Pemphigoid Flashcards
Whats the target auf auto-ab in pemphigoid?
hemidesmosomal proteins (BP180, BP230, a6b4 integrin, laminin 322, lamining1, type 7 collagen)
Name the subtypes of pemphigoid
Bullous pemphigoid, linear IgA disease, Mucus membrane pemphigoid, P. gestationis, Anti-p200/laminin g1 P., EBA
Where are the splits formed and what kind of blisters occur in Pemphigoid?
Subepidermal, tense blisters
Median age of onset in Pemphigoid?
75-80
Incidence and mortality of BP
20/1. Mio/year (prominent heterogeneity in distribution of cases)
1-year mortality 15-40%
BP is associted with …
hematological malignancies, no association for solid tumors for BP, but for MMP, EBA
Whats important for the diagnosis of pemphigoid?
Clinical criteria direct IF microscopy serology histopathology detection of tissue-bound and circulating ab
Ab and symptoms of BP
tense blisters (on erythematous skin) erosions and haemorrhagic crusts, mild oral lesions in 10-20%
Ag: BP180 (BP230)
Ab and symptoms in Pemphigoid gestationis
in 2nd/3rd trimester of pregnancy or immediatly after
large, itchy, inflammatory plaques and subepithelial blisters
Ag BP180 NC16A
Ab and symptoms in MMP
oral cavity, conjunctivae, skin, nasal cavity, oesophagus
Ag BP180 and others
Ab and symptoms in linear IgA disease
Most frequent autoimmune blistering disease in children
crown of jewel around blisters
Ag BP180 (LAD1, IgA reactivity), BP230
Ab and symptoms Anti-p200/laminin g1 pemphigoid
Ag p200, laminin g1
AB and symptoms in EBA
Mucosal involvement in about 60%
Ag classical: collagen type 7
Differences in IF microscopy in pemphigoid
U-serrated pattern: EBA
N-serrated pattern: others pemphigoids
