
including kidney, skin,
blood cells and the CNS.
#
Etiology-
• The precise reason for the abnormal
autoimmunity that causes lupus is not known.
• Inherited genes, viruses, ultraviolet light, and
drugs may all play some role.
• Genetic factors increase the tendency of
developing autoimmune diseases, and
autoimmune diseases such as lupus, rheumatoid
arthritis ,
• disorders are more common among relatives of
patients with lupus than the general population.
• immune system in lupus is more easily stimulated
by external factors like viruses or ultraviolet light.
#
• Sometimes, symptoms of lupus can be
precipitated or aggravated by only a brief
period of sun exposure.
• Dozens of medications have been reported to
trigger SLE; however, more than 90% of this
"drug-induced lupus" occurs as a side effect of
one of the following six drugs:
• hydralazine (used for high blood pressure),
• quinidine and procainamide (used for abnormal
heart rhythm),
• phenytoin (used for epilepsy),
• isoniazide (used for tuberculosis),
• d- penicillamine (used for rheumatoid arthritis). .
#
• These drugs are known to stimulate the immune
system and cause SLE. Fortunately, drug-induced
SLE is infrequent (accounting for less than 5% of
SLE among all patients with SLE) and usually
resolves when the medications are discontinued.
• It also is known that some women with SLE can
experience worsening of their symptoms prior to
their menstrual periods. This phenomenon,
together with the female predominance of SLE,
suggest that female hormones play an important
role in the expression of SLE. This hormonal
relationship is an active area of ongoing study by
scientists.
#
Clinical features-
• It is an serious cutaneous systemic disorder
• Disease is at its peak at the age of 30 yrs in
female and 40 yrs in male.
• Prevalence rate is higher in female than males.
• Female to male ratio is 2:1 before puberty and
after puberty the ratio is 4 :1
• Patients with SLE can develop different
combinations of symptoms and organ
involvement.
• Common complaints and symptoms include
• fatigue,
• low-grade fever,
#
• loss of appetite,
• muscle aches,
• arthritis,
• ulcers of the mouth and nose, facial rash
("butterfly rash"),
• unusual sensitivity to sunlight
(photosensitivity),
• inflammation of the lining that surrounds the
lung (pleuritis) and the heart (pericarditis),
and
• poor circulation to the fingers and toes with
cold exposure (Raynaud's phenomenon).
#
• Most patients with SLE
will develop arthritis
during the course of
their illness. Arthritis in
SLE commonly involves
swelling, pain, stiffness,
and even deformity of
the small joints of the
hands, wrists, and feet.
Sometimes, the arthritis
of SLE can mimic that
of rheumatoid arthritis
(another autoimmune
disease).
#
• Inflammation of the lining of the lungs
(pleuritis) and of the heart (pericarditis) can
cause sharp chest pain. The chest pain is
aggravated by coughing, deep breathing, and
certain changes in body position. The heart
muscle itself rarely can become inflamed
(carditis). It has also been shown that young
women with SLE have a significantly
increased risk of heart attacks from coronary
artery disease.
• Kidney inflammation in SLE can cause
leakage of protein into the urine, fluid
retention, high blood pressure, and even
kidney failure
#
• Involvement of the brain can cause
personality changes, thought disorders
(psychosis), seizures, and even coma.
Damage to nerves can cause numbness,
tingling, and weakness of the involved body
parts or extremities. Brain involvement is
called cerebritis.
• Many patients with SLE experience hair loss
(alopecia). Often, this occurs simultaneously
with an increase in the activity of their
disease.
#
Q- How is lupus diagnosed?
• Since patients with SLE can have a wide
variety of symptoms and different
combinations of organ involvement, no single
test establishes the diagnosis of systemic
lupus.
• To help doctors improve the accuracy of the
diagnosis of SLE, eleven criteria were
established by the American Rheumatism
Association.
#
• These eleven criteria are closely related to the symptoms.
Some patients suspected of having SLE
may never develop enough criteria for a
definite diagnosis. Other patients
accumulate enough criteria only after
months or years of observation. When a
person has four or more of these
criteria, the diagnosis of SLE is strongly
suggested
#
• The eleven criteria used for diagnosing systemic lupus erythematosus are:
1.malar (over the cheeks of the face) "butterfly" rash.
2.Discoid skin rash: patchy redness that can cause
scarring
3.Photosensitivity: skin rash in reaction to sunlight
exposure
4.Mucus membrane ulcers: ulcers of the lining of the mouth,
nose or throat
5.Arthritis: 2 or more swollen, tender joints of the
extremities
#
6.Pleuritis/pericarditis: inflammation of the lining tissue
around the heart or lungs, usually associated with
chest pain with breathing
7.Kidney abnormalities: abnormal amounts of urine protein
or clumps of cellular elements called casts
8.Brain irritation: manifested by seizures (convulsions)
and/or psychosis
9.Blood count abnormalities: low counts of white or red
blood cells, or platelets
10.Immunologic disorder: abnormal immune tests include
anti-DNA or anti-Sm (Smith) antibodies, falsely
positive blood test for syphilis, anticardiolipin
antibodies, lupus anticoagulant, or positive LE
prep test
#
• 11.Antinuclear antibody: positive ANA antibody
testing.
Other tests can be helpful in evaluating patients with SLE to
determine the severity of organ involvement. These include-
1. routine testing of the blood to detect
inflammation (for example, a test called the
sedimentation rate),
2. blood chemistry testing,
3.direct analysis of internal body fluids,
4.and tissue biopsies. Abnormalities in body
fluids and tissue samples (kidney, skin, and
nerve biopsies) can further support the
diagnosis of SLE.
#
Oral manifestation-
• Oral mucous mem involvement is reported in 20-
50 % cases of discoid lupus erythematosus and
slightly more frequently in systemic form of the
disease.
• Oral lesion are formed either prior to or following
the development of skin lesions or even in the
absence of skin lesion.
• Oral lesion in SLE are very similar to that to DLE
except that hyperemia, edema and extension of
the lesion is more pronounced and there may be
more tendency towards bleeding, petechiae and
superficial ulceration which is surrounded by red
halo as a result of superficial telengiectasis.
#
Histological finding-
• Histological appearance of SLE and DLE is
similar, only differs in in the degree of certain
findings.
#
Laboratory finding-
• A specific test have been estb for its diagnosis-
the test consist of addition of blood serum from
a person under suspicion to the buffy coat of
normal blood. if the patient is suffering from SLE
,typical LE cells will develop. this cell or
phenomena consist of a rosette of neutrophils
surrounding a pale nuclear mass apparently
derived from a lymphocytes.
• There is also anemia ,leukoplakia
thrombocytopenia and elevated sedimentation
rate as well as elevated serum gamma globulin
level and a positive Coombs test, in a significant
% of people with either SLE or DLE.
#
• Antinuclear antibodies can be demonstrated in
patient of SLE and DLE.
Treatment-
• The most imp tool is careful and frequent clinical
and laboratory evaluation to tailor medical
regime.
• There is no permanent cure for SLE. The goal of
treatment is to relieve symptoms and protect
organs by decreasing inflammation.
• Many patients with mild symptoms may need no
treatment or only intermittent courses of anti-
inflammatory medications.
#
• Those with more serious illness involving
damage to internal organ(s) may require high
doses of corticosteroids in combination with other
medications that suppress the body's immune
system.
• Patients with SLE need more rest during periods
of active disease. Researchers have reported
that poor sleep quality was a significant factor in
developing fatigue in patients with SLE
• Nonsteroidal anti-inflammatory drugs (NSAIDs)
are helpful in reducing inflammation and pain in
muscles, joints, and other tissues. Examples of
NSAIDs include aspirin, ibuprofen (Motrin),
naproxen (Naprosyn), and sulindac (Clinoril). S
#
Discoid lupus erythematosus
• It is an chronic scarring atrophy
producing photosensitive dermatosis.
• Discoid lupus erythematosus (DLE) is
a disease in which coin-shaped
(discoid) red bumps appear on the
skin.
• The disease called discoid lupus
erythematosus only affects the skin,
although similar discoid skin lesions
can occur in the serious disease
called systemic lupus erythematosus
(SLE)..
#
• The tendency to
develop DLE seems
to run in families.
Although men or
women of any age
can develop DLE, it
occurs in women
three times more
frequently than in
men. The typical DLE
patient is a woman in
her 30s.
#
Etiology-
• It mainly occur in genetically predisposed
individual.
• It has been sugested that heat shock protein
is induced in the keratinocytes following
ultraviolet light exposure and stress and the
protein may act as a target for T-cell.
Clinical features-
• In DLE, the characteristic skin lesion is
circular and raised.
#
• The reddish rash is about 5-10 mm in
diameter, with the center often somewhat
scaly and lighter in color than the darker outer
ring. The surface of these lesions is
sometimes described as "warty.“
• There is rarely any itching or pain associated
with discoid lesions.
• They tend to appear on the face, ears, neck,
scalp, chest, back, and arms.
• DLE lesions heal, they leave thickened,
scarred areas of skin. When the scalp is
severely affected, there may be associated
hair loss (alopecia).
#
• People with DLE tend to be quite sensitive
to the sun. They are more likely to get a
sunburn, and the sun is likely to worsen
their discoid lesions.
• The typical cutaneous lesion are slightly
elevated red or purple macules that often
covered with gray or yellow adherent
scales. forceful removal of scales revel
numerous ‘carpet tack’ extensions which is
dipped into enlarged pilosebaceous ca
• The lesion increase in size by peripheral
growth this is its typical feature.
#
• The periphery of the lesion appear pink
or red while the center exhibit an atrophic
scarred appearance indicative of long
standing nature of the disease with
characteristic healing.
• the discoid form of the disease may also
assume a typical ‘butterfly’ distribution on
the malar process of the nose. since it is
not a constant feature as it may appear
in some other disease also. so its
diagnostic significance should not be
overemphasized.
#
• Oral manifestation-
• There is 20-25 % of oral mucous mem
involvement in DLE.
• Oral mucosa is either involved prior or following
the development of the skin lesion. The oral
lesion begin as erythematous area ,sometimes
slightly elevated but more often depressed
usually without induration and typically with white
spots.
• Superficial ulcers with crusting or bleeding may
form but without actual scales. Margins of lesions
are not sharply demarcated but shows narrow
zone of keratinization.
#
• Central healing may result in
depressed scarring.
• Lesions are mainly on buccal
mucosa ,palate and tongue.
• In case of tongue atrophy of
papillae and severe fissuring is
seen.
• Vermillion border of the lip is also
involved.
• The erythematous atrophic plaque
surrounded by a kera5otic border
may involve the entire lip.
Ulceration of the palate
Ulceration of buccal mucosa
#
Histological finding-
• It is characterized
by hyperkeratosis
with keratotic
plugging, atrophy
of rete pegs,
perivascular
infiltration of
lymphocytes and
their collection in
dermal
appendages
#
• Liquefaction
degeneration of basal
layer of cell.
• Discoid form of the
lesion exhibit
hyperorthokeratosis
hyper parakeratosis.
• Acanthosis and
pseudoepitheliomatous
hyperplasia.
• Hydrophobic
degeneration and
liquefaction necrosis of
basal cell layer occurs
#
• Thickening of the basement mem can be
demonstrated as a homogenous broad
eosinophillic and PAS positive band.
• Diffuse infiltration of lymphocytes with
smaller number of plasma cells and
occasionally polymorphonuclear
leukocytes in superficial in deep CT
tissue.
Diagnosis-
• Diagnosis of DLE usually requires a skin
biopsy.
#
• A small sample of a discoid lesion is
removed, specially prepared, and examined
under a microscope. Usually, the lesion has
certain microscopic characteristics that allow
it to be identified as a DLE lesion.
• Blood tests will not reveal the type of
antibodies present in SLE, and physical
examination usually does not reveal anything
other than the skin lesions. If antibodies exist
in the blood, or if other symptoms or physical
signs are found, it is possible that the discoid
lesions are a sign of SLE rather than DLE.
#
Treatment-
• Treatment of DLE primarily involves the use
of a variety of skin creams. Sunscreens are
used for protection. Steroid creams can be
applied to decrease inflammation.
Occasionally, small amounts of a steroid
preparation will be injected with a needle into
a specific lesion. Because of their long list of
side effects, steroid preparations taken by
mouth are avoided. Sometimes, short-term
treatment with oral steroids will be used for
particularly severe DLE outbreaks.
Medications used to treat the infectious
disease malaria are often used to treat DLE.
#
Alternative treatment-
• Alternative treatments for DLE include eating
a healthy diet, low in red meat and dairy
products and high in fish containing omega-3
fatty acids. These types of fish include
mackerel, sardines, and salmon. Following a
healthy diet is thought to decrease
inflammation. Dietary supplements believed
to be helpful include vitamins B, C, E, and
selenium. Vitamin A is also recommended to
improve DLE lesions. Constitutional
homeopathic treatment can help heal DLE as
well as help prevent it developing into SLE.
#
Prognosis-
• For the most part, the prognosis for people
with DLE is excellent. While the lesions may
be cosmetically unsightly, they are not life
threatening and usually do not cause a
patient to change his or her lifestyle. Only
about 10% of patients with DLE will go on to
develop SLE.
Prevention-
• DLE cannot be prevented. Recommendations
to prevent flares of DLE in patients with the
disease include avoiding exposure to sun and
consistently using sunscreen.

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skin diseases shaffer................ppt

  • 2. # ECTODERMAL DYSPLASIA (hereditary ectodermal dysplasia) • It represents a group of the inherited disorder in which two or more ectodermally derived anatomic structures fail to develop, thus hyperplasia or aplasia develops. example-skin, teeth, nails, hair. • The most common syndrome associated within this group include are hypohidrotic ED and hidrotic ED.
  • 3. # Clinical features • EDS is most common in males, females are carrier • Dental, nail and hair anomalies are evident during childhood and infancy. • Such patient are intolerant to heat to the decreased sweat glands. Hence cannot regulate body temperature.
  • 4. # • Salivary gland are developed ectodermally hence shows marked xerostomia. • Decreased no of mucous salivary gland. • Decreased lacrimal gland secretion. • Deficient hearing and vision. • Nails are brittle or deformed • Hair are sparse or there is complete absence • Tooth shows abnormal morphology. • Lack of breast development. • Saddle shaped nose.
  • 5. # • Hypohidrotic(anhidrotic)ED- (CHRIST-SIEMENS-TOURAINE SYNDROME ) It is the most common. It is usually inherited as an X-linked recessive trait. It is characterized by- Frontal bossing Sunken cheeks Thick everted lips Wrinkled , hyper pigmented skin around the eyes.
  • 6. # Dental manifestation- 1. Conical and pegged teeth. 2. Hypodontia or complete anodontia. 3. Delayed eruption of permanent teeth.
  • 7. # • Presence of eczema • Short stature • decreased flow of tears • photophobia
  • 8. # Oral manifestation • Absence or rudimentary salivary gland. Hence xerostomia is there. • Teeth shows abnormal morphology. Shape of the teeth may be truncate or conical. • High arch palate , cleft palate. Protuberant lips.
  • 9. # Hidrotic ED (Clouston syndrome) Nail dystrophy. Hair defect and palmoplantar dyskeratosis. Paronychial infection. Scalp hair is very thin, fine and brittle. Eyebrows are thinned or absent These patient have normal facies normal sweating and no specific dental defect.
  • 10. # Histological finding- There is absence of • Sweat gland • Hair follicles • Salivary gland , • Sebaceous gland • Lacrimal gland • Sparse or complete absence of hair
  • 11. # Treatment- Dental defects or abnormality can be treated by proving prosthesis such as complete denture ,RPD etc
  • 12. # Oral lichen planus (lichen rubber planus) • It is relatively common chronic dermatological disease that often effects oral mucosa. • This condition mainly affects the skin and the oral mucosa. • It causes bilateral white striation ,papules and plaque on tongue ,buccal mucosa and gingivae.
  • 13. # etiology- 1. drug induced(lichenoid drug reaction) 2. Contact allergen in restorative material or toothpaste. 3.mechinical trauma. 4.viral infection 5. in nervousness This disease seldom occur in carefree people.
  • 14. # • Grins pan's syndrome- it is an syndrome associated with oral lichen planus which is an triad of lichen planus, diabetes mellitus and vascular hypertension.
  • 15. # Clinical features- • Female to male ratio is 1.4:1 • It mainly occur in adults older than 40 years. • Skin lesions of lichen planus appears as  Small ,angular, flat topped papules only few cm to mm in diameter. These may be discrete earlier and later on coalesce to form larger plaque. These are covered by fine glistening scale. They are demarcated from skin.
  • 16. #  the lesion may appear as reddish , purple ,or violaceous hue. Later on dirty brownish color develops. Center of the papule is umblicated. The surface of the lesion is covered with very fine grayish-white lines called as Wickham's striae.
  • 17. # Location- Any where on the skin in the bilaterally symmetrical pattern most often on the- a) Flexor surface of the wrist and forearm. b) Inner aspect of knees, thighs and trunk.
  • 18. # Oral manifestation characterized by-  radiating grey or white, velvety, threadlike papules Arrangement-  In linear ,annular or retiform arrangement forming lacy ,reticular patches, rings or streak  Location-  over buccal mucosa and to a lesser extent to lips ,tongue and palate.
  • 19. #
  • 20. # • A tiny white elevated dot present at the intersection of white lines is known as striae of Wickham. • % of distribution of oral lesions- 85% -buccal mucosa 65% -tongue 20% -gingivae ,floor of the mouth and palate.
  • 21. # Types of lichen planus • Bullous form of lichen planus • Erosive formof lichen planus-  These lesions are more symptomatic. Clinically they are atrophic erythematous area with clinical ulceration of varying degree. • Atrophic form of lichen planus- Appear clinically as smooth red poorly defined area often but not always with peripheral striae.
  • 22. # • Hypertrophicform- • it may also occur on the oral mucosa, as a well circumscribed, elevated white lesion resembling leukoplakia. In this case biopsy is necessary to establish the diagnosis. • The oral manifestation of lichen planus may occur weeks or months before the appearance of the skin lesion. • Other mucous membrane affected are of penis , vagina and epiglottis.
  • 23. # • There are variety of drugs that may cause lesion similar to lichen planus they are termed as lichenoid lesion. • Causes- inflammatory drugs Sulfonylurea's  anti malarial  beta blockers ACE inhibitors. idiopathic • In rare cases it may occur after dental restoration is performed or when patient start wearing denture.
  • 24. # Histopathological finding • Typical findings are- Hyperparakeratosis or hyperorthokeratosis with thickening of granular layer Acanthosis with intercellular edema of spinous cell  “SAW tooth” shaped rete pegs. Degenerating basal keratinocytes that form colloid bodies. Formation of MAX –JOSEPH SPACES Subepithelial collection of chronic inflammatory infiltration is seen in a band like manner.
  • 25. #
  • 26. # • Differential diagnosis 1. Leukoplakia 2. Lichenoid reaction 3. Candidasis 4. Pemphigius 5. Erythema multiforme 6. Recurrant apthae 7. Lupus erythematous
  • 27. # Psoriasis • It is an non-contagious skin disorder that most commonly appear as inflamed, edematous skin lesion covered with silvery white scales • Most common type of psoriasis is Plaque psoriasis $ it is characterized by patches on scalp , trunk and limbs. • Nails may be pitted or thickened.
  • 28. # • Etiology- Cause is unknown, but do have genetic prediposition. It is an autoimmune d/s Psoriasis is associated with increased activity of T cell in underlying skin.  2.5 % persons with HIV develop psoriasis during the course of the disease  Stress can cause exacerbation of psoriasis
  • 29. # Pathogenesis-  It is due to the increased turn over rate of the dermal cells, from the normal turnover from duration of 23 days to 3-5 days in the affected area. Also increase in mitotic index of psoriatic skin
  • 30. #
  • 31. # Clinical features • Characterized by- By occurrence of small ,shapely delineated, dry papules each covered by delicate slivery scale which resembles to that of mica. If these deep dry scales are removed bleeding points are disclosed, it is an characteristic feature of psoriasis and is called as Auspitz”s sign. After the removal of scale skin is red and dusky in appearance.
  • 32. # • Cutaneous lesion are painless and seldom pruritic,they may be few in no. • Papules enlarge at the periphery and tend to elevate whereas smaller lesion coalesce to form larger plaque of irregular outline Location- 1.On the extensor surface of the extremities especially elbow ,knee, scalp , back, chest , face and abdomen. • Diseases commences with the appearance of small papules that gradually increases in size.
  • 33. # • New lesion arises over a period of time . • The disease may remain static over a period of time, thus involving more of the skin surface. • The disease is more severe in winter and less in summers due to the increased exposure to the UV rays. • Mental anxiety increases the chances of the diseases. • Arthritis is complication of psoriasis. It most frequently increases in 2 to 3 decade of life. • Psoriasis is more common in females.
  • 34. # Oral manifestation • Lesions are mainly reported on lips , buccal mucosa, palate, gingiva and floor of the mouth. • These lesions are clinically described as gray or yellowish-white plaque, as silvery white scaly lesion with erythematous base.
  • 35. # Histological features 1. Uniform parakeratosis 2. Absence of stratum granulosum 3. Elongated and clubbing of rete pegs 4. Epithelium over the connective tissue is thinned and from these points bleeding occurs when the scales are peeled. 5. .Monor’s abscesses are common. 6. mild lymphocytic and histiocytic infiltration of connective tissue is common
  • 36. # Treatment- • It include UV-A light. • Psoralen plus UV-A light . • Retinoid. • Methotrexate ,cyclosporine.
  • 37. # • It is an ulcerative mucocutaneous disease of uncertain pathogenesis. • In erythema multiforme there is mild skin eruption and mucosal involvement whereas in erythema multiforme and in Stevens Johnson's syndrome there is severe skin and mucosal involvement. Erythema multiforme (Stevens-Jhonsons syndrome)
  • 38. # • Differentiation b/w Stevens Johnson's syndrome can only be made by there lesions • In EM the lesions is raised atypical target and mucosal erosion • Whereas in SJS there is mucosal erosion with flat atypical target cells
  • 39. # • Etiology- 1.It is caused by drugs such as sulpha drugs. 2.herpes simplex virus. 3.mycoplasm infection. Clinical feature- • It mainly occur in adults. • It may occur at any age. • It is most common in 2 and 3 decade. • It is more common in males than females.
  • 40. # • It is characterized by- Occurrence of asymptomatic,vividly erythematous discrete macules,papule,vesicle ,or bullae in symmetrical pattern over hand arms feet legs face and neck. Lesion may vary from few cm to mm in diameter.
  • 41. # A concentric ring like appearance of lesion -resulting from varying shade of erythema and this gives rise to the term target , iris or bull’s eye. They are most common on hands, wrist , and ankles. Mucous membrane involvement, including the oral cavity is common. Lesions appears within few days or two and persist for several days to few weeks and gradually disappears.
  • 42. # Oral mucous membrane- •Lesions are usually not so significant except the pain and the discomfort it may cause. •Hyperemic papule ,macules ,or vesicles may erode and bleed profusely. •Tongue , palate , gingiva are commonly involved
  • 43. # Stevens Johnsons syndrome- • it is an severe form of erythema multiforme with widespread involvement of skin , oral cavity , eye and genital. Oral mucous membrane lesion- 1.difficulty in chewing and mastication 2.severe bleeding and crusting of the lips
  • 44. # Eye lesion- • Conjunctivitis • Corneal ulceration. • Keratoconjunctivitis • Blindness may occur. Genital lesions- • Urethritis • Vaginal ulcers. • Balanitis.
  • 45. # Histological features • Cutaneous and mucosal lesion usually exhibit intercellular edema. • Edema of superficial CT tissue. • Zone of severe liquefaction degeneration I the upper layer of the epithelium. • Interepithelium vesicle formation and thinning or absence of basement membrane. • Dilatation of superficial capilaries. • Varying degree of inflammatory cell infiltrate.
  • 46. # Differential diagnosis- 1. Apthous stomatitis. 2. Contact dermatitis. 3. Stomatitis. 4. Acute necrotizing gingivitis. 5. Pemphigus 6. Dermatitis 7. Bullous lichen planus. 8. Herpes zoster. 9. Chicken pox. 10.Toxic epidermal necrolysis.
  • 47. # Toxic epidermal necrolysis • It is an serious, often fatal ,Bullous drug eruption. • It is so severe that large sheet of skin peel off, giving the appearance of widespread scalding burn. • It is considered form of Stevens Johnson's syndrome.
  • 48. # • Toxic epidermal necrolysis must be differentiated from Staphylococcal scalded skin syndrome, which appear clinically similar even though the latter is milder diseases with a better prognosis.
  • 49. # • Treatment- • If the drug is suspected withdraw the drug. • Infection should be treated after culture. • Oral antihistamines. • Analgesics. • Local skin care. • Soothing mouth wash. • Topical steroid. • oral antacids are helpful in oral ulcers, • liquid antiseptics such as 0.05 chlorhexidine during bathing. • systemic corticosteroids.
  • 50. # Mucocutaneous lymph node syndrome(kawasaki disease) • It is an acute self limiting febrile illness. • Mucocutaneous lymph node syndrome , Kawasaki syndrome or KD , is an systemic vasculitis of unknown etiology. • Hallmark of KD are- Fever of unknown origin for more than five days.  Generalized erythema and desquamation of skin.  cervical nonsuppurative
  • 51. #  Lymphadenopathy. Swelling of hand and feet. Etiology- 1.Abnormal inflammatory response triggered by a neoantigen. 2.Epstein-barr virus. 3.Retrovirus. 4.Streptococcus species. 5.Streptococcus viridians. 6.Chlamydia and pseudomonas species.
  • 52. # Clinical features- • Majority of cases occur b/w three month and 12 yrs of age. • KD more often occur in boys than girls, ratio of about 1.4:1 • SYMPTOMES- Fever for 5 or more days, with no response to antibiotics. Bilat. Congestion of ocular conjunctivitis. Reddening of oral and pharyngeal mucosa.
  • 53. #  Changes in lip and mouth including dryness , redness, and swelling of tongue papillae.  In durative edema and erythema of palms and soles.  Polymorphous exanthema of torso.  Acute , non purulent swelling of cervical lymph node.
  • 54. # • Differential diagnosis- • No lab test is present for the diagnosis of the diseases. • So diagnosis is based on clinical manifestation. • It should be distinguished from- 1.Scarlet fever. 2.Erythema multiforme or Stevens- johnson syndrome.
  • 55. # treatment- • Reduction of the fever. And inflammation. • Intravenous administration of gamma globulin.
  • 56. # Keratosis follicularis (Darier diseases • Also known as Darier-White disease, or Keratosis follicularis, Darier's disease. • It is a rare inherited disease of keratinization characterized by scaly, crusted papules typically occurring on seborrheic areas. Mucous membrane and nail changes also occur.
  • 57. # • ETIOLOGY & PATHOGENESIS- • Darier's disease is inherited as an autosomal dominant trait with high penetrance. • The Darier's disease gene is located on chromosome 12 and encodes a adenosine triphosphatase calcium pump (SERCA 2). • Mutations of the gene results in abnormality in epidermal cell-cell adhesion and premature and faulty keratinization leading to intraepidermal clefts and dyskeratosis cells. • Precipitating factors include heat and humidity, mechanical trauma like friction, sunlight, and secondary bacterial infections.
  • 58. # CLINICAL FEATURES- • Darier's disease occurs in all races. • Both sexes are equally affected. • The onset of disease is during the first or second decade. • Initial lesions are skin colored papules that develop scales or crust on its surface which, on removal may reveal a depression. The lesions gradually darken to take a brownish or blackish hue.
  • 59. # • The discrete, warty papules may coalesce to become crusted plaques. On intertriginous areas, vegetative lesions may become malodorous. • The distribution is typically on the seborrheic areas: chest, back, back of ear, forehead, scalp, neck, nasolabial folds, groin, and axillae. In extensive disease, any area of the body may be involved. The affection is symmetrical. • The scalp may show greasy scales. • Localized, unilateral disease along Blaschko's lines may occur due to somatic mutation.
  • 60. # • Oral mucosa: whitish papules with central umbilications on gingiva, palate. Pebbly white plaques (cobble stoning) may also be found. Involvement of oropharynx, trachea and esophagus has also been described. • Nails: Thinning of plate, hyperkeratosis, longitudinal ridging, alternate, longitudinal white and red bands. V-shaped, triangular scalloping of distal nail plate is characteristic.
  • 61. # DIFFERENTIAL DIAGNOSIS- • Seborrheic dermatitis • Hailey-Hailey disease • Pemphigius vegetans • Pemphigius foliaceus • Grover's disease (transient acantholytic dermatosis) • Localized form: epidermal nevus • Histologic D/D: Epidermal nevus, warty dyskeratoma, actinic Keratosis •
  • 62. # DIAGNOSIS- • Family history of similar affection in parents/siblings. • Typical clinical features of dirty, Keratosis papules at sites of predilection • Histology- • Hyperkeratosis, parakeratosis, Acanthosis Suprabasal intraepidermal clefts Acantholysis Dyskeratosis cells in spinous layer ( corps ronds) and keratin layer (grains)
  • 63. # COURSE AND PROGNOSIS- • Darier's disease runs a life-long course. • Seasonal variations in severity occurs. TREATMENT- • Avoidance of frictions/mechanical irritations. • Use of sunscreens. • Topical retinoid: tazarotene, adapalene • Systemic retinoid: isotretinoin or acitretin may induce temporary remission.
  • 64. # • Systemic retinoid: isotretinoin or acitretin may induce temporary remission. • Topical and systemic antibiotics to treat secondary bacterial infections. • Hypertrophic lesions may be treated with dermabrasion, excision and grafting, or laser excision.
  • 65. # White sponge nevus familial white folded dysplasia of mucous mem, cannons disease, white folded gingivostomtitis • It is an uncommon condition of oral mucosa. • Clinical features- • This mucosal abnormality is congenital in many instances. • It does not appear until infancy, childhood, or even adolescence, by this time it reaches its full extent.
  • 66. # Oral lesions- The lesions involve the • cheek , • palate, • gingiva, • floor of the mouth • and portions of the mouth.  The mucosa appear thickened and folded or corrugated with a soft or spongy texture and a peculiar white opalescent hue.
  • 67. # • Sometimes a minimal amount of folding is present. • Ragged white area can be scrapped out without ensuing bleeding. Histological features- • Epithelium is thickened. • Hyperkeratosis and Acanthosis. • Basal layer is intact. • Intercellular edema.
  • 68. # • Vacuolated cells may show pyknotic nuclei. • Para keratin plugs running deep into the spinous layer. • Sub mucosa may show mild inflammation. • Treatment and prognosis- • There is no t/t. • Its benign, the prognosis is excellent. • Has no complication. The microscopic features of white sponge nevus are distinctive. There are two characteristic cell types: dense keratinized cells and pale vacuolated ones. There is no evidence of dysplasia or anaplasia in this condition
  • 69. # Pemphigus • It is an serious chronic disease characterized by the appearance of vesicles and bullae with small or large fluid filled blisters that develop in cycles. • Pemphigus includes a group of autoimmune blistering disease of the skin and the mucous mem. • Characterized histologically by intradermal blisters and immunologically by the finding of the circulating IgG antibodies.
  • 70. # – There are primary three types of Pemphigus:- 1. Pemphigus vulgaris (PV) 2. Pemphigus foliaceus 3. Pemphigus paraneoplastic. • Pemphigus vulgaris accounts for approx 70% of Pemphigus.
  • 71. # Pemphigus vulgaris • Pemphigus vulgaris is an auto immune disease of the skin and the mucous mem. • Blister in PV is associated with the binding of IgG auto antibodies to keratinocytes cell surface. • Pemphigus antibodies bind to keratinocytes cell surface molecule desmoglein 1 and 3.
  • 72. # Clinical features- • Pemphigus vulgaris affects all races, with equal gender distribution among males and females. • Mean age of onset is approx 50- 60yrs. • It is characterized by rapid appearance of vesicles and bullae, varying in dia. from few cm to mm , in such large skin surface is covered • Lesions are fluid filled soon after development and soon become.
  • 73. # -purulent, and soon the skin become eroded when the bullae rupture. • The characteristic feature of Pemphigus is NIKOLSKY’S SIGNS, in which there is loss of epithelium by rubbing the unaffected skin. • It is caused by the peri vesicular edema which disrupts the dermal epidermal junction.
  • 74. # • Nikolsky's sign is a condition caused by a staphylococcal infection • In which the superficial layers of skin slip free from the lower layers with a slight rubbing pressure. Large areas of the skin will blister and peel away leaving wet, red and painful areas
  • 75. # • Nikolsky's sign is either positive or negative. A positive result may be present in several different medical conditions. Persons with a positive sign have loose skin that slips free from the underlying layers when rubbed. The area beneath is pink and moist and may be very tender. • Typically, your health care provider will test for this sign simply by twisting a pencil eraser against your skin. If positive, a blister will form in the area, usually within minutes
  • 76. # • Pemphigus vegetans is an uncommon variant of the Pemphigus vulgaris. • It occur in 1-2% of the case of the PV. • The median age of onset of the Pemphigus vegetans is 40-50yrs. • Its two subtypes are- 1. Neumann (flaccid bullae and erosions) 2. Pustules(Hallopeau) • Both the subtypes develops into hyper pigmented vegetative plaque with pustules and hypertrophic granulation at the periphery. • Its characteristic feature is the cerebriform tongue ,characterized by the presence of sulci and gyri on the dorsum of the tongue.
  • 77. # Oral manifestation- • Mucous mem is typically affected in PV. The disease typically the mucous mem in 50-70%. • Patients typically have ill defined, irregularly shaped gingival buccal or palatine erosions which are painful and slow to heal. • The erosion extend peripherally with the shedding of the epithelium. • Erosion may be seen on any part of the oral cavity.
  • 78. # • Erosions may spread to involve the larynx with subsequent hoarseness. • The patient is often has difficulty in eating and drinking because of the lesion. • Other mucosal surface involve conjunctiva, esophagus, labia , vagina , cervix, penis , urethra and anus.
  • 79. # Histological finding- • Pemphigus is characterized by the presence of the formation of vesicle or bullae entirely intraepithelially and just above the basal layer producing the distinctive “suprabasilar split” •Suprabasal separation of epithelium •Intraepithelial vesicle
  • 80. # • Perivascular edema appears to weaken this junction, and the intercellular bridges between the epithelium cells disappear. • This results in the loss of cohesiveness or Acantholysis Acantholyis
  • 81. # • The clumps of the epithelial cells are found lying free with in the vesicular space. these have been called as “Tzank cells” and are characterized particularly by degenerative changes which include swelling of the nuclei and hyper chromatic staining. Acantholysis - Tzank cells (epithelial cells which are smaller, rounder)
  • 82. # • The fluid in most vesicle ,particularly those more than a day or two old, Contains- • Polymorhonuclear leukocytes • Lymphocytes • Scarce inflammatory cell infiltrate, in both the vesicular fluid and in the connective tissue at the base of the vesicles or bullae is suggestive of Pemphigus.
  • 83. # • Immunoflurescent test has proven to be of great value in estb the diagnosis of Pemphigus. • Direct immunofluorescence- Is used to demonstrate the presence of immunoglobuluns,pred ominantly IgG, and sometimes C3, IgA and IgM. Direct immunofluorescence: IgG deposition in intercellular areas
  • 84. # • A biopsy of a skin sample is treated in the laboratory with a chemical compound to find the abnormal desmoglein antibodies that attack the skin. The specific type of antibodies that form may indicate what type of Pemphigus exists. • Indirect immunofluorescence-. • Sometimes called an antibody titer test, a sample of blood is tested to measure Pemphigus antibody levels in the blood and help determine the severity of the disease. Once treatment begins, this blood test may also be used to find out if treatment is working
  • 85. # • Indirect immunofluorescence has also been highly beneficial. this is accomplished by incubating normal animal or human mucosa with serum from the patient suspected of having the diseases and adding fluorescein conjugated human antiglobulin. • Positive reaction indicates presence of circulating immunoglobulin antibodies. Direct immunofluorescence: IgG deposition in intercellular areas
  • 86. # • Differential diagnosis- 1. Dermatitis herpetiformis. 2. Erythema multiforme bullosum 3. Bullous lichen plan us 4. Epidermolysis bullosa 5. Bullous pemphigoid 6. Cicatricial pemphigoid.
  • 87. # • Treatment- • T/t for Pemphigus vulgaris involves using one or more drugs. • High-dose oral corticosteroids, such as prednisone or prednisolone, are the main treatment for Pemphigus. These are anti-inflammatory medicines that suppress the immune system. • High doses are often required to bring Pemphigus under control. To minimize the side effects patients may experience, once the disease begins to subside the corticosteroid levels are reduced slowly to the lowest level required to prevent new blisters or sores from appearing.
  • 88. # • Many patients will go into complete remission with treatment, although this may take a number of years. Other patients will need to continue to take small doses of medication to keep the disease under control • Prednisone is usually taken by mouth, but can also be injected into a vein, muscle, or directly into a blister. The route depends on the type and severity of disease. Usually, a corticosteroid cream will be used directly on the blisters.
  • 89. # • To keep the levels of corticosteroid use to a minimum, immunosuppressive drugs are often added to a patient’s treatment. These are drugs that stop or slow down the immune system’s response to what it sees as an attack on the body. They include: • Myco-phenolate mofetil (CellCept) • Azathioprine (Imuran) • Cyclophosphamide (Cytoxan) • Methotrexate. • Other drugs that may be used include: • Dapsone (DDS) • Antibiotics such as tetracycline.
  • 90. # Pemphigus foliaceus • Pemphigus foliaceus is a benign condition of pemphigus. • It is an autoimmune skin disorder characterized by the loss of intercellular adhesion of keratinocytes in the upper part of the epidermis(acantholysis),resulting in the formation of superficial blister. • It is characterized by the involvement of healthy appearing skin that blisters when rubbed(Nikolsky’s sign).
  • 91. # • PF is characterized by a chronic course, with little or no involvement of mucous mem. • Superficial blisters are induced by IgG autoantibobies directed against a cell adhesion molecule ,desmoglein 1
  • 92. # • Subtypes of pemphigus foliaceus are- 1. Pemphigus erythematosus. 2. Pemphigus herpetiformis. 3. Endemic pemphigus foliaceus 4. Immunoglobulin pemphigus foliaceus. 5. Drug induced pemphigus foliaceus. • Clinical features- • PF is characterized by early bullous lesions which rapidly rupture and dry to leave masses of flakes or scales suggestive of an exfoliative dermatitis or eczema. • It is a mild form of pemphigus and common in older adults.
  • 93. # • Histological finding- • Acantholysis of upper epidermis. • It usually detaches without bullae formation. more estb lesions have acanthosis and mild to moderate papillomatosis. • Hyperkeratosis and parakeratosis, with dyskeratosis cell within the granular layer
  • 94. # Treatment- • Therapy for PF is usually less aggressive that of PV ,because of low mortality and morbidity rate. • Non steroidal t/t is necessary. • Mestinon is used to slow down the progression of the disease.
  • 95. # Paraneoplastic pemphigus (PNP) demonstrating an erosive cheilitis. This is a frequent feature in PNP and often responds very poorly to treatment. Paraneoplastic pemphigus is the most fatal type of the pemphigus. It may even cause death due to the multi organ failure and respiratory failure, thus causing direct effect on the respiratory epithelium. The of diagnosis of paraneoplastic pemphigus involves- Paraneoplastic pemphigus 1. Painful mucosal erosion , sometimes with a skin eruption that results in blister and erosions. 2. Histopathological changes of acantholysis ,keratinocytes , necrosis and dermatitis. 3. Direct immunofluorescence revels IgG and complement C3 .
  • 96. # 4. Indirect immunofluorescence revels circulating antibodies specific for stratified squamous epithelium. Etiology- • Tumor antigen are believed to evoke an immune response that led to the development of autoimmune response to intercellular adhesins. • Autoantibobies leads to blistering in the mucosa and other epithelium.
  • 97. # Clinical features- • Mean age of onset is 60 yrs. • Male and females are equally affected. • All the patient with paraneoplastic pemphigus have tumor, most of which are malignant. • Most common malignancy associated are- 1. Non Hodgkin's lymphoma 2. Chronic lymphocytic leukemia. 3. Castel man tumor 4. Giant cell lymphoma. 5. Macroglobulinemia. 6. thymoma,. 7. Poorly differentiated sarcoma. 8. Bronchogenic squamous cell carcinoma.
  • 98. # Oral manifestation- • Patient has oral ulceration and erosions. • Patient with oral erosions often has cutaneous eruptions. • Erosions has wide variety of patterns. • Erosions can occur anywhere in the mouth. • Erosions and subsequent crusting of the vermillion border of the lips are typical and is similar to Stevens Johnson's syndrome.
  • 99. # Histological finding- • Vesicular lesions express the most characteristic histopathologic features. Oral and cutaneous lesions show variable epidermal necrosis, suprabasal acantholysis, dyskeratotic keratinocytes, vacuolar interface dermatitis, and lymphocytic exocytosis. Substantial inflammation can be present, even in early lesions • Oral mucosal lesions show the greatest acantholysis, while some skin lesions may not have any acantholysis. • When present, suprabasal acantholysis can result in clefts and tomb stoning, which is the appearance of the basal cell layer below the cleft, and it can be indistinguishable from pemphigus vulgaris.
  • 100. # • A distinctive feature of paraneoplastic pemphigus is dyskeratosis. • The presence of dyskeratosis in a person with a suprabasal acantholytic bullous disorder is a clue to the presence of paraneoplastic pemphigus. • Interface dermatitis is a frequent finding in persons with paraneoplastic pemphigus, and it can be found both with and without acantholysis. • Exocytosis of inflammatory cells into the epidermis is common. • Dermal changes include a superficial perivascular infiltrate of variable intensity, which is mostly composed of lymphocytes.
  • 101. # • The inflammation can sometimes be lichenoid, leading to a misdiagnosis of lichen planus. Papillary dermal edema is present in early lesions, whereas older lesions may exhibit mild dermal fibrosis. Treatment- 1. Warm compresses. 2. Non adherent dressing. 3. Topical antibiotics ointments. 4. Potent immunosuppressive. In general- skin lesions are more responsive than that of mucosal leions.
  • 102. # • Other therapeutic options include- plasmapheresis immunophoresis. • For solid neoplasm- curative resection. • The prognosis for paraneoplastic pemphigus is poor.
  • 103. # Familial benign pemphigus • Familial benign pemphigus originally was described by the Hailey brothers in 1939.1 It is a chronic autosomal dominant disorder with incomplete penetrance. Approximately two thirds of patients have a family history of the disorder. A history of multiple relapses and remissions is characteristic. Decreased numbers of desmosomes have been implicated in the pathogenesis of benign familial pemphigus. Therapeutic options are limited
  • 104. # Clinical features- • The disease first manifest itself during adolescence or young adult life. • There is no predilection in either gender. • Lesions develops as small vesicles appearing on normal or erythematous skin, which soon rupture to leave eroded skin. • Lesion appear too enlarge on periphery and heal at center. • Heat and sweating increases its outbreak, spontaneous remission occur in cold weather. • Lesion develop in the area of excessive friction.
  • 105. # • Tender and enlarged lymph node are also present. • It is been seen that bacterial infection ppt the appearance of the lesion, mostly by Candida albicans. Oral manifestation- • Oral lesions rarely occur in such patients. • The lesions develop as crop of vesicles and rupture rapidly leaving raw eroded area. histological features- • It bears close resemblance to PV, Keratosis follicularis and Darier's disease. • It as extensive acantholysis than PV. • less damage to acantholytic cells.
  • 106. # • Characteristic feature of this disease is that intercellular bridges persist so that adjacent epithelial cell still adhere to each other and are not entirely acantholytic. • This appearance is called as “dilapidated brick wall "effect. • Treatment- 1. Soothing compress followed by mild use of corticosteroid prep 2. topical antibiotics. 3. Erythromycin and tetracycline. 4. Bacterial culture and sensitivity test to help guide appropriate therapy.
  • 107. # Cicatricial pemphigoid (benign mucous membrane pemphigoid, ocular pemphigus) • Cicatricial pemphigoid is an auto immune blistering disease that affects the mucous mem including the mouth , oropharynx , conjunctiva ,nares and the genitalia. • Patient with cutaneous involvement present with tense blister and erosion, often on head and neck. Cicatricial pemphigoid may affect skin as well as the mucous membranes, usually on the head and neck (termed the Brunsting Perry type).
  • 108. # Etiology- • Cicatricial pemphigoid (CP) is due to antibodies that target several potential antigens. Clinical features- • the disease occur twice as frequently in females as that in males • Peak age of involvement is 40-50yrs. • Vesicobullous lesion occur on the oral mucous mem and conjunctiva. • Lesion also occur on skin particularly around genitalia. • Lesion heal by scar formation, particularly around conjunctiva.
  • 109. # • Other mucous mem surfaces to be involved are nose , larynx , pharynx , esophagus. • Ocular involvement is the most serious complication. following initial conjunctivitis, adhesion develops between the palpebral and bulbar conjunctiva resulting in obliteration of palpebral fissure c/a adhesions symblephoron formation-closes of the opening of lachrymal gland –blindness and scar formation. • with opacity and thus complete blindness
  • 110. # Oral manifestation- • Gingivitis in a patient with Cicatricial pemphigoid. This pattern of periodontal, chronic, red, velvety gingivitis is very suggestive of this disorder but may occur in other blistering diseases. • the mucosal lesion are vesiculobullous in nature but appear to be relatively thick walled so that it persist for 24-48hr before rupturing. • Ruptured surface do not leave raw eroded bleeding surface.
  • 111. # • Cicatricial pemphigoid affecting the tongue. The lesions are rather annular, and may be mistaken for migratory glossitis (geographic tongue)unless there are other associated features. • Involvement of the palate in Cicatricial pemphigoid, manifest as purpuric spots. Intraoral lesions of dermatitis herpetiformis may also produce similar purpuric lesions..
  • 112. # Histological finding- • In the histological finding of the CP , the vesicles and bullae are sub epidermal rather than suprabasilar and there is no evidence of acantholysis. • The basement structure appear to detach with the epithelium from underlying CT. • There is chronic inflammatory cell infiltrate in CT , chiefly lymphocytes ,plasma cells and eosinophils. • Immunofluorescence studies reveled the presence of tissue bound basement mem zone antibodies,as well as circulating antibasement mem zone antibodies in serum
  • 113. # • Immunofluorescence pattern of CP was indistinguishable from the pattern observed in bullous pemphigoid. Differential diagnosis- 1. Pemphigus vulgaris 2. Bullous pemphigoid. 3. Erosive lichen planus. 4. Bullous erythema multiforme. Treatment- • The goal of treatment is to suppress the blister formation and to promote the healing and to prevent scarring.
  • 114. # • The lowest dose of medication to suppress disease activity and to minimize risk. • It has severe complication that causes visual loss or blindness , airways stenosis , esophageal stricture, cutaneous blistering and milia formation.
  • 115. # Bullous pemphigoid • BP is a chronic , autoimmune ,sub epidermal , blistering skin disease that rarely involves mucous mem. • It is characterized by the presence of IgG auto antibodies.
  • 116. # What Is Pemphigoid, and How Is It Different From Pemphigus? • Pemphigoid is also a blistering disorder caused by autoimmune problems that result in an attack on the skin cells by a person’s own antibodies. Pemphigoid produces a split in the cells where the epidermis and dermis, the layer below the epidermis, meet, causing deep, tense blisters that do not break easily. Pemphigus, on the other hand, causes a separation within the epidermis, and the blisters are soft, limp, and easily broken. Pemphigoid is seen most often in the elderly and may be fatal. Usually, both pemphigus and pemphigoid are treated with similar medications. Severe cases may require different treatment
  • 117. # COMPARISON BETWEEN BULLOUS PEMPHIGOID AND PEMPHIGUS VULGARIS Characteristic Bullous pemphigoid Pemphigus Frequency Uncommon Rare Age group Most > 70 years Often 50-60 years Blister morphology Tense, clear fluid content May be pompholyx changes on palms or soles Fragile, may just be blister remnants at the margin of erosions Adjacent skin May be urticated or eczematous Normal, but may be able to induce blister by shearing force (Nikolsky's sign) Mucous membrane involvement About 25%, usually minor Present in 80%, typically precedes skin involvement by several months Direct immunofluorescence Positive at basement membrane zone Positive epidermal intercellular Response to treatment Usually good Often refractory
  • 118. # Pathogenesis- • IgG autoantibodies • Skin basement membrane • Complement and inflammatory mediators • Inflammatory cells to the basement mem. • Proteases which degrades hemidesmosomal proteins. leads to blister formation Bind to activates attracts release
  • 119. # Clinical features- • It is basically a disease of elderly people. • Mainly occur at the age of 60 yrs to above. • It can even occur earlier I life. • There is no gender predilection. Cutaneous lesions- • Generalized nonspecific rash, commonly on limbs, which appears urticarial or eczematous and may persist for several weeks or months before its ultimate appearance
  • 120. # • Vesicles and bullae are relatively thick walled and may remain intact for some days. • Rupture does not always occur, although when it occur it leaves raw eroded area which heals rapidly. Oral manifestation- • Frequency of oral lesion are very less. • Oral lesion BP are described as vesicles and area of erosions and ulceration. • Its important feature is its similarity to the gingiva lesions to those of the Cicatricial pemphigoid. • Gingival lesions involve the gingival mucosa and it is extremely painful.
  • 121. # • The gingival tissue appear extremely erythematous and may even desquamate as a result of minor friction. • Hence vesicles form and erosion may occur. • Lesion may also occur on buccal mucosa , palate, floor of the mouth and tongue. Histological features- • Vesicle and bullae in it are sub epidermal and non specific. • There is no evidence of acantholysis of epithelial ; in fact the epithelium is normal. • Vesicles contain fibrinous exudates admixed with inflammatory cells
  • 122. # • Electron microscope studies revel- basement mem remain attached to the CT • Ultra structural studies revel-  primary changes in bullous pemphigoid appear to occur in the CT where the blood vessels shows alteration in their penetrability. Basement mem shows thickening with interruption in continuity. • Indirect immunofluorescence studies revel- circulating basement mem zone antibodies are found. • Direct immunofluorescence studies revel- tissue bound antibasement mem zone antibodies
  • 123. # Treatment- 1. To decrease blister formation. 2. To promote healing of blisters and erosions. 3. Therapy must be individualized for each patient. 4. Treatment for most of the patients require 6- 60 months.
  • 124. # Systemic lupus erythematosus • Systemic lupus erythematosus is an autoimmune disease characterized by the autoantibodies, immune complex formation, and immune dysregulation resulting in damage to essentially any organ, including kidney, skin, blood cells and the CNS.
  • 125. # Etiology- • The precise reason for the abnormal autoimmunity that causes lupus is not known. • Inherited genes, viruses, ultraviolet light, and drugs may all play some role. • Genetic factors increase the tendency of developing autoimmune diseases, and autoimmune diseases such as lupus, rheumatoid arthritis , • disorders are more common among relatives of patients with lupus than the general population. • immune system in lupus is more easily stimulated by external factors like viruses or ultraviolet light.
  • 126. # • Sometimes, symptoms of lupus can be precipitated or aggravated by only a brief period of sun exposure. • Dozens of medications have been reported to trigger SLE; however, more than 90% of this "drug-induced lupus" occurs as a side effect of one of the following six drugs: • hydralazine (used for high blood pressure), • quinidine and procainamide (used for abnormal heart rhythm), • phenytoin (used for epilepsy), • isoniazide (used for tuberculosis), • d- penicillamine (used for rheumatoid arthritis). .
  • 127. # • These drugs are known to stimulate the immune system and cause SLE. Fortunately, drug-induced SLE is infrequent (accounting for less than 5% of SLE among all patients with SLE) and usually resolves when the medications are discontinued. • It also is known that some women with SLE can experience worsening of their symptoms prior to their menstrual periods. This phenomenon, together with the female predominance of SLE, suggest that female hormones play an important role in the expression of SLE. This hormonal relationship is an active area of ongoing study by scientists.
  • 128. # Clinical features- • It is an serious cutaneous systemic disorder • Disease is at its peak at the age of 30 yrs in female and 40 yrs in male. • Prevalence rate is higher in female than males. • Female to male ratio is 2:1 before puberty and after puberty the ratio is 4 :1 • Patients with SLE can develop different combinations of symptoms and organ involvement. • Common complaints and symptoms include • fatigue, • low-grade fever,
  • 129. # • loss of appetite, • muscle aches, • arthritis, • ulcers of the mouth and nose, facial rash ("butterfly rash"), • unusual sensitivity to sunlight (photosensitivity), • inflammation of the lining that surrounds the lung (pleuritis) and the heart (pericarditis), and • poor circulation to the fingers and toes with cold exposure (Raynaud's phenomenon).
  • 130. # • Most patients with SLE will develop arthritis during the course of their illness. Arthritis in SLE commonly involves swelling, pain, stiffness, and even deformity of the small joints of the hands, wrists, and feet. Sometimes, the arthritis of SLE can mimic that of rheumatoid arthritis (another autoimmune disease).
  • 131. # • Inflammation of the lining of the lungs (pleuritis) and of the heart (pericarditis) can cause sharp chest pain. The chest pain is aggravated by coughing, deep breathing, and certain changes in body position. The heart muscle itself rarely can become inflamed (carditis). It has also been shown that young women with SLE have a significantly increased risk of heart attacks from coronary artery disease. • Kidney inflammation in SLE can cause leakage of protein into the urine, fluid retention, high blood pressure, and even kidney failure
  • 132. # • Involvement of the brain can cause personality changes, thought disorders (psychosis), seizures, and even coma. Damage to nerves can cause numbness, tingling, and weakness of the involved body parts or extremities. Brain involvement is called cerebritis. • Many patients with SLE experience hair loss (alopecia). Often, this occurs simultaneously with an increase in the activity of their disease.
  • 133. # Q- How is lupus diagnosed? • Since patients with SLE can have a wide variety of symptoms and different combinations of organ involvement, no single test establishes the diagnosis of systemic lupus. • To help doctors improve the accuracy of the diagnosis of SLE, eleven criteria were established by the American Rheumatism Association.
  • 134. # • These eleven criteria are closely related to the symptoms. Some patients suspected of having SLE may never develop enough criteria for a definite diagnosis. Other patients accumulate enough criteria only after months or years of observation. When a person has four or more of these criteria, the diagnosis of SLE is strongly suggested
  • 135. # • The eleven criteria used for diagnosing systemic lupus erythematosus are: 1.malar (over the cheeks of the face) "butterfly" rash. 2.Discoid skin rash: patchy redness that can cause scarring 3.Photosensitivity: skin rash in reaction to sunlight exposure 4.Mucus membrane ulcers: ulcers of the lining of the mouth, nose or throat 5.Arthritis: 2 or more swollen, tender joints of the extremities
  • 136. # 6.Pleuritis/pericarditis: inflammation of the lining tissue around the heart or lungs, usually associated with chest pain with breathing 7.Kidney abnormalities: abnormal amounts of urine protein or clumps of cellular elements called casts 8.Brain irritation: manifested by seizures (convulsions) and/or psychosis 9.Blood count abnormalities: low counts of white or red blood cells, or platelets 10.Immunologic disorder: abnormal immune tests include anti-DNA or anti-Sm (Smith) antibodies, falsely positive blood test for syphilis, anticardiolipin antibodies, lupus anticoagulant, or positive LE prep test
  • 137. # • 11.Antinuclear antibody: positive ANA antibody testing. Other tests can be helpful in evaluating patients with SLE to determine the severity of organ involvement. These include- 1. routine testing of the blood to detect inflammation (for example, a test called the sedimentation rate), 2. blood chemistry testing, 3.direct analysis of internal body fluids, 4.and tissue biopsies. Abnormalities in body fluids and tissue samples (kidney, skin, and nerve biopsies) can further support the diagnosis of SLE.
  • 138. # Oral manifestation- • Oral mucous mem involvement is reported in 20- 50 % cases of discoid lupus erythematosus and slightly more frequently in systemic form of the disease. • Oral lesion are formed either prior to or following the development of skin lesions or even in the absence of skin lesion. • Oral lesion in SLE are very similar to that to DLE except that hyperemia, edema and extension of the lesion is more pronounced and there may be more tendency towards bleeding, petechiae and superficial ulceration which is surrounded by red halo as a result of superficial telengiectasis.
  • 139. # Histological finding- • Histological appearance of SLE and DLE is similar, only differs in in the degree of certain findings.
  • 140. # Laboratory finding- • A specific test have been estb for its diagnosis- the test consist of addition of blood serum from a person under suspicion to the buffy coat of normal blood. if the patient is suffering from SLE ,typical LE cells will develop. this cell or phenomena consist of a rosette of neutrophils surrounding a pale nuclear mass apparently derived from a lymphocytes. • There is also anemia ,leukoplakia thrombocytopenia and elevated sedimentation rate as well as elevated serum gamma globulin level and a positive Coombs test, in a significant % of people with either SLE or DLE.
  • 141. # • Antinuclear antibodies can be demonstrated in patient of SLE and DLE. Treatment- • The most imp tool is careful and frequent clinical and laboratory evaluation to tailor medical regime. • There is no permanent cure for SLE. The goal of treatment is to relieve symptoms and protect organs by decreasing inflammation. • Many patients with mild symptoms may need no treatment or only intermittent courses of anti- inflammatory medications.
  • 142. # • Those with more serious illness involving damage to internal organ(s) may require high doses of corticosteroids in combination with other medications that suppress the body's immune system. • Patients with SLE need more rest during periods of active disease. Researchers have reported that poor sleep quality was a significant factor in developing fatigue in patients with SLE • Nonsteroidal anti-inflammatory drugs (NSAIDs) are helpful in reducing inflammation and pain in muscles, joints, and other tissues. Examples of NSAIDs include aspirin, ibuprofen (Motrin), naproxen (Naprosyn), and sulindac (Clinoril). S
  • 143. # Discoid lupus erythematosus • It is an chronic scarring atrophy producing photosensitive dermatosis. • Discoid lupus erythematosus (DLE) is a disease in which coin-shaped (discoid) red bumps appear on the skin. • The disease called discoid lupus erythematosus only affects the skin, although similar discoid skin lesions can occur in the serious disease called systemic lupus erythematosus (SLE)..
  • 144. # • The tendency to develop DLE seems to run in families. Although men or women of any age can develop DLE, it occurs in women three times more frequently than in men. The typical DLE patient is a woman in her 30s.
  • 145. # Etiology- • It mainly occur in genetically predisposed individual. • It has been sugested that heat shock protein is induced in the keratinocytes following ultraviolet light exposure and stress and the protein may act as a target for T-cell. Clinical features- • In DLE, the characteristic skin lesion is circular and raised.
  • 146. # • The reddish rash is about 5-10 mm in diameter, with the center often somewhat scaly and lighter in color than the darker outer ring. The surface of these lesions is sometimes described as "warty.“ • There is rarely any itching or pain associated with discoid lesions. • They tend to appear on the face, ears, neck, scalp, chest, back, and arms. • DLE lesions heal, they leave thickened, scarred areas of skin. When the scalp is severely affected, there may be associated hair loss (alopecia).
  • 147. # • People with DLE tend to be quite sensitive to the sun. They are more likely to get a sunburn, and the sun is likely to worsen their discoid lesions. • The typical cutaneous lesion are slightly elevated red or purple macules that often covered with gray or yellow adherent scales. forceful removal of scales revel numerous ‘carpet tack’ extensions which is dipped into enlarged pilosebaceous ca • The lesion increase in size by peripheral growth this is its typical feature.
  • 148. # • The periphery of the lesion appear pink or red while the center exhibit an atrophic scarred appearance indicative of long standing nature of the disease with characteristic healing. • the discoid form of the disease may also assume a typical ‘butterfly’ distribution on the malar process of the nose. since it is not a constant feature as it may appear in some other disease also. so its diagnostic significance should not be overemphasized.
  • 149. # • Oral manifestation- • There is 20-25 % of oral mucous mem involvement in DLE. • Oral mucosa is either involved prior or following the development of the skin lesion. The oral lesion begin as erythematous area ,sometimes slightly elevated but more often depressed usually without induration and typically with white spots. • Superficial ulcers with crusting or bleeding may form but without actual scales. Margins of lesions are not sharply demarcated but shows narrow zone of keratinization.
  • 150. # • Central healing may result in depressed scarring. • Lesions are mainly on buccal mucosa ,palate and tongue. • In case of tongue atrophy of papillae and severe fissuring is seen. • Vermillion border of the lip is also involved. • The erythematous atrophic plaque surrounded by a kera5otic border may involve the entire lip. Ulceration of the palate Ulceration of buccal mucosa
  • 151. # Histological finding- • It is characterized by hyperkeratosis with keratotic plugging, atrophy of rete pegs, perivascular infiltration of lymphocytes and their collection in dermal appendages
  • 152. # • Liquefaction degeneration of basal layer of cell. • Discoid form of the lesion exhibit hyperorthokeratosis hyper parakeratosis. • Acanthosis and pseudoepitheliomatous hyperplasia. • Hydrophobic degeneration and liquefaction necrosis of basal cell layer occurs
  • 153. # • Thickening of the basement mem can be demonstrated as a homogenous broad eosinophillic and PAS positive band. • Diffuse infiltration of lymphocytes with smaller number of plasma cells and occasionally polymorphonuclear leukocytes in superficial in deep CT tissue. Diagnosis- • Diagnosis of DLE usually requires a skin biopsy.
  • 154. # • A small sample of a discoid lesion is removed, specially prepared, and examined under a microscope. Usually, the lesion has certain microscopic characteristics that allow it to be identified as a DLE lesion. • Blood tests will not reveal the type of antibodies present in SLE, and physical examination usually does not reveal anything other than the skin lesions. If antibodies exist in the blood, or if other symptoms or physical signs are found, it is possible that the discoid lesions are a sign of SLE rather than DLE.
  • 155. # Treatment- • Treatment of DLE primarily involves the use of a variety of skin creams. Sunscreens are used for protection. Steroid creams can be applied to decrease inflammation. Occasionally, small amounts of a steroid preparation will be injected with a needle into a specific lesion. Because of their long list of side effects, steroid preparations taken by mouth are avoided. Sometimes, short-term treatment with oral steroids will be used for particularly severe DLE outbreaks. Medications used to treat the infectious disease malaria are often used to treat DLE.
  • 156. # Alternative treatment- • Alternative treatments for DLE include eating a healthy diet, low in red meat and dairy products and high in fish containing omega-3 fatty acids. These types of fish include mackerel, sardines, and salmon. Following a healthy diet is thought to decrease inflammation. Dietary supplements believed to be helpful include vitamins B, C, E, and selenium. Vitamin A is also recommended to improve DLE lesions. Constitutional homeopathic treatment can help heal DLE as well as help prevent it developing into SLE.
  • 157. # Prognosis- • For the most part, the prognosis for people with DLE is excellent. While the lesions may be cosmetically unsightly, they are not life threatening and usually do not cause a patient to change his or her lifestyle. Only about 10% of patients with DLE will go on to develop SLE. Prevention- • DLE cannot be prevented. Recommendations to prevent flares of DLE in patients with the disease include avoiding exposure to sun and consistently using sunscreen.