Pemphigoid and Pemphigus2

20/4/2018 ORAL MEDICINE
PEMPHIGOID
is the term given to a group of uncommon sub-epithelial immunologically mediated vesiculobullous

disorders (SEIMD) which can affect stratified squamous epithelium characterized by damage to one of
the protein constituents of the basement membrane zone (BMZ) anchoring filaments components.
A number of other sub-epithelial vesicullobullous disorders may produce similar clinical features(table1)
table 1: uncommon sub-epithelial vesiculobullous disorders:
● pemphigoid variants
● acquired epidermolysis bullosa (EBA)
● toxic epidermal necrolysis(TEN)
● Erythema multiforme
● Dermatitis herpetiforms
● linear IgA disease
● chronic bullous dermatosis of childhood
the main types of pemphigoid that involve the mouth are:
● mucous membrane pemphigoid(MMP) in which mucosal lesions predominate but skin lesions
are rare
● oral mucosal pemphigoid patients with oral lesions only without a progressive ocular scarring
process and without serologic reactivity to bullous pemphigoid (BP) antigens
● bullous pemphigoid (BP) which affects mainly the skin
● ocular pemphigoid which is sometimes termed cicatricial pemphigoid (CP) since it may cause
serous conjunctival scarring.
Mucous membrane/oral pemphigoid
mucous membrane pemphigoid (benign mucous membrane pemphigoid) is an uncommon chronic

disease twice as common in females and usually presenting in the fifth to sixth decades.
Mucous membrane pemphigoids is an autoimmune type of disorder with a genetic predisposition.
The precipitating event is unclear in most cases but rare cases are drug induced(eg by furosemide or
penicillamine).
it is characterized immunologically by deposition of IgG and C3 antibosies directed against the epithelial
basement memrane zone(BMZ)
There are also circulating antibodies in BMZ components and desmosomes of the lamina ........?
the antibodies damage the BMZ and histollogically there is a sub-basilar split.
Clincal features
The oral lesions affect especially the gingivae and palate and include bullae or vesicles which are tense
may be blood filled and remain intact for several days
persistent irregular ulcers appear after the blisters rupture anf if on the gingivae can produce
desquamative gingivitis -the most common oral finding.
This is characterized by erythematous ulcerated tender gingiva in a patchy rather than continuous
distribution.
The majority of the people with MMP have only oral lesions but general involvement can cause great
morbidity and untreated ocular involvement can lead to blindness . Nasal laryngeal and skin blisters are
rare.
Diagnosis
the oral lesions of the pemphigoid may be confused clinically with pemphigus or occasionally erosive
lichen planus erythema multiforme or the subepithelial blistering conditions shown in table1
biopsy of prelesional tissue with histological and immunstaining examination can therefore be essential
to the diagnosis
Management
spontaneous remission is rare and thus treatment is indicated. Specialist advice is usually needed.
An opthalmology consultation can be needed .
The majority of cases respond well to topical corticosteroids. Non-steroidal immunosupresive agents
may be needed if the response is inadequate.
severe pemphigoid may need to be treated with systemic corticosteroids.
PEMPHIGUS
pemphigus is a group of rare potentially life-threatening chronic disease characterized by epithelial

blistering affecting cutaneous and/or mucosal surfaces.
There are several varients with different autoantibody profiles and clinical manifestations but the main
type is pemhigus vulgaris this includes an uncommon variant pemhigus vegetans.
pemphigus vulgaris is seen mainly in middle aged and elderly females of Mediterranean Arabs,
Ashkenazi jewish or south Asian descent.
Pemphigus vulgaris is an autoimmune disorder in which there is fairly srong genetic background.
Rare cases have been trigered by medications ( especially captopril, penicillamine, rifampine and
diclofenac) or other factors.
The autoantibodies are directed against stratified squamous epithelium desmosomes .
Damage to the Desmosomes leads to loss of cell-cell contact (acantholysis), and thus INTRA-EPITHELIAL
vesiculation.
Clinical features
pemphigus vulgaris typically runs to chronic course, causing blisters, erosions on the mucosa and blisters
and scabs on the skin.
oral lesions are common(95%) may be an early manifestation (50%).
Blisters rapidly breakdown to leave erosions seen mainly on the palate (soft palate and posterior hard
palate),buccal mucosa,lips and gingiva white lesions usually comprise severe desquamative or erosive
gingivitis, flaps of peeling tissue with red erosions or deep ulcerative craters are seen, mainly on the
attached gingiva)
The NIKOLSKY sign(the spreading of a blister by pressure) is positive.
Diagnosis
to differentiate pemphigus from other vesiculobullous diseases, a careful history and physical
examination are important, but biopsy of prelesional tissue, with histological and immunostaining
examination are crucial.
serum should be collected for antibody titres.
Management
Before the introduction of corticosteroids, pemphigus vulgaris typically was fatal, mainly from
dehydration or secondary systemic infections.
Current treatment, by systemic immunosupression, has significantly reduced the mortality to about
10%.
Specialist care is mandatory.

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20/4/2018 ORAL MEDICINE

is the term given to a group of uncommon sub-epithelial immunologically mediated vesiculobullous

table 1: uncommon sub-epithelial vesiculobullous disorders:

mucous membrane pemphigoid (benign mucous membrane pemphigoid) is an uncommon chronic

Mucous membrane pemphigoids is an autoimmune type of disorder with a genetic predisposition.

An opthalmology consultation can be needed .

severe pemphigoid may need to be treated with systemic corticosteroids.

pemphigus is a group of rare potentially life-threatening chronic disease characterized by epithelial

The autoantibodies are directed against stratified squamous epithelium desmosomes .

oral lesions are common(95%) may be an early manifestation (50%).

The NIKOLSKY sign(the spreading of a blister by pressure) is positive.

serum should be collected for antibody titres.

Specialist care is mandatory.

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