EPIDERMOLYSIS BULLOSA

&
ERYTHEMA MULTIFORME
ABSTRACT

EB
The disorder known as epidermolysis bullosa constitutes of a
group of genetic disease of the skin. The mildest form is
debilitating throughout life and in most severe form result in
death at early age
EM
Is an episodic variable, self-limited, often recurrent.
Inflammatory disorder of skin and mucous membrane.
EPIDERMOLYSIS
BULLOSA
INTRODUCTION
BULLA: fluid-filled blister more than 5mm in diameter

EPIDERMOLYSIS: loosening of epidermis with extensive

blistering of skin and mucous membrane

EPIDERMOLYSIS BULLOSA: a group of inherited bullous

disorder characterized by blister formation in response to
mechanical trauma
CLASSIFICATION
EPIDERMOLYSIS BULLOSA

EB SIMPLEX JUNCTIONAL EB DYSTROPHIC EB EB AQUISTA

(EBS) (JEB) (DEB) (acquired)

GENERALIZED DOMINANT

LOCALIZED
(Weber- Cockayne RECESSIVE
Syndrome)
ETIOLOGY
EB is a subepithelial and subepidermal mechanobullous disease
In the hereditary form, antibodies are not evident.
Etiology is mainly due to genetic defects in basal cell,
hemidosmosomes or anchoring connective tissue filaments.
Dental abnormalities have been associated with different types of
EB. For example:
anodontia
enamel hypoplasia
pitting of the enamel
neonatal teeth
severe dental caries
ETIOLOGY
EBS JEB DEB
Mutation of genes Caused by one of Caused by mutation
coding for two recurrent non- of one of the genes
Keratin 5 & 14 severe mutation in coding for Type VII
the LAMB3 gene collagen.
Anchoring fibrils are
also affected in
patient with DEB
SIGN & SYMPTOMS
Blistering of the skin
Tooth decay
Deformed or lack of finger/ toe nails
Internal blistering in the throat, stomach, and intestine
Scalp blistering and loss of hair
Excessive sweating
Hardening of the skin on the feet or hands
Thin skin appearance with white bumps
Difficulty in swallowing
CLINICAL FEATURES
EPIDERMOLYSIS BULLOSA SIMPLEX
Mild form; autosomal dominant
Sites of trauma/ friction
Involve hand, feet and neck; occasionally knees and elbow
Teeth are not affected; intraoral blister seen
Appears during infancy

Localised blister at site of trauma

Hemorrhagic bulla on buccal mucosa
JUNCTIONAL EPIDERMOLYSIS BULLOSA
Severe form; autosomal recessive
Haemorrhagic blister; loss of nail, large blister of face, trunk and
extremities
Generalized scarring and atropy
Intraorally- haemorrhagic blister of palate, perioral and perinasal
area
Erupted teeth exhibited hypoplastic and severely pitted enamel
prone to caries
DYSTROPHIC EPIDERMOLYSIS BULLOSA
DOMINANT RECESSIVE (classic)
Onset in infancy Onset at birth
Heals by scarring which is sometimes keloidal Heals by severe milia, scar and pigmentation
in type
Oral Manifestation: 1)bullae, preceded by white spot on the oral
1)Mild gingiva erythema mucous membrane.
2)oral milia 2)obliteration of sulci & restriction of tongue
3)Teeth are unaffected movement
3)Congenitally absent teeth, hypoplastic teeth
Blistering on ankles, knees, elbows feet & head Sites: feet, scapulae, elbows, fingers & occiput

Nail are thick & dystrophic Hair be sparse & nail dystrophic/absent
DOMINANT DYSTROPHIC EB
RECESSIVE DYSTROPHIC EB
HISTOLOGICAL FEATURES
Lysis of basal and suprabasal cells
within the epidermis, resulting in
development of bullae and vesicle
Basal and suprabasal cells become
edematous- showing dissolution of
organelles and tonofibrils, with
displacement of nucleus to the
upper end of cells
 EB DYSTROPHIC
EB DYSTROPHIC
EB SIMPLEX RECESSIVE &
DOMINANT
JUNTIONAL EB

Immediately beneath
LOCATION OF Intraepidermal and Along the basement
the basement
BULLAE suprabasal membrane
membrane

FIBRES IN THE Elastic, pre elastic and

Elastic, pre elastic and
UNDERLYING Absence of elastic and oxytalin fibres are
oxytalin fibres are
CONNECTIVE oxytalin fibres increased in number
normal
TISSUE

Normal, seen in Normal fragments of

Normal, flattened on
BASAL LAYER dermal side of the basal layer may
the roof of the blister
separation adhere to the dermis
DIFFERENTIAL DIAGNOSIS
Pemphigus
Epidermolysis Bullosa Acquisita DIAGNOSIS
Bullous Pemphigoid Clinical diagnosis: Nikolskys sign
Linear IgA Disease is positive
Bullous Erythema Multiforme Histopathology
Dermatitis Herpetiformis Direct and Indirect
immunofluorescence
Cicatricial Pemphigoid of
Childhood
Bullous Dermatoses of Childhood
 TREATMENT MANAGEMENT
Types of lesions Mucous bulla
Milder Food
Large blister
Severe cases
Prognosis
Mitten deformity of hands
Oesophagus
 ERYTHEMA
MULTIFORME
INTRODUCTION
It is an acute self-limiting dermatitis
It is characterized by blistering, ulcerative mucocutaneous
condition
CLASSIFICATION
ERYTHEMA MULTIFORME

STEVEN- JOHNSON
MINOR MAJOR
SYNDROME (SJS)

No mucosal Localised skin Involvement of

involvement eruption skin, oral
Severe cavity, genital
eruption with
mucosal
involvement
ETIOLOGY

Infectious agent drug (sulfa drug)

EM minor is triggered by HSV infection in most cases
CLINICAL FEATURES
Most seen in young adult (20-40 yrs)
Males > female
Present over hands and arms, feet, leg, face, neck
Only few cm or less in diameter
Concentric circular erythematous ring resembling a target or
bulls eye or iris
ORAL MANIFESTATION
Hyperemic macules, papules or vesicle may become roded or
ulcerated and bleed freely
Tongue, buccal mucosa, palate, gingiva are commonly involve
Healing take up to
3 weeks in EM minor
6 weeks in EM major
STEVEN- JOHNSON SYNDROME (SJS)
A very serious bullous form of erythema multiforme
It involved skin, oral cavity, eyes and genital
It start abruptly with fever, malaise, photophobia
Oral mucous membrane lesion maybe extremely severe and are so
painful that mastication is impossible
Lip may exhibit ulceration with bloody crusting and painful
Toxic epidermal necrolysis (TEN) is a very serious, often fatal bullous
drug eruption
TEN now considered to be a confluent form of SJS
HISTOLOGIC FEATURE
Cutaneous or mucosal lesion
generally exhibit intracellular
edema of spinous layer of
epithelium and edema of the
superficial connective tissue
Severe liquefaction degeneration
of upper layer of epithelium
Dilatation of superficial capillaries
and lymphatic vessel of the
subepithelial connective tissue
Inflammatory infiltrate
composed of chiefly lymphocytes
but often neutrophils and eosinophils
are also present
DIFFERENTIAL DIAGNOSIS
Vesicular allergic contact dermatitis

Herpetic Gingivostomatitis

Chronic pemphigus

Benign mucosal pemphigoid

Bechet's Syndrome

Reiters syndrome
DIAGNOSIS
Nikolsky sign (-ve)
Bulls eye lesion with haemorrhagic crusting of the lip
Positive serology for HSV antibodies
Biopsy of professional tissue
MANAGEMENT
Supportive care: electrolyte, liquid diet and nutritional support
should be started as early as possible
Removal of cause
Rehydration
Topical mouthwash or gel: topical anaesthetic agent (lidocaine
gels, benzocaine lozenges) and topical corticosteroids (1%
triamanolone acetonide, 0.05% clobatasol propionate)
Immunomodulatory drugs: dapsone 50-100mg/day
Acyclovir- oral acyclovir 400mg twice a day 4-6 month
LETS PLAY SOME
GAME!
EB B 1)Epidermolysis bullosa is group of
inherited bullous disorder characterized
S I by formation in response to
mechanical trauma

W E E R C K Y E
P

D
H M D S O S M

C L H I I
EB B 1)Epidermolysis bullosa is group of
L inherited bullous disorder characterized
S I by formation in response to
mechanical trauma
S
T
W E E R C K Y E
P R

D
H M D S O S M

C L H I I
EB B 2)Localized EB Simplex also known as
L . Syndrome
S I
S
T
W E E R C K Y E
P R

D
H M D S O S M

C L H I I
EB B 2)Localized EB Simplex also known as
L . Syndrome
S I
S
T
W E B E R C O C K A Y N E
P R

D
H M D S O S M

C L H I I
EB B 3)EB is a subepithelial and
L mechanobullous disease
S I
S
T
W E B E R C O C K A Y N E
P R

D
H M D S O S M

C L H I I
EB B 3)EB is a subepithelial and
L mechanobullous disease
S I
U S
B T
W E B E R C O C K A Y N E
P R
I
D
H E M D S O S M
R
M
A
C L H I I
EB B 4)Etiology of EB is mainly due to genetic
L defects in basal cell, or
S I anchoring connective tissue filaments.
U S
B T
W E B E R C O C K A Y N E
P R
I
D
H E M D S O S M
R
M
A
C L H I I
EB B 4)Etiology of EB is mainly due to genetic
L defects in basal cell, or
S I anchoring connective tissue filaments.
U S
B T
W E B E R C O C K A Y N E
P R
I
D
H E M I D O S M O S O M E
R
M
A
C L H I I
EB B 5).. is given in EB acquired
L
S I
U S
B T
W E B E R C O C K A Y N E
P R
I
D
H E M I D O S M O S O M E
R
M
A
C L H I I
EB B 5).. is given in EB acquired
L
S I
U S
B T
W E B E R C O C K A Y N E
P R
I
D
H E M I D O S M O S O M E
R
M
A
C O L C H I C I N
EM D H
Y E R M C

P
A
S T V E J H S N

T S

B L S Y E M
1) EM is an acute self-limiting .
L
E
EM D H
Y E R M C
R
M P
A
S T V E J H S N
I
T S
I
B L S Y E M
1) EM is an acute self-limiting .
L
E
EM D H
Y E R M C
R
M P
A
S T V E J H S N
I
T S
I
B L S Y E M
2) EM can be classified into 3 types: major,
minor and .. Syndrome L
E
EM D H
Y E R M C
R
M P
A
S T E V E N J O H N S O N
I
T S
I
B L S Y E M
2) EM can be classified into 3 types: major,
minor and .. Syndrome L
E
EM D H
Y E R M C
R
M P
A
S T E V E N J O H N S O N
I
T S
I
B L S Y E M
3) EM minor is triggered by Virus
infection in most cases L
E
EM D H
Y E R M C E
R R
M P
A E
S T E V E N J O H N S O N
I
T S
I I
B L S Y E M
3) EM minor is triggered by Virus P
infection in most cases L
E
X
EM D H
Y E R M C E
R R
M P
A E
S T E V E N J O H N S O N
I
T S
I I
B L S Y E M
4) Oral Manifestation: .. macules, P
papules or vesicle may become roded or L
ulcerated and bleed freely E
X
EM D H
H Y P E R E M I C E
R R
M P
A E
S T E V E N J O H N S O N
I
T S
I I
B L S Y E M
4) Oral Manifestation: .. macules, P
papules or vesicle may become roded or L
ulcerated and bleed freely E
X
EM D H
H Y P E R E M I C E
R R
M P
A E
S T E V E N J O H N S O N
I
T S
I I
B L S Y E M
5) Oral Manifestation: . lesion with P
haemorrhagic crusting of the lip L
E
X
EM D H
H Y P E R E M I C E
R R
M P
A E
S T E V E N J O H N S O N
I
T S
I I
B U L L S E Y E M
5) Oral Manifestation: . lesion with P
haemorrhagic crusting of the lip L
E
X
SUMMARY
INTRODUCTION ?

CLASSIFICATION ?

ETIOLOGY ?

CLINICAL FEATURES ?

STEVEN JOHNSON SYNDROME

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