Autoimmune bullous diseases
nIntraepidermal – pemphigus
nSubepidermal – pemphigoid group, DHD
n
nDiagnostics – (Tzanck test), histology, immunofluorescence direct & indirect, ELISA, immunoblot

111-1160_IMG



Pemphigus
nrare bullous disorder with autoantibody-induced intraepidermal blisters  (desmosomes -
acantholysis)
nchronic disease, can be lethal
naverage age 40-60 years
ndrug induced
nparaneoplastic pemphigus
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pemph-fol-jedl-fitc-igk-40x--saveim 129-2942_IMG



Pemphigus
nSuprabasal – pemphigus vulgaris
n                          vegetans
nSuperficial – pemphigus foliaceus
n                        fogo selvagem
n                        drug induced (thiol group)
n                        pemphigus erythematosus
n
n

pemphigus-vulgaris-zada pemphigus-vulgaris-obl1-u pemphigus-vulg-detail pemfigus-vulgaris-hyzde



Pemphigus vegetans
n
pv-klis-axila pemphigus -veg-trislo

pemphigus-erythematosus-zada Pvegnavel pemphvegpust



Pemphigus
nTherapy
nCorticosteroids (Prednisone 1-1,5mg/kg/d)
nImmunosuppresives – mycophenolate                mofetil
n                                    - Cyclophosphamide
n                                     - Azathioprine
nRituximab   anti CD20 antibody
nIVIG
nImmunoadsorption

Pemphigoid bullosus
nRelatively rare disorder with autoantibody-induced subepidermal blisters (basement membrane –
hemidesmosomes)
nBullous pemphigoid
nCicatricial pemphigoid
nEpidermolysis bullosa acquisita
nIgA linear dermatosis
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Pemphigoid bullosus
nElder people – over 70 years
nParaneoplastic disease (13%)
nDrug induced (gliptins, ACE inhibitors)
nMany concomittant diseases
nNeurodegenerative diseases! (40%)

orságBPtělo šustBP



123-2328_IMG pemphigoid-bullosus-paze



Pemphigoid bullosus
nTherapy
nCorticosteroids (Prednisone 40-60mg)
nImmunosuppresives ( azathioprine, methotrexate)
nDapsone
nPotent topical corticosteroids

Cicatricial pemphigoid
nMucose membranes
nConjunctiva                  blindness
nPharynx, larynx, genital area             stenosis, scarring

jizvící-pemfigoid-buk-sliz jizvici-pemfigoid-gingivitis 122-2264_IMG



Dermatitis herpetiformis Duhring
n gluten sensitive entheropathy – coeliakia
n IgA antibodies against endomysium (tissue transglutaminase)
ncross reaction with eTG on reticulin fibers in dermal papilae
nSensitivity to gluten, iodine
n

Dermatitis herpetiformis Duhring
nYoung adults
nHLA DQ2 association
nTherapy
nGluten free diet
nDapsone
nTopical corticosteroids
n
n
n

dhd-detail dermatitis-herpetiformis-hyzde