This presentation will will give you the idea of a dermatological disoders with clinicopathological features.
A vesiculobullous disease is a type of mucocutaneous disease characterized by vesicles and bullae (i.e. blisters).
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Vesiculobullous disease
1. M I S B A H N A S I M
3 R D Y E A R B D S
Vesiculobullous Disease.
ِن ٰمْحَّالر ِهللا ِمْسِب
ِْمي ِحَّالر
2. Vesicubullous disease
Vesicle & Bulla
A clear fluid lesion just below the epithelium which
ruptures to form an ulcer.
If the lesion is smaller than 5mm than it is vesicle.
If larger than 5mm than it is a bulla.
5. Intraepithelial Vesiculobullous disease
IVD can be further subdivide into two groups
depending on the mechanism of formation of the
lesion
Acantholytic vesicles or bullae,
The lesions are produced by breakdown of intercellular attachment
(desmosomes) between epithelial cells.
Non-acantholytic vesicles or bullae,
The lesions are produced by death and rupture of groups of epithelial
cells.
6. Phemphigus Vulgaris
Autoimmune disease.
Most common in middle-aged Females.
Clinical Features
Characterized by fluid-filled bullae eruptions involving the
skin and mucous membrane.
These bullae are fragile and ruptures forming crusted or
weeping areas of denudation on the skin or mucosal ulcers.
Nikolsky’s sign positive.
LOCATION: Most affected areas are Soft palate, Buccal
mucosa & Lips.
8. Histopathology
Intraepithelial cleft like spaces are produced by
acantholysis.
The changes occur in stratum spinosum and basal cell
layer.
Unlike normal polyhedral stratum spinosum cells are small
rounded and contain enlarged hyperchromatic nuclie
(tzanck cells).
In basal layer the cell project into the bulla like a row of
tombstones.
Inflammatory cells are scanty however eosinophil may be
seen.
14. Clinical features
Location: skin, oral, genital, and
ocular mucosa.
Mucosal vesicles/oral ulceration.
Concentric rings with centric
crusted bulla.
Erosion on the lips are
accompanied by bleeding and
crusting.
Ocular mucosa -> conjunctival
scarring & visual impairment.
15. Histopathology
Necrosis of keratinocytes and vesicle formation
Infiltration of inflammatory cells.
Oedamatous epithelium and varying degree of
cellular damage.
17. Pemphigoid
Autoimmune vesiculobullous disease.
Affects skin and oral mucosa.
Characterized by the formation of bulla.
Subdivides into two groups
1. Bullous pemhigoid:
lesions involving skin alone with only minimal mucosal
involvment.
2. Mucous membrane pemphigoid:
lesions involving mucosa alone with only minimal skin
involvment.
19. Mucous membrane pemphigoid
Also called cicatrical phemphigoid.
More common in elderly females.
Characteristics
Bullae are tense, relatively tough and may remain intact for
a few days.
When they ruptures -> give rise to erosion which heals
slowly with scarring.
In addition to oral mucosa, the conjunctiva and mucosa of
nose, larynx, pharnyx, oesophagus and genitals may
involved.
20. Dermatitis herpetiformis
Oral manifestations are
variable & range from
small symptomless
erythematous areas to
extensive erosion.
90% of patients have
abnormality of their jejunal
mucosa associated with
gluten hypersensitivity.
21. Linear IgA
Rare subepithelial blistering
disease of the skin.
Patients may have gluten
hypersensitivty.
24. Epidermolysis bullosa
Cause: Gene mutations resulting in intraepithelial
and subepithelial bullae.
Extreme fragility of skin.
Develops in response to minimal trauma or pressure.
Claw like deformity.
Difficulties in eating, speaking and swalloing because
of the involvment of mouth, larynx and pharynx.