Arranged by: Putri Kurnia (1102014214)

Mentor: Kolonel CKM dr. Dian Andriani Ratna Dewi, SpKK, M.Biomed, MARS, FINSDV
Definition
Pemphigus is a collection of chronic autoimmune skin diseases, due to the
process of acantolysis and antibodies to components of the desmosome found
on the surface of IgG type keratinocytes.

Classification
1. Pemphigus vulgaris
2. Pemphigus erythematosus
3. Pemphigus foliaseus
4. Pemphigus vegetans
Definition
Pemphigus Vulgaris is an autoimmune disease with manifestations of blisters
on the surface of the skin and / or mucosa.

Epidemiology
The most common form (80%). Generally about middle age (4th and 5th
decade), but it can also be about all ages, including children.
Etiology
Pemphigus is autoimmune, because in the patient's serum is found
autoantibodies, it can also be caused by exogenous factors such as food,
drugs, viruses, ultraviolet radiation, X-rays, excessive pesticide exposure,
emotional stress, vaccination, high estrogen levels in pregnancy, and is
associated with other autoimmune diseases (rheumatoid arthritis, myasthenia
gravis, lupus erythematosus, or pernicious anemia).
Pathophysiology
PV is characterized by IgG autoantibodic deposition of glycoprotein
desmosomes, which causes damage to desmosomes by autoantibodies that
attack epithelial cell adhesion molecules. Because the oral epithelium mostly
expresses Dsg3 (Dsg1 and Dsg3 skin expressing), Dsg3 damage produces oral
lesions at an early stage.

Clinical Manifestations
This disease can start as a lesion on the hairy scalp or oral cavity, in the form of
erosion accompanied by crustal formation. Patients often complain of pain in
the skin being peeled.
Soft blisters filled with clear liquid

Erosion and healing area

Erosion
Diagnosis
Histological results in patients showed acolytic cells, Tzank cells were found,
and basal layers that remained attached to the basement membrane. Direct
immunofluorescence (DIF), used to detect the presence of autoantibodies.

Differential diagnosis
• Dermatitis Herpetiformis
• Pemfigoid Bulosa
Treatment
The main drug is corticosteroids because it is immunosuppressive, which is
often used is prednisone and dexamethasone. The dose of prednisone is 60-
150 mg. At high doses dexamethasone i.m or i.v. should be given To reduce the
side effects of corticosteroids can be combined with the strongest adjuvant,
namely cytostatic for pemphigus is azathioprine which is commonly used. The
dose is 50-150 mg a day.

Prognosis
Treatment with corticosteroids makes a better prognosis.
Definition
Pemphigus erythematosus or Senear-Usher syndrome, is a syndrome that
overlaps with the features of lupus erythematosus (LE) which is characterized
by acantholysis and deposits of immunoglobulins in interkeratinocyte
substances.

Epidemiology
There is no difference in the occurrence of pemphigus erythematosus between
2 sexes. Pemphigus erythematosus can occur at any age, but is not unusual in
children.
Etiology
Patients with pemfigus develop an autoimmune response directed at
desmosomes.

Pathophysiology
Patients with pemphigus erythematosus come with vesiculobullae or
superficially eroded lesions in areas exposed to sunlight, such as the face,
upper chest, and back.
Clinical Manifestations
Erythema spots are firmly bounded with squares and crusts on the face
resemble butterflies so that they resemble lupus erythematosus and seborrheic
dermatitis.

Erythematous, eroded and marshy scalp Violaceous and hyperpigmented patches and
flabby bullae that are slightly erythematous
Diagnosis
The diagnosis of PE is made with DIF which shows IgG and complement
deposition both on intercellular and on Dermoepidermal Junction (DEJ)
Serology of positive antinuclear antibodies is very positive patients have
positive ANA titers. ELISA serology also helps, often producing positivity for
both antibodies to desmoglein 1 and desmoglein 3. Histopathology alone shows
acantholysis in the superficial epidermis, consistent with PF.

Differential diagnosis
this disease resembles lupus erythematosus which is characterized by
erythema and squamous, there is atrophy and telangiectasia.
Diagnosis
The diagnosis of PE is made with DIF which shows IgG and complement
deposition both on intercellular and on Dermoepidermal Junction (DEJ)
Serology of positive antinuclear antibodies is very positive patients have
positive ANA titers. ELISA serology also helps, often producing positivity for
both antibodies to desmoglein 1 and desmoglein 3. Histopathology alone shows
acantholysis in the superficial epidermis, consistent with PF.

Differential diagnosis
this disease resembles lupus erythematosus which is characterized by
erythema and squamous, there is atrophy and telangiectasia.
Treatment
Treatment with corticosteroids such as pemphigus vulgaris and added adjuvant
drugs such as pemphigus.

Prognosis
This disease is considered to be a benign form of pemphigus, therefore the
prognosis is better than pemphigus vulgaris
Definition
Pemphigus foliaceus (PF) is an autoimmune blister disease in which the
immune system produces IgG autoantibodies that target adhesion between
desmoglein-1 glycoprotein cells

Epidemiology
Pemphigus foliaceus is rare and sporadic throughout the world. The average
age of patients at the beginning of pemphigus foliaceus is around 50-60 years
Etiology
Viral infections transmitted by insects, deposited by exposure to UV light or
burns, and by various drugs (for example, penicillamine, angiotensin convertase
inhibitors, nonsteroidal antiinflammatory agents).

Pathophysiology
Superficial blisters in pemphigus foliaceus are induced by immunoglobulin G
(IgG4) immunants. The polyclonal mixture in patients with pemphigus foliaceus
from IgG anti-Dsg1 antibodies increases the activity of pathogens for blister
formation.
Clinical Manifestations
The disease starts with the onset of vesicles, squads, and crustaceans and is
slightly exudative, then breaks down and leaves erosion.

A history of 9-year-old pemphigus foliaceus, Superficial bullae of pemphigus foliaceus.

often with erythrodermic flares.
Diagnosis
Based on three criteria: (1) Overall clinical picture, including patient history and
physical examination; (2) Histopathological findings; and (3) The presence of
autoantibodies detected by direct and indirect studies of immunofluorescence.

Differential diagnosis
• Erythroderma

Treatment
The treatment is the same as pemfigus erythematosus

Prognosis
Disease will be chronic
Definition
Pemphigus vegetans is the rarest pemphigus, characterized by eroded bullae or
eroded pustules

Classifications
1. Type Neumann
2. Type of Hallopeau (pyodermite vegetante)
Etiology
Pemphigus vegetans is caused by inter-cell autoanti-bodies especially against
desmogleins 1 and 3.

Clinical Manifestations
1. Type Neumann
There are sagging loops, erosion and then vegetative and proliferative
papillomatosa.

2. Type of Hallopeau (pyodermite vegetante)

Primary lesions are united pustules, becoming vegetative and covering large
areas.
 Erosive lip vesicles (A) and vegetative plaque hypertrophic verbs in inguinal folds (B)

Hypertrophic, cracked plaque with fluid flowing and peeling around the mouth, stretching above into the ala nasii
Diagnosis
The histopathology of the neumann type has early lesions similar to those of
pemphigus vulgaris, papillary papillary proliferation upwards, and intraepidermal
abscesses. While the hallopeau type was found to have the same initial lesions
as the neumann type, suprabasal acolysis. In further circumstances
papillomatosis and hyperkeratosis without abscess will appear.

Differential Diagnosis
Vegetan piostomatitis

Treatment
Same as in pemphigus vulgaris.

Prognosis
Hallopeau type has a better prognosis because it tends to heal.
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