BLISTERING DISEASES OF SKIN

NUR FADZLINA
ZABRI
08201310006
SERIAL NO 4

Macule : A circumscribed change in the color

of skin that is neither raised nor depressed

Papule : A small swelling forming part of the

rash

Blister : A small bubble on the skin filled with

serum and caused by friction, burning, or other
damage.

Vesicle : Air or fluid-filled cavity

Conditions causing blisters : Skin

exposed to friction and rubbing

Heat and burns
Contact with irritant
Infection : Chicken pox, Herpes
Autoimmune

disease : skin disease

Autoimmune blistering
diseases
Group of disorders in which the body
mistakenly attacks healthy tissue,
causing blistering lesions that primarily
affect the skin and mucous and
membranes.

Autoimmune blistering diseases

Vesicle and blisters (bullae) usually occurs as

a secondary phenomena
May occur as primary phenomenon as well
Occurs at multiple levels within skin
Types
Pemphigus
Bullous

Pemphigoid
Dermatitis Herpetiformis

Types

Pemphigus

Bullous
Pemphigoid

Dermatitis
Herpetiformis

Pemphigus

A rare autoimmune blistering disorder

Due to loss of integrity of normal intercellular

attachments within the epidermis and mucosal
epithelium

In middle-aged and older

Pemphigus
vulgaris

Pemphigus
foliaceus

Pemphigu
s

Paraneoplasti
c pemphigus

Pathogenesis

Pemphigus vulgaris and foliceus are caused

by Type II hypersensitivity reaction

Patients sera contain pathogenic IgG

antibodies to intercellular desmosomal
proteins

Morphology

Acantholysis (separation of individual

keratinocytes due to lysis of intercellular
adhesion sites)
Detached acantholytic cells become rounded

Pemphigous foliaceus

Pemphigous vulgaris

Pemphigous
paraneoplastic

Acantholysis involves
superficial epidermisstratum granulosum

Acantholysis involves the

layer of cells just above
the basal layer

Acantholysis involves the

layer between epidermal
and dermis

Subcorneal blister

Suprabasal blister

Subepidermal blister

Clinical features

Pemphigus vulgaris
Involves mucosa and skin
Especially scalp, face, skin, groin, trunk
Primary lesion : superficial vesicle and bullae that rupture
easily
Leaving erosion covered with serum crust

Pemphigus foliaceus

More rare and benign form of pemphigus

Results in bullae confined to skin
Infrequent involvement of mucous membrane
Blisters are superficial
Only zones of erythema and crusting sites of previous blister
rupture are detected

Paraneoplastic pemphigus
Associated

with internal malignancy

Painful mucosal erosions , sometimes with a skin
eruption that eventually results in blisters and erosions,
in the setting of confirmed or occult malignancy
Demonstration

of antiplakin antibodies
Histopathologic changes of acantholysis or
lichenoid/interface dermatitis

 Patients

with tumors may develop paraneoplastic

pemphigus. These include thymoma, sarcoma, and
lung carcinoma.

A sample from the buccal mucosa reveals irregular epidermal

hyperplasia, dyskeratosis, and blister formation. There is a
dense lichenoid infiltrate comprised primarily of lymphocytes
as well as a deep mixed inflammatory infiltrate

Affects elderly people

Presents with bullous lesion on normal or
erythematous skin and mucosa
Bullae are tense and filled with clear fluid
Sites :

Inner aspects of thighs, flexor surface of forearms, axillae,

groin, lower abdomen

Pathogenesis

Autoimmune disorder
Linear deposits of IgG antibodies and
complement in the basement membrane zone
Area affected is the basal cell-membrane
attachment (hemidesmosomes), where bullous
pemphigoid antigen is located
This protein is involved in dermoepidermal
bonding
IgG autoantibodies to hemidesmosome
components fixes complement with
subsequent tissue injury

Morphology

Characterized by subepidermal non

acantholytic blister
Lesion show perivascular inflammation
Superficial dermal edema and associated
basal cell liquefaction
Eventually give rise to blister

Affects predominantly male in 3rd and 4th

decades
May be associated with gluten sensitive
enteropathy (celiac disease)
Urticarial plaque and vesicle are seen
Bilaterally symmetrical distribution on :

Elbow , knees, upper backs, and buttock

Pathogenesis

Presence of IgA antibodies to dietary gluten

Antibodies cross react with reticulin
A component of fibril which anchor the
epidermal basemant to the superficial dermis
Resulting injury produces a subepidermal
blister

Morphology

Formation of microabscess
Basal cells show vacuolization and focal
dermoepidermal separation
Leading to formation of subepidermal bulla
Direct immunofluorescence show
discontinous, granular deposit of IgA localized
in the tip of dermal papillae

LAB DIAGNOSIS

Lab diagnosis
Microscopy
Tzanks

smear
Nikolskys sign
Direct immunofluorescence

Nikolskys sign

Tzanks smear

Pemphigus (Tzanck smear): Clusters of

acantholytic cells with round.

Pemphigus vulgaris

Paraneoplastic pemphigus

Direct immunofluorescence

Pemphigus

Bullous pemphigoid

Dermatitis
herpetiformis

Conclusion
Pemphigus vulgaris

Bullous pemphigoid

Blistering diseases and examples

Types of pemphigus
Bullous Pemphigoid
Dermatitis Herpetiformis
Lab diagnosis

References

Robbins Basic Pathology 8th edition by Kumar

and Abbas
Pathology 2nd edition by Geetika Khanna for
Elsevier
www.ncbi.nlm.nih.gov
www.mayoclinic.com