engl bulózní dermatózy pro mediky štork

7/30/2019 engl bulzn dermatzy pro mediky tork

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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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Antiodies to hemidesmosomes, laminin 5, 64 integrin


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scarring


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Dermatitis herpetiformis Duhring


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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



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Direct immunofluorescencegrainy IgAin papillas of dermis


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Pruritic vesicles

arranged in herpetiform fashion

Vesicle at the periphery of

Figurate erythematouspatches


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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



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engl bulózní dermatózy pro mediky štork