JOURNAL READING

REVIEW
DIAGNOSIS AND CLASSIFICATION OF
PEMPHIGUS AND BULLOUS PEMPHIGOID
(JOESTIANTHO LAURENZ KILMANUN)

30/10/2018 Consultan : dr. Novriyani Masuku, Sp.KK, M.Kes

 Introduction
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•Pemphigus and bullous pemphigoid are part of a large group of cutaneous

autoimmune blistering disorders of unknown etiology

•Pemphigus encompasses a group of life-threatening diseases of which there are

three major variants: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic
pemphigus

•Bullous pemphigoid represents the most common type of autoimmune blistering

disorder, and is characterized by autoantibodies located at the dermo-epidermal
junction against specific antigens in the hemidesmosomes thus causing complete
separation of the epidermis from the dermis.
 History
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Beutner and Jordan utilized

Both pemphigus and bullous
indirect immunofluoresence
pemphigoid have served as Acantholysis in
(IIF) in discovering that the
models for studying and pemphigus patients was first
sera of pemphigus vulgaris
understanding autoimmune described by Civatte in 1943
patients contained IgG
diseases
autoantibodies against the
cell surface of keratinocytes
 History
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Jordan and Beutner were the

Lever who in 1953 first to discover through indirect
first described bullous pemphigoid and direct immunofluorescence
as a sole entity distinct from other (DIF) that the autoantibodies in
bullous disorders bullous pemphigoid patients were
localized in the basement membran
 Epidemiology
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Pemphigus vulgaris is the most frequent form of pemphigus.

The mean age of onset is 50–60 years of age, although many cases
have been described in the elderly and children

The incidence worldwide ranges from 0.7 to 5 new cases per million
per year.

Bullous pemphigoid represents the most common type of autoimmune

blistering disorder, with an incidence of 6–7 new cases per million per year
which rises to 150–330 per million per year in those over 80 years old
 Pathogenesis Pemphigus
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 It is well established that IgG plays a pivotal role in

pemphigus
 Desmosomes, which are composed of desmosomal
cadherins, plakoglobin, plakophilin and esmoplakin,
are molecular complexes specialized for cell-to-cell
adhesion by anchoring intermediate filaments
 The target antigens identified in pemphigus vulgaris
and foliaceus are part of the cadherin family. They
are Desmoglein 1 (DSG1) and Desmoglein 3 (DSG3)
 Pathogenesis Pemphigus
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 The antigens described in paraneoplastic

pemphigus patients are primarily those of the
plakin family these being envoplakin,
desmoplakin I, desmoplakin II, plectin and
periplakin. However, other antigens have also
been reported such as DSG3, DSG1, bullous
pemphigoid antigen 1 (BPAG1) and, most
recently, the alpha-2 macroglobulin-like-1
 Pathogenesis Bullous pemphigoid
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 In bullous pemphigoid the antigens targeted by

circulating autoantibodies have been described
as BPAG1 and bullous pemphigoid antigen 2
(BPAG2) or type XVII collagen. These are key
components of the hemidesmosomes and play a
major role in cell matrix adhesion
 While both antigens are recognized and targeted
by T and B cells in bullous pemphigoid, it has
been demonstrated that the NCA16 region of
BPAG2 is the main site.
 Pathogenesis Bullous pemphigoid
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 These autoantibodies that bind complement lead

to the disruption of the dermo-epidermal junction
 Classification
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 Other diagnostic tests
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 The autoantibodies present in both pemphigus

and bullous pemphigoid can be detected by
more accurate serological assays, including
immunoprecipitation, immunoblotting and ELISA.
However, these are not currently used as routine
tests, and are indicated in cases that are difficult
to diagnose.
 Other diagnostic tests
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 Even though immunofluoresence continues to be

the test of choice in the diagnosis of bullous
pemphigoid, it does not provide information
regarding the exact molecular target of the
autoantibodies. Therefore, other techniques,
such as immunoprecipitation and
immunoblotting have gained diagnostic weight.
 Diagnostic criteria
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Pemphigus Vulgaris Bullous Pemphigoid

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THANK YOU
Joestiantho Laurenz Kilmanun
 Pathogenesis Pemphigus
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 The desmosome is a critical junction for

cell to cell adhesion in the skin and
mucous membranes. The major
desmosomal proteins are desmogleins,
desmocollins, plakoglobin, plakophilins,
and desmoplakin. Desmogleins and
desmocollins are
transmembrane glycoproteins of the
cadherin (calcium-dependent cell
adhesion molecule) family. Plakoglobin
and plakophilins are components of the
desmosomal plaque. Desmoplakin binds
to plakoglobin and links the desmosomal
plaque to keratin filaments of the
cytoskeleton. Envoplakin and periplakin
are additional desmosomoal proteins
found in the desmosomal plaque.
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 Pemphigus Vulgaris
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 Pemphigus Vulgaris
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 Pemphigus Vulgaris
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 Pemphigus Foliaceus
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 Pemphigus Foliaceus
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 Paraneoplastic pemphigus
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 Paraneoplastic pemphigus
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 Pathogenesis Bullous Pemphigoid
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 Bullous Pemphigoid
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 Bullous Pemphigoid
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