Vesiculobullous Disorders

Dr / Ali Farhan

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

The skin is
composed of an
epidermis,
dermis, and
subcutis.

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

In normal
tissue, epithelial
cells are held
together by
adhesion
molecules

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

The epidermis
connect to dermis
By
Dermoepidermal
Junction
(basement
membrane zon)

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Granular cell
layer Subcorneal
Acantholysis is the
term used to describe Spinous layer
a loss of cohesion
between keratinocytes
due to the break down Basal cell area

of intercellular
bridges. It results in
the formation of
intraepidermal clefts,
vesicles and bullaes

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

• Vesicle
Elevated,circumscribed
<1 cm in diameter Filled
with fluid.
• Bulla :
Elevated,circumscribed
>1 cm in diameter Filled
with fluid.

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

There are many
disorders that
can cause defect
in this adhesion
molecules and
lead to blistering
disease

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

CLASSIFICATION OF VESICULOBULLOUS DISORDERS

Primary causes secondary causes

• burns,
• Inherited/genetic • suction blisters.
blistering disorders:
• ischemia
• insect bite reaction.
• Acquired autoimmune • infections
blistering disorders

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

CLASSIFICATION OF VESICULOBULLOUS DISORDERS

Primary causes

• Inherited/genetic • Acquired autoimmune

blistering disorders: blistering disorders

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 CLASSIFICATION OF VESICULOBULLOUS DISORDERS

Primary causes
• Inherited/genetic • Acquired autoimmune
blistering disorders: blistering disorders

Epidermolysis bullosa Intraepidermal (PEMPHIGUS).

Subepidermal
(BULLOUSPEMPHIGOID)

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Epidermolysis bullosa

Epidermolysis bullosa (EB)

comprises a group of
genetically determined skin
disorders characterized by
blistering of the skin and
mucosae at birth or soon
afterwards, following mild
mechanical trauma (due to
increased fragility of skin).

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Epidermolysis bullosa

intraepidermal split due

EB simplex to disruption of basal
keratinocytes

(split through the

Junctional
basement membrane
EB
zone)

Dystrophic split in the subepidermal

EB level

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Epidermolysis bullosa

Diagnosis

✦SKin Biopsy.
✦Electron Microscopy
To Ascertain The Level Of Split And
Structures Involved

Treatment

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Epidermolysis bullosa

Treatment

✦ No Specific Treatment Is Available For Eb.

✦ Avoidance Of Provoking Factors.
✦ Maintaining Adequate Nutrition And
Hydration .
✦ Prevention Of Sepsis

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Intraepidermal (PEMPHIGUS).

• Pemphigus is derived from the Greek

‘pemphix' meaning blister or bubble and
is characterized by intraepidermal
blistering at various levels in the
epidermis.

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Intraepidermal (PEMPHIGUS).

• The key pathogenic process in this group

of disorders is disruption of the
intercellular cementing substance due to
an autoantibody attack on the cellular
adhesion proteins (desmogleins) leading
to acantholysis

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Pemphigus vulgaris

• Most common form of pemphigus, accounting for up to 80% of

pemphigus cases.

• Occurs at any age, most commonly between fourth- sixth

decades.

• It is due to IgG antibodies directed against epidermal cell

adhesion molecules (desmoglein 3)- disruption of intercellular
cementing substance—loss of adhesion between epidermal cells
(acantholysis) intraepidermal blistering.

Clinical Features

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Clinical Features

• Almost all patients have mucosal lesions, 50–

70% present with painful oral erosions.

• Skin – flaccid bullae on normal or

erythematous skin, with a predilection for scalp,
face ,trunk,axillae and groins.

• Bullae rupture producing painful erosions.

• Pruritus is absent .

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Clinical Features

• Nikolsky's sign gential pressure with finger tip producing moist

erosion due to peeling of skin.

• gentle pressure on an intact bulla forces the fluid to spread under the
skin away from the site of pressure (Asboe-Hansen sign)

Diagnosis

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Diagnosis

• Tzanck smear from the floor of the

blister shows acantholytic cells.
Acantholytic cell is a large, rounded
epidermal cell with a large nucleus,
perinuclear halo.

• Histopathological examination of a
blister shows a supra-basal cleft in the
epidermis.

• Immunofluorescence studies are the

gold standard in diagnosis of the
autoimmune blistering disorders. Treatment

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Pemphigus vulgaris
Treatment

• The mainstay of therapy in the pemphigus group of disorders is

with systemic steroids ,oral prednisolone in the dose of 1 mg per
kg body weight)

• The other modalities of therapy include adjuvant therapy with

dapsone, azathioprine, cyclosporine, methotrexate

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Pemphigus vegetans

clinical variant of pemphigus vulgaris

characterized by vegetating lesions
primarily in the flexures

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Pemphigus foliaceus

characterized by blistering at a higher level

in the epidermis is less common than
pemphigus vulgaris and accounts for only
15–20 % of pemphigus cases.

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Pemphigus erythematosus

It is a variant of pemphigus
foliaceous characterized by
immunological features of
both pemphigus and lupus
erythematosus (LE)

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Subepidermal
(BULLOUSPEMPHIGOID)

• Autoimmune subepidermal blistering

disorder of the elderly, with onset usually
after 60 years of age.

• Basic pathogenic process is due to IgG

antibodies against components of the
basement membrane zone ➡ separation
of the intact epidermis from underlying
dermis ➡ subepidermal cleft formation.

Clinical Features

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Clinical Features

• Preceded by pruritus with or without urticarial wheals

lasting usually for 1–3 weeks.
• Tense bullae on normal or erythematous skin
predominantly over flexural aspects of the limbs, lower
abdomen , groins and axillae.
• Lesions rupture to leave erosions that heal
spontaneously with postinflammatory
hyperpigmentation.
• Nikolsky's sign usually negative; Bulla spread sign +/-
• Mucosal lesions rare and less severe than in pemphigus
vulgaris and almost never the presenting feature. Diagnosis

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Diagnosis

• Histopathological examination
reveals subepidermal cleft with intact
epidermis.

• Direct immunofluorescence.

Treatment

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

 Treatment

• The mainstay of treatment is a topical or systemic steroid

depending on the severity of the disease

• Other modalities include cyclophosphamide, azathioprine,

dapsone, tetracycline.

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022

Characteristic BULLOUS PEMPHIGOID Pemphigus vulgaris

Age > 60 years Middle age

Pruritus ++ Not present
Blister Tense Flaccid
Flexures, groins, axillae, Face, scalp, trunk,
Site
abdomen pressure points
Erosion Spontaneously heal Extend peripherally
Nikolsky's sign Negative Positive
40 % less severe, almost 80–90 % severe
Oral lesions never apresenting commonly the
feature Presenting symptom
Prognosis Benign, self limiting Poor without treatment

Vesiculobullous disorders Dr.Ali Farhan - 16 December 2022