blistering diseases dr. abdulmajeed alajlan associate professor hair transplant & laser...
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Blistering Diseases
Dr. Abdulmajeed Alajlan
Associate ProfessorHair Transplant & Laser
Department of Dermatology- KSU
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Blistering Diseases
Objectives• To know the definition
& classification of Blistering diseases• To recognize the primary presentation of
different types of main blistering diseases• To understand the possible pathogenesis of
the main types of blistering diseases• To have an overview about managements
lines of these diseases
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• Vesicles and bullae are raised lesions that contain fluid.
• A vesicle is less than 0.5 cm in diameter. • A bulla is larger than 0.5 cm in diameter.
definition
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CLASSIFICATION OF VESICULOBULLOUS DISEASES
INTRA EPIDERMAL BLISTERS
The lesion is formed within the epidermis
SUB EPIDERMAL BLISTERS :
Lesions formed between the epidermis and the dermis
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PEMPHIGUS VULGARIS
BULLOUS PEMPHIGOID
CHRONIC BULLOUS DISEASE OF CHILDHOOD
PARANEOPLASTIC PEMPHIGUS
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Blistering Diseases
Objectives• To know the definition
& classification of Blistering diseases• To recognize the primary presentation of different
types of main blistering diseases
• To understand the possible pathogenesis of the main types of blistering diseases
• To have an overview about managements lines of these diseases
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Dermo-edidermal junction
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PEMPHIGUS VULGARIS
BULLOUS PEMPHIGOID
CHRONIC BULLOUS DISEASE OF CHILDHOOD
PARANEOPLASTIC PEMPHIGUS
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• Accurate pathological diagnosis requires a biopsy of a small newly formed lesion and of perilesional skin for immunopathological studies.
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Diagnostic tests
1. Routine histology– Lesional sample –small bulla or edge of
large one.
2. Direct immunofluorescence
– Perilesional sample
3. Indirect immunofluorescence
– Patient’s serum is added to specific substrates that express antigen of interest.
4. Electron microscopy.
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1. Routine histology
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immunofluorescence
IgGC3IgA
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Dermo-edidermal junction
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PEMPHIGUS VULGARIS
BULLOUS PEMPHIGOID
CHRONIC BULLOUS DISEASE OF CHILDHOOD
PARANEOPLASTIC PEMPHIGUS
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Blistering Diseases
Pemphigus Vulgaris
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Pemphigus• Pemphigus is a group
characterized by blistering of the skin and mucous membranes.
• Auto-antibodies against
DESMOSOMES in epidermis and mucosal surface.
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Intra epidermal blister
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Four sub-clinical varients :
Pemphigus Vulgaris: is the most common Pemphigus variant, and the form usually responsible for oral lesions
Others are:
Folacious, vegetens, erythematosus
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Pemphigus Vulgaris
• Begins with erosions on mucous membrane then other skin areas.
• Mostly erosions and few flaccid blisters• Very painful. • +ve Nikolsky’s sign
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+ve Nicholsky sign– Twisting pressure on normal skin shears skin.
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Pemphigus Vulgaris
• Begins with erosions on mucous membrane then other skin areas.
• Very painful. • +ve Nikolsky’s sign.• Age: middle-age 40-60 years.• Secondary infection and disturbance of fluid
and electrolyte balance are common complications .
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Pathology and immunopathology
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Acantholysis
ImmunofluorescenceIgG and C3
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Treatment • High dose systemic steroids 60-100 mg of
prednisolone. • Immunosuppressive agent such as azathioprine
cyclophosphamide , Methotrexate or mycophenolate
• Topical therapy is mainly symptomatic.• Patient will probably have to remain on
systemic steroids for long time.• Antibiotics
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Biological Rx:Rituximab
Biological Rx:IVIG (intravenous Ig)
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How do I treat pemphigus?I. mild
a.class III/IV corticosteroid creme / intralesional injection
II.severea.prednisolon 80 mg, taper in 5
months
b. Other immunosuppressive: azathioprine, Mycophenolate, etc
C. Biological Rx: Rituximab and IVIG (intravenous Immunoglobuling)
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Paraneoplastic pemphigus
• The least common and most severe type of pemphigus is paraneoplastic pemphigus (PNP). This disorder is a complication of cancer,
• usually lymphoma and Castleman's disease. It may precede the diagnosis of the tumor. Painful sores appear on the mouth, lips, and the esophagus.
• Complete removal and/or cure of the tumor may improve the skin disease,
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Both Intra epidermal and sub epidermal blister
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Bullous pemphigoid
• Characterized by large intact blisters on an erythematous base. it can errupt
to form erosions• Mainly in older age group• more than 60 y.
• The prognosis is usually good.
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Clinical features
• Elderly patents.• Large tense blisters on upper arms and
thighs.• Eczematous base .• Itch rather than pain.• Oral lesions are less frequent than
pemphigus.
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Pathology
• Sub epidermal between epidermis and dermis the epidermis forms the roof of the blister.
• Antigens identified are in hemidesmosomes.• Immunoglobulin and complement are
deposited in the lamina lucida of the basement membrane in a linear band.
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Treatment
Topical Steroids• Severe pemphigoid :Systemic steroids , but
unlike pemphigus, usually possible to discontinue Rx.
• The addition of either azathioprine enable the oral steroid dose to be reduced more rapidly.
• Milder may also respond very well to potent or moderately potent topical steroids alone.
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• Other treatment:
Antibiotics like tetracyclines group
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CHRONIC BULLOUS Disease OF CHILDHOOD
• Chronic blistering dis. which occur in children, usually starts before the age of 5yrs
• Small and large blisters appears predominantly on the lower trunk, genital area, and thighs
• May also affects the scalp and around the mouth • New blisters form around healing old blisters
forming a CLUSTER OF JEWELS
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CHRONIC BULLOUS Disease OF CHILDHOOD
• Course is chronic and spontaneous remission usually occurs after an average of 3-4 yrs
• IgA autoantibodies binds to proteins at dermo epidermal junction as linear pattern like the pattern of bullous pemphigoid
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CLINICAL FEATURES
• Circular clusters of large intact blisters and can erupt to form erosions
• It involves the perioral area, lower trunk, inner thighs and genitalia
• Blistering may spread all over the body
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INVESTIGATION
• Skin Biopsy will show subepidermal splits• Direct IF reveals IgA along the BM of the
epidermis in a linear pattern
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TREATMENTS
• Oral dapsone (check G6PD)What the side effect of dapsone?
• Sulphonamides and immunosupressants• Erythromycine• Flucloxacillin
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Drug-induced PV
Drugs can induce PV Drugs reported most significantly in
association with PV are;Penicillamine captopril
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Blistering Diseases
Objectives• To know the definition
& classification of Blistering diseases• To recognize the primary presentation of
different types of main blistering diseases• To understand the possible pathogenesis of
the main types of blistering diseases• To have an overview about managements
lines of these diseases