Bullous Dermatoses112

Bullous Dermatoses
Liangdan Sun
Institute of Dermatology Anhui Medical University
Institute of Dermatology
Anhui Medical University
Classification of bullous dermatoses
Etiological classification
Anatomy Category
Genetic Autoimmune Allergic Infectious Metabolic Physical
Epidermolysis Pemphigus Contact Impetigo bullosa Porphyria Frictional

bullosa dermatitis bullous
hereditaria Pemphigoid Bulla erysipelas Diabetes disease
Bulla bullous
Familial benign Epidermolysis multiform SSSS, etc disease, etc Chilblain
chronic bullosa acquisita erythema
pemphigus Burn, etc
Linear IgA TEN, etc
Ichthyosis bullous
dermatosis
Incontinentia
pigmenti, etc Dermatitis
herpetiformis
Anatomy Category
Anatomy Category
Intraepidermal vesicular disease Subepidermal vesicular disease
Pemphigus Bullous pemphigoid
Epidermolysis bullosa acquisita
Linear IgA bullous dermatosis
Dermatitis herpetiformis
Bullous Systemic Lupus Erythematosus
Pathogenesis
Localization of target adhesion sites and cleft formation in selected hereditary and
autoimmune bullous diseases.
Adhesion of keratinocytes
Keratinocytes are firmly adhered by desmosomes.

Transmembrane adhesion molecules in the
cadherin superfamily, such as desmoglein 1
(Dsg1), desmoglein 3 (Dsg3), and desmocolin
cadherin (DC), are important to intercellular
adhesion.
In pemphigus, autoantibodies are produced against Dsg1 and Dsg3, some of whose
molecular functions are disturbed. This causes acantholysis.
Main Contents
1 Pemphigus
2 Bullous Pemphigoid
3 Dermatitis Herpetiformis
Pemphigus
 Autoantibodies against desmocollins and desmogleins in the epidermis
 Superficial erosions and blisters on both epidermal and mucosal surfaces
 Desmocollins and desmogleins are transmembrane desmosomal glycoproteins
Ag
Cell signal transduction Activation of

pathways proteolytic enzymes
Ab
Acantholysis
Hydrolysis connecting
Blister on both epidermal
structure between cells
and mucosal surface
Classification of Pemphigus
Pemphigus
Pemphigus vulgaris Pemphigus vegetans
Pemphigus
Pemphigus foliaceus
erythematosus
Specific types
Herpetiform
IgA pemphigus
pemphigus
Paraneoplastic
Drug-induced
pemphigus
Epidemiology
• Pemphigus vulgaris（PV）
Rare, more common in Jews and people of Mediterranean descent. In
Jerusalem the incidence is estimated at 16 per million, whereas in France and
Germany it is 1.3 per million.
• Pemphigus foliaceus（PF）
Also rare but endemic in rural areas in Brazil (fogo selvagem), where the
prevalence can be as high as 3.4%.
• Age of Onset
40 to 60 years; PF also in children and young adults.
• Sex
Equal incidence in males and females, but predominance of females with PF
in Tunisia and Colombia.
Etiology and pathogenesis
 An autoimmune disorder.
 Loss of the normal cell-to-cell adhesion in the epidermis ( acantholysis) occurs as a
result of circulating antibodies of the IgG class;
 These antibodies bind to desmogleins, transmembrane glycoproteins in the
desmosomes, members of the cadherin superfamily.
 In PV, desmoglein 3 (in some, also desmoglein 1).
Pathogenesis
Mechanism of pemphigus, according to the expression of desmoglein 1 and desmoglein 3
Clinical Manifestation
Pemphigus vulgaris: Classic initial lesion: flaccid, easily ruptured vesicle or bulla on normal-
appearing skin. Ruptured vesicles lead to erosions that subsequently crust.
Pemphigus vulgaris
 The most common form of pemphigus,

generally seen in elderly patients
 Involve the scalp，chest，and intertriginous
areas
 Thin walled, relatively flaccid, easily ruptured

bullae, apparently normal skin and mucous
 “Nikolsky sign” positive
Histopathology and Immunopathology
Acantholysis is observed on the

basement cells
Intercelluar deposition of IgG is observed
Institute of Dermatology in the epidermis

Pemphigus vegetans
 A subtype of pemphigus vulgaris .
 Often accompanied by vesicles

and pustules.
 There is a strong odor, and the

disorder frequently occurs on
areas exposed to friction.
 Has a better prognosis.
Pemphigus Foliaceus
 Most commonly affects the middle–

aged and elderly.
 Extremly fragile flaccid vesicles are

produced, some of which dry to
become leafy and to exfoliate
successively.
 The face, head, back and chest are

commonly affected.
 Does not involve the mucosa.

Nikolsky’s sign is positive.
Pemphigus Erythematosus
 A subtype of pemphigus foliaceus, and it

occurs most commonly in the middle-aged and
elderly.
 Erythema or seborrheic dermatitis-like

eruptions occur
 The mucosa is not involved.
Diagnosis
LABORATORY EXAMINATIONS
Dermatopathology
PV：Separation of keratinocytes, suprabasally, leading to split just above the basal cell
layer and vesicles containing separated,rounded-up (acantholytic) keratinocytes.
Immunopathology
Direct immunofluorescence (IF) staining reveals IgG and often C3 deposited in lesional
and paralesional skin in the intercellular substance of the epidermis .
Serum
Autoantibodies (IgG) detected by indirect IF (IIF) or enzyme-linked immunosorbent
assay (ELISA). Titer usually correlates with activity of disease process.
Diagnosis
ELISA
Anti-Dsg1 IgG Anti-Dsg3 IgG Diagnostic name
antibody antibody
− + Pemphigus vulgaris(mucosa predominant type)
+ + Pemphigus vulgaris(mucocutaneous type)
+ − Pemphigus foliaceus
− − Normal or Non-pemphigus
Autoantibody against desmoglein detected by ELISA, and comfirmed diagnosis
Diagnosis
Types of pemphigus groups
Pemphigus Pemphigus Pemphigus Pemphigus
vulgaris vegetans faliaceus erythematosus
Middle age of Middle age of Middle age of
Age of onset elderly elderly Midderl age elderly
Whole body
Frequent site of skin,oral
skin lesion mucosa intertriginous areas Whole body skin Oily areas of skin
Erosion,butterfly
Blister,erosion,papil rash,seborrheic
lary Erosion,lamellar
dermatitis-like skin
Skin Blister,erosion acanthosis,pustules exfoliation,crustslesion
Mucosal lesions ✚✚ ✚ − −
Clinical finding Nikolsky's sign ✚ ✚ ✚ ✚
skin intraepidermal blisters(acantholysis)
Tzanck test ✚ ✚ ✚
Pathological Lower epidermal layer (directly on Upper epidermal layer(granular cell
finding Site of acantholysis basal cells) layer)
Target antigen Only Dsg3,Dsg3 and Dsg1 Only Dsg1
ELISA Dsg1(✚/-),Dsg3(✚) Dsg1(✚),Dsg3(-)
Immunofluoresce Direcet(skin lesion) IgG(✚)in the epidermal intercellar space,C3(✚) positive

nce technique Indirect (serum) IgG(✚)
Steroids, immunosuppressants, plasmaphersis, human immunoglobulin
Treatment therapy
Management
Glucocorticoids
2 to 3 mg/kg body weight of prednisone until cessation of new blister formation
and disappearance of Nikolsky sign.
Then rapid reduction to about half the initial dose until patient is almost clear,
followed by very slow tapering of dose to minimal effective maintenance dose.
Concomitant Immunosuppressive Therapy

Immunosuppressive agents are given concomitantly for their glucocorticoid-
sparing effect:
Other Measures
Cleansing baths, wet dressings,topical and intralesional glucocorticoids,
antimicrobial therapy per documented bacterial infections.
Correction of fluid and electrolyte imbalance.
Summary
 A serious, acute or chronic, bullous autoimmune disease of skin and mucous

membranes based on acantholysis.
 Two major types: pemphigus vulgaris (PV) and pemphigus foliaceus (PF)
 PV: flaccid blisters on skin and erosions on mucous membranes. PF: scaly and
crusted skin lesions.
 PV: suprabasal acantholysis. PF: subcorneal acantholysis
 IgG autoantibodies to desmogleins, transmembrane desmosomal adhesion molecules.
 Serious and often fatal unless treated with immunosuppressive agents.
Bullous pemphigoid
Epidemiology
 Age of Onset 60 to 80 years.
 Sex Equal incidence in males and females. No known racial predilection.
 Incidence
The most common bullous autoimmune disease. Seven per million in Germany and
France. Far more common in very old people.
Etiology and pathogenesis
 Interaction of autoantibody with bullous pemphigoid antigen [BPAG1 and

BPAG2 (collagen type XVII)] in hemidesmosomes of basal keratinocytes
 Followed by complement activation and attraction of neutrophils and

eosinophils.
 Bullous lesion results from interaction of multiple bioactive molecules

released from inflammatory cells.
Clinical features
• Erythematous, papular or urticarial-
type lesions may precede bullae
formation by months.
• Bullae: large, tense, firm-topped, oval
or round
• May arise in normal, erythematous,

or urticarial skin and contain serous
or hemorrhagic fluid
• Mucous Membranes Practically only

in the mouth (10–35%); less severe
and painful and less easily ruptured
than in pemphigus
Clinical features
Generalized eruption with confluent

urticarial plaques and multiple tense blisters.
The condition is severely pruritic.
Note urticarial plaques and a small, tense

blister with a clear serous content.
Histopathology and Immunopathology
A distinct subepidermal blister. Inflammatory

cells including eosinophils are seen.
Linear deposition of IgG is observed in the

epidermal basement membrane.
Diagnosis and treatment
Bullous pemphigoid
Age of onset Elderly (youth in some cases
Frequent site of
skin lesion Whole body
Findings Tense blisters, edematous erythema, itching
Clinical finding Mucosal infiltration ✚

Pathological
finding Eosinophilic infiltration
Target antigen BP180,BP230
Linear deposition of IgG and C3 in the epidermal
Direct basement membrane zone(BMZ)
Immunofluorescen
ce Findings Indirect(Serum) Detection of antibasement membrane antibody
Treatment Oral steroids,immunosuppressant, DDS
Cicatricial pemphigoid
 A rare disease, largely of the elderly.
 Blisters that rupture easily and also erosions resulting from epithelial fragility in the
mouth; oropharynx;
 Ocular involvement may initially manifest as unilateral or bilateral conjunctivitis

with burning, dryness, and foreign-body sensation.
 Chronic involvement results in scarring, symblepharon, in severe disease, fusion of

the bulbar and palpebral conjunctiva.
 Entropion and trichiasis result in corneal irritation, superficial punctate

keratinopathy, corneal neovascularization, ulceration, and blindness.
 This scarring condition in a 78-

year-old female started with
bilateral conjunctival pain and
foreign body sensation as the first
symptoms.
 The conjunctiva then became

erosive with scarring and fibrous
tracts between eyelids and the eye.
Pemphigoid gestationis
 A rare pruritic and polymorphic inflammatory bullous dermatosis of pregnancy and

the postpartum period.
 Extremely pruritic vesicular eruption mainly on the abdomen but also on other
areas, with sparing of the mucous membranes.
 Lesions vary from erythematous, edematous papules to urticarial plaques to
vesicles and tense bullae.
 Histopathologically: it is a subepidermal blistering condition, and there is a heavy
linear deposition of C3 along the basement membrane zone with concomitant IgG
deposition in roughly 30% of patients.
A.
Erythematous papules that were
highly pruritic and had appeared on
the trunk and abdomen of this 33-
year-old pregnant female (third
trimester) and were a cause of great
concern.
At this time there were no blisters and
diagnosis was established by biopsy
and immunopathology.
(Continued)
B. Urticarial plaques and small vescicles and blisters in another patient who had
similar eruptions in previous pregnancies. She responded rapidly to systemic
glucocorticolds. The delivery was uneventful, and the baby was healthy.
Dermatitis Herpetiformis
 Definition
 Etiology and Pathogenesis
 Epidemiology
 Clinical manifestations
 Diagnosis
 Differential diagnosis
 Treatment
Definition
Dermatitis herpetiformis (DH) is a chronic, relapsing, severely pruritic disease

Characteristic by grouped, symmetrical lesions on extensor surfaces, the scalp, nuchal area,
and buttocks.
Clinical Features
The eruption usually occurs on an erythematous base

and may be papular, papulovesicular, vesiculobullous
bullous, or urticarial.
Linear petechial lesions may be noted on

the volar surfaces of the fingers as well
as the palms
Clinical Features
Dermatitis herpetiformis:
These are the classic early lesions. Papules, urticarial plaques, small grouped vesicles,
and crusts on the elbow of a 23-year-old male.
Clinical Features
 In this 56-year-old male patient with a
generalized highly pruritic eruption.
 Upon close inspection there are grouped
papules, small vesicles, crusts, and
erosions on an erythematous base.
Clinical Features
 Itching and burning

 Paroxysmal and intense
 Spontaneous remissions lasting as long as a week and terminating
abruptly with a new crop of lesions are a characteristic feature of
the disease.
Clinical Features
Dermatitis herpetiformis.
Pattern of distribution.
Typical and almost diagnostic:
extensor areas elbows, knees.
Buttocks, scapular and sacral areas
Pathogenesis
 Genetic Factors:
 Seventy-seven to 90% of patients with DH and IgA deposits in
the skin are HLA-B8 positive.
 HLA antigens DR3 and DQw2 are also increased in frequency.
 Causative Factors:
 Gluten, a protein found in cereals except for rice and corn,
provokes flares of the disease.
 Oral iodides will cause a flare of the disease
Pathogenesis
 IgA immune complexes was suspected playing some

role in the pathogenesis.
 Villous atrophy of the jejunum and inflammation of the small
bowel occurs.
 IgA is bound to the skin, and this apparently activates
complement, primarily via the alternate pathway.
Associated Disease
Thyroid disorders are An increased incidence of

increased in incidence in malignancy, especially small bowel
patients with DH. lymphoma has also been noted.
Epidemiology
 This disease has an equal male-to-female incidence.

 The average age of onset is between 20 and 40 years.
 It does occur with some frequency in children.
 Black and Asian persons are rarely affected.
Histopathology
 Edema, focal fibrin, and neutrophilic microabscesses at the

tips of the dermal papillae.
 The cellular infiltrate contains many neutrophils, but may also
include a few eosinophils.
 Subepidermal separation.
 Lymphocytic infiltrate, ectatic capillaries, and fibrosis in the
dermal papillae.
Histopathology
Histopathologic features of erythema multiforme

(Hematoxylin and eosin stain; original magnification×5)
Direct Immunofluorescence(DIF)
IgM and IgG deposits are occasionally

observed in association with IgA junction.
The granules may be vertically
elongated, giving a "picket-fence"
appearance
DIF of noninvolved perilesional skin
reveals deposits of IgA alone or together
with C3 arranged in a granular pattern at
the dermoepidermal junction.
Diagnosis
 Clinical Features  Skin biopsy

 Linear petechial lesions;  Edema, focal fibrin, and neutrophilic
 Erythematous base and bullous ; microabscesses at the tips of the

dermal papillae.
 Itching;
 The cellular infiltrate contains many
neutrophils, but may also include a
 DIF
few eosinophils.
 "picket-fence" appearance;
 Subepidermal separation.
 IgA/C3 at the dermoepidermal junction;  Lymphocytic infiltrate, ectatic
capillaries, and fibrosis in the dermal

papillae.
Prevention
 Gluten-Free Diet
 Patients must strictly avoid
wheat, barley, and rye.
 Moderate amounts of oats
may be tolerated.
 Corn and rice are generally
well tolerated.
Treatment
 Dapsone
 The dosevaries between 50 and 300 mg/day，
 G6PD level should be done before therapy.
 Liver function tests should be monitored bimonthly for the first
4 months, then checked with the hematologic studies every 4 to
6 months.
Treatment
 Sulfapyridine
 After a test dose of 0.5 g of sulfapyridine, one tablet (0.5 g)
four times a day is given.

 Usually 1 to 4 g/day is required for good control.
Bullous Dermatoses
Diagnosis and Differential diagnosis
Hereditary epidermolysis bullosa
Based on level of cleavage and blister formation there are three main types:
 Epidermolytic.
Cleavage occurs in keratinocytes: EB simplex (EBS)
 Junctional.
Cleavage occurs in basal lamina: junctional EB (JEB)
 Dermatolytic.
Cleavage occurs in most superficial papillary dermis: dermolytic, or dystrophic, EB (DEB)
Classification of EB
In each of main types groups there are several distinct types of EB based on clinical,
genetic, histologic/electronmicroscopic, and biochemical evaluation.
EB Simplex (EBS)
 A trauma-induced, intraepidermal blistering
 Most cases on mutations of the genes for keratins 5 and 14 resulting in a
disturbance of the stability of the keratin filament network.
 Causes cytolysis of basal keratinocytes and a cleft in the basal cell layer.
 Different subgroups have considerable phenotypic variations, eight distinct forms,
dominantly inherited.
EB Simplex (EBS)
 This 4-year- old girl, blistering

since very early infancy with
predilection for traumatized
body sites.
 Despite multiple blistering
episodes there is hardly any
evidence of scarring on the
palms of this child.
Generalized EBS
EB Simplex (EBS)
 The most common form of EBS.
Onset in childhood or later.
 May not present itself until
adulthood, when thick-walled
blisters on the feet and hands occur
after excessive exercise, manual
work, or military training.
 Increased ambient temperature
facilitates lesions. Hyperhidrosis of
palms and soles is associated, and
Localized EBS
secondary infection of blistered
lesions often occurs.
Junctional EB (JEB)
 All forms of JEB share the pathologic feature of blister formation within the lamina
lucida of the basement membrane.
 Mutations are in the gene for collagen XVII and laminin.
 This trait is autosomal recessive and comprises clinical phenotypes depending on the
type of genetic lesion and environmental factors.
 Three principal subtypes:
I) JEB Gravis (Herlitz EB)
II) JEB Mitis
III) Generalized Atrophic Benign Epidermolysis Bullosa (GABEB)
JEB Gravis (Herlitz EB)
 There are large eroded, oozing

and bleeding areas that occurred
intrapartum.
 When this newborn is lifted up,

dislodgment of epidermis as well
as erosions occur with manual
handling.
Junctional epidermolysis bullosa

(Herlitz variant)
GABEB
Generalized atrophic benign epidermolysis bullosa
This 19-year-old man has had cutaneous

blistering since birth, with blisters and erosions This 20-year-old man has had generalized
arising on the elbows and knees but also on the cutaneous blistering since birth. Note: erosions
trunk and arms following trauma. Note: ill- on the left lower back and hemorrhagic crusts
defined erythemas at sites of previous blistering. on the lower arms. Erythema on the back
There is no scarring but some spotty atrophy. indicates sites of previous blistering.
Dystrophic Epidermolysis Bullosa ( DEB )
 A spectrum of dermolytic diseases where blistering occurs below the basal lamina;
 Healing is therefore usually accompanied by scarring and milia formation, thename

dystrophic.
 There are four principal subtypes, and all are due to mutations in anchoring fibril
type VII collagen.
I) Dominant DEB
II) Recessive DEB (RDEB)
Recessive DEB (RDEB)
Generalized recessive dystrophic epidermolysis bullosa
Blisters lead to erosions and Loss of all fingernails, syndactyly,

these become ulcers that have a and severe atrophic scarring on the
low tendency to heal. dorsa of the hand
Familial benign pemphigus
 Hailey-Hailey disease, is a rare genodermatosis with dominant inheritance, with
blistering disorder but actually presents as an erythematous, erosive, oozing
condition with cracks and fissures localized to the nape of the neck, axillae.
 Submammary regions, inguinal folds, and scrotum are major sites of involvement.
 The pathologic process is acantholysis whereby the fragility of the epidermis, a
defect in the adhesion complex between desmosomal proteins and tonofilaments.
 The genetic abnormality lies in ATP2CI, encodes an ATP-powered calcium pump.
Familial benign pemphigus
 This 46-year-old male, oozing lesions in

both axillae, occasionally in the groins,
also on the nape of the neck
 Eruptions worsen during the summer
months. The father and sister have
similar lesions that wax and wane.
 Lesions are painful and show typical
cracks and fissures within an erosive
erythematous plaque
 Familial benign pemphigus hardly ever
Familial benign pemphigus shows intact vesicles and is often

mistaken for intertrigo.
Linear IgA Dermatitis
 A rare, immune-mediated, subepidermal

blistering skin disease defined by the
presence of homogeneous linear deposits
of IgA at the cutaneous basement
membrane zone.
 Clinical manifestations are very similar

to those of DH, but there is more
blistering. Patients present with
combinations of annular or grouped
papules, vesicles, and bullae
It is identical with chronic bullous

disease of childhood, which is a
rare blistering disease that occurs
predominantly in children <5 years.
Presence of homogeneous linear

deposits of IgA at the cutaneous
basement membrane zone.
Linear IgA dermatosis
(chronic, bullous disease of childhood)
 Extensive blistering on the upper extremities
and trunk in a 7-year-old child.
 Note: blisters are both tense and flaccid.
They are grouped and there is no notable
inflammation.
 A chronic subepidermal bullous disease associated with autoimmunity to the type

VII collagen within the anchoring fibrils in the basement membrane zone.
 This is a the bullous pemphigoid-like

presentation with tense bullae, erosions,
and crusts on an erythematous base.
 There is extensive postinflammatory

pigmentation due to previous blistering.
 Histopathology of lesional skin:

subepidermal blisters with a clean
separation between the epidermis and
dermis.
 Immunopathology reveals linear IgG,

at the dermalepidermal junction. If salt
split-skin IIF is performed, circulating
antibasement membrane zone.
Diagnosis
Differential Diagnosis of Important Acquired Bullous Diseases
Diagnosis
Differential Diagnosis of Important Acquired Bullous Diseases
Bullous dermatosis
Laboratory testing Methods
Methods
Histopathology
Immunopathology
Enzyme linked immunosorbent assay
Immunoblotting
Electron microscope
Institute of Dermatology 76
Histopathology
Intraepidermal vesicular Subepidermal vesicular
Immunopathology
Direct immunofluorescence Indirect immunofluorescence
Mainly used to detect antibodies or Mainly used to detect the presence of circulating
complement present in the diseased tissue. serum autoantibodies.
DIF IIF
fluorescein
fluorescein
anti-antibody
anti-antibody
specific antibody serum antibody
Skin antigen
Substrate
Enzyme linked immunosorbent assay
The enzyme-linked immunosorbent

assay (ELISA) is a test that uses
antibodies and color change to
identify a substance.
ELISA is a popular format of "wet-

lab" type analytic biochemistry
assay that uses a solid-phase enzyme
immunoassay (EIA) to detect the
presence of a substance, usually an
antigen, in a liquid sample or wet
sample.
Immunoblotting
Pemphigus Pemphigoid
Epidermolysis
bullosa acquisita
Electron microscope
Basal cells Acantholysis
The city of HeFei
AnHui Medical
University
Institute of Dermatology and Department
of Dermatology at No. Hospital
1

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Bullous Dermatoses

Institute of Dermatology Anhui Medical University

Genetic Autoimmune Allergic Infectious Metabolic Physical

Epidermolysis Pemphigus Contact Impetigo bullosa Porphyria Frictional

Intraepidermal vesicular disease Subepidermal vesicular disease

Pemphigus Bullous pemphigoid

Epidermolysis bullosa acquisita

Linear IgA bullous dermatosis

Bullous Systemic Lupus Erythematosus

Keratinocytes are firmly adhered by desmosomes.

Cell signal transduction Activation of

Pemphigus vulgaris Pemphigus vegetans

 Loss of the normal cell-to-cell adhesion in the epidermis ( acantholysis) occurs as a

result of circulating antibodies of the IgG class;

 These antibodies bind to desmogleins, transmembrane glycoproteins in the

desmosomes, members of the cadherin superfamily.

 In PV, desmoglein 3 (in some, also desmoglein 1).

Mechanism of pemphigus, according to the expression of desmoglein 1 and desmoglein 3

 The most common form of pemphigus,

 Involve the scalp，chest，and intertriginous

 Thin walled, relatively flaccid, easily ruptured

 “Nikolsky sign” positive

Acantholysis is observed on the

Intercelluar deposition of IgG is observed

Institute of Dermatology in the epidermis

 A subtype of pemphigus vulgaris .

 Often accompanied by vesicles

 There is a strong odor, and the

 Has a better prognosis.

 Most commonly affects the middle–

 Extremly fragile flaccid vesicles are

 The face, head, back and chest are

 Does not involve the mucosa.

 A subtype of pemphigus foliaceus, and it

 Erythema or seborrheic dermatitis-like

 The mucosa is not involved.

− + Pemphigus vulgaris(mucosa predominant type)

+ + Pemphigus vulgaris(mucocutaneous type)

Autoantibody against desmoglein detected by ELISA, and comfirmed diagnosis

Immunofluoresce Direcet(skin lesion) IgG(✚)in the epidermal intercellar space,C3(✚) positive

Concomitant Immunosuppressive Therapy

 A serious, acute or chronic, bullous autoimmune disease of skin and mucous

 Age of Onset 60 to 80 years.

 Sex Equal incidence in males and females. No known racial predilection.

France. Far more common in very old people.

 Interaction of autoantibody with bullous pemphigoid antigen [BPAG1 and

 Followed by complement activation and attraction of neutrophils and

 Bullous lesion results from interaction of multiple bioactive molecules

• May arise in normal, erythematous,

• Mucous Membranes Practically only

Generalized eruption with confluent

Note urticarial plaques and a small, tense

A distinct subepidermal blister. Inflammatory

Linear deposition of IgG is observed in the

Findings Tense blisters, edematous erythema, itching

Clinical finding Mucosal infiltration ✚

Treatment Oral steroids,immunosuppressant, DDS

 A rare disease, largely of the elderly.

 Ocular involvement may initially manifest as unilateral or bilateral conjunctivitis

 Chronic involvement results in scarring, symblepharon, in severe disease, fusion of

 Entropion and trichiasis result in corneal irritation, superficial punctate

 This scarring condition in a 78-

 The conjunctiva then became