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Pemphigus vulgaris
anti-desmoglein 1, 3
antibodies
Bullous pemphigoid
anti BPAg1, 2
anti bullous
pemphigoid antigen
1 (230 kDaltons)
2 (180 kDaltons)
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Pemphigus vulgarisintraepidermal acantholytic blister, suprabasal
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Pemphigus vulgaris
direct immunofluorescence
epidermal intercellularIgG
C3
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Pemphigus vulgaris
Middle age 40-50y
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Pemphigus vulgaris- Nikolski sign
direct indirect
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Pemphigus vulgaris
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Pemphigus vulgaris
mucosal involvement
to 90 % of patients
In about 70% of cases
starts with mucosal
involvement
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Pemphigus - therapy
First choice: Systemic Corticosteroidshigh dose(prednisone p.o. 100mg/die), iv pulse therapy
Before the era of corticosteroids - 75% mortality in
one year after the onset, in 5 years all diedOther:
Immunosupressive: azathioprine, cyclophosphamide,
methotrexate, mycophenolate mofetil,Antiinflammatory: gold, antimalarics, sulphones
Immunomodulation: iv. Immunoglobulins,
plasmapheresis
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Pemphigus vegetans
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Pemphigus vegetans
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Doc. Cetkovsk, Kon klinika FN Plze
Pemphigus vegetans
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Pemphigus foliaceus
Antibodies to desmoglein 1
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Pemphigus seborrheic/erythematosusAnti desmoglein-1 antibody
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Pemphigus foliaceus-Anti desmoglein-1 antibody
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Bullous pemphigoid
Elderly persons > 60 y
Incidence is increasing withage
Antibodies to hemidesmosomal
proteins:
anti bullous pemphigoid antigen
(BPAg) 1 (230 kDaltons)
and
Anti-BPAg 2 (180 kDaltons)
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Bullous pemphigoidsubepidermal blister,
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Bullous pemphigoiddirect immunofluorescence
linear along epidermal basal membrane IgG, C3
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Bullous pemphigoid
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Bullous pemphigoid
Oral involvement < 20% cases
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First choice:
Systemic Cortisteroidsmedium dose
(prednisone 40-60mg/die)
Other:
tetracyklin, dapson, potent local corticosteroids
Immunosupressive:
azathioprine, cyclophosphamide, methotrexate,
mycophenolate mofetil,
Bullous pemphigoidtherapy
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Pemphigoid (herpes) gestationis
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Cicatricial pemphigoid
Ci t i i l hi id
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Cicatricial pemphigoid
Antiodies to hemidesmosomes, laminin 5, 64 integrin
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Cicatricial pemphigoid
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Cicatricial pemphigoid
scarring
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Cicatricial pemphigoid
Dermatitis herpetiformis Duhring
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Dermatitis herpetiformis Duhring
Men : women = 2:1, main onset 10-30 years of age
Pathogenesis: Relation to gluten sensitive enteropathyin both -
abnormal immune reaction to gluten antigens (substance of many grains
most sensitising is gliadinsubstrate of tissue transglutaminase)antibodies agains tissue transglutaminase cross-react with similar
epidermal TG producing skin lesion, lesser
to jejunal mucosa
and GIT symptoms
HLA association
90% HLA-DQ2
10% HLA-DQ8
Disease association:
Diabetes mellitus,
vitiligo, pernicious
anemia, thyreopathy,
Subepidermal blister
Microabscess
with neutrophils
Dermatitis herpetiformis Duhring
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Dermatitis herpetiformis Duhring
Direct immunofluorescencegrainy IgAin papillas of dermis
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Dermatitis herpetiformis Duhring
Pruritic vesicles
arranged in herpetiform fashion
Vesicle at the periphery of
Figurate erythematouspatches
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Dermatitis herpetiformis Duhring
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Dermatitis herpetiformis Duhring
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Dermatitis herpetiformis Duhring
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Dermatitis herpetiformis Duhring - therapy
Diet
gluten-free diet (protects against increased frequency of
gastrointestinal B lymphoma, malababsorption
syndromes) exclusion of food with high content of iodine (seafood)
Drug of choice
diaminodifenylsulfon = dapson
very quick effect (1-2 days)
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Pemphigus seborrheic/erythematosus
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Pemphigus seborrheic/erythematosus
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Pemphigus foliaceus
Dermatitis herpetiformis Duhring
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Dermatitis herpetiformis Duhring
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