BULLOUS DISORDERS

DR MARIA MANSOOR
ASSISTANT PROFESSOR
DEPARTMENT OF DERMATOLOGY
DOW UNIVERSITY OF HEALTH SCIENCES
 CLASSIFICATION

IMMUNOLOGICAL
NON IMMUNOLOGICAL
1. INFECTION 1. EPIDERRMAL
 Bacterial  PEMPHIGUS VULGARIS
 Viral  PEMPHIGUS VEGETANS
 fungal  PEMPHIGUS FOLIACEUS
2. TRAUMA
 Burn
2. SUBEPIDERMAL
 PEMPHIGOID
3. DRUGS
 LINEAR IGA
 Barbiturates
 DERMATITIS
4. METABOLIC HERPETIFORMIS
 Diabetes mellitus
5. GENETIC(MECHANOBULLOU)
 Epidermolysis Bullosa
BULLOUS IMPETIGO
VIRAL INFECTION
BURN
STEVEN JOHNSON SYNDROME
MICROSCOPIC LEVEL OF BLISTERING
DERMOEPIDERMAL JUNCTION
1. EPIDERRMAL BLISTER
 PEMPHIGUS VULGARIS
 PEMPHIGUS VEGETANS
 PEMPHIGUS FOLIACEUS
 PEMPHIGUS VULGARIS

TARGET ANTIGEN
DESMOSOME
PEMPHIGUS VULGARIS
PEMPHIGUS VULGARIS
PEMPHIGUS VULGARIS
AGE MIDDLE AGE
SITE SCALP
FACE
FLEXURES
MUCOSA
BLISTER FLACCID

ANTIGEN DESMOSOME(DEMOGLEIN 3)

ANTIBODIES IgG
IMF INTERCELLULAR

 At higher power, the suprabasilar cleft

becomes more apparent. Dyscohesion
along the superficial and lateral borders
of the basal keratinocytes, or
"tombstoning", is present.
PEMPHIGUS VULGARIS
PEMPHIGUS VEGETANS

A. Crusted, vegetating lesions in pemphigus vulgaris.

B. Extensive, vegetating granulomatous lesions in pemphigus vegetans
 PEMPHIGUS VEGETANS

AGE MIDDLE AGE

SITE FLEXURES
MUCOSA

BLISTER VESICLE PUSTULE

ANTIGEN DESMOSOME(DEMOGLEIN 1)

ANTIBODIES IgG

IMF INTERCELLULAR
PEMPHIGUS FOLIACEUS
 PEMPHIGUS FOLIACEUS

AGE MIDDLE AGE

SITE SCALP,FACE,CHEST,
UPPER BACK

BLISTER SCALY PAPULE,CRUSTED

EROSION

ANTIGEN DESMOSOME(DEMOGLEIN1)

ANTIBODIES IgG

IMF INTERCELLULAR
2. SUBEPIDERMAL BLISTER
 PEMPHIGOID
 LINEAR IgA
 DERMATITIS HERPETIFORMIS
PEMPHIGOID

TARGET ANTIGEN
HEMIDESMOSOME
TARGET ANTIGEN
 BULLOUS PEMPHIGOID

AGE ELDERLY
SITE TRUNK,LIMB,
FLEXURES
MUCOSA
BLISTER TENSE
ANTIGEN HEMIDESMOSOME
ANTIBODIES IgG
IMF LINEAR BMZ
 At higher power of the cleavage plane,
there are numerous eosinophils within
the papillary dermis, migrating into the
epidermis and blister cavity. The blister
cavity also contains fibrin. Mild spongiosis
is also noted. Immunofluorescence was
performed and confirmed the diagnosis
(not shown).
BULLOUS PEMPHIGOID
BULLOUS PEMPHIGOID
LINEAR IG A

TARGET ANTIGEN
ANTIGEN
HEMIDESMOSOME
LINEAR Ig A

AGE YOUNG ADULTS

SITE TRUNK LIMB

BLISTER TENSE

ANTIGEN HEMIDESMOSOME
LAMINA LUCIDA

ANTIBODIES IgA

IMF LINEAR BMZ

LINEAR Ig A
 CHRONIC BULLOUS DERMATOSIS OF CHILDHOOD

 AGE YOUNG ADULTS

 SITE TRUNK LIMB
 BLISTER TENSE
 ANTIGEN HEMIDESMOSOME
 LAMINA LUCIDA
 ANTIBODIES IgA
 IMF LINEAR
BMZ
CHRONIC BULLOUS DERMATOSIS OF
CHILDHOOD (string of pearls sign)
LINEAR IG A
 Histologic sections of skin show
separation of the epidermis from
the dermis in a subepidermal
plane. Within the papillary
dermis and the blister cavity,
there are numerous neutrophils
with admixed eosinophils. Well-
formed papillary microabscesses
are not appreciated
LINEAR IG A
 The finding of a linear band of
IgA at the dermal-epidermal
junction is characteristic of
linear IgA bullous dermatosis
but also found in some cases of
bullous pemphigoid.
LINEAR IGA
 The diagnosis of this disease
requires three criteria: a vesicular
or bullous eruption; a
subepidermal vesicle with a
predominantly neutrophilic
infiltrate on histology; and
basement membrance zone-
specific IgA antibody deposited in
a linear pattern On Direct
immunofluorescence. There have
been cases that also involved IgG
antibodies deposited linearly at
the basement membrane in
addition to the IgA.
 DERMATITIS HERPETIFORMIS

 AGE YOUNG
ADULTS

 SITE TRUNK LIMB

 BLISTER TENSE

 ANTIGEN HEMIDESMOSOME
 LAMINA LUCIDA

 ANTIBODIES IgA

 IMF granular
deposits
DERMATITIS HERPETIFORMIS
 Histologic sections of skin show
several collections of
neutrophils, in clusters,
concentrated in the dermal
papillae. There is focal linear
deposition of neutrophils along
the dermal-epidermal junction,
with focal clefting.
DERMATITIS HERPETIFORMIS

Granular deposits of IgA in the dermal papillae and

along the basement membrane
GENETIC BLISTERING DISEASES

 Epidermolysis bullosa (EB) is a group

of genetic conditions that result in skin
fragility & easy blistering of the skin and
mucous membranes. ... EB is due to a
mutation in at least one of 18 different genes.
Some types are autosomal dominant while
others are autosomal recessive.
GENETIC BLISTERING DISEASES
CLASSIFICATION
 EPIDERMOLYSIS BULLOSA SIMPLEX
 JUNCTIONAL EPIDERMOLYSIS BULLOSA
 DYSTROPHIC EPIDERMOLYSIS BULLOSA
 EPIDERMOLYSIS BULLOSA
PATHOGENESIS

DIMINISHED
BASAL CELL AND/OR
DEGENERATION ABNORMAL
EBS HEMIDESMOSOME
JEB

DIMINISHED
AND/OR ABNORMAL
ANCHORING
FIBRILS
EBD
 EPIDERMOLYSIS BULLOSA SIMPLEX
PATHOGENESIS

BASAL CELL
DEGENERATION
EBS
EPIDERMOLYSIS BULLOSA SIMPLEX
 JUNCTIONAL EPIDERMOLYSIS BULLOSA
PATHOGENESIS

DIMINISHED
AND/OR
ABNORMAL
HEMIDESMOSOME
JEB
JUNCTIONAL EPIDERMOLYSIS BULLOSA
EPIDERMOLYSIS BULLOSA DYSTROPHICA
PATHOGENESIS

DIMINISHED
AND/OR ABNORMAL
ANCHORING
FIBRILS
EBD
DYSTROPHIC EPIDERMOLYSIS BULLOSA