epidermolysis bullosa acquisita Flashcards
Achronicsubepidermalbullousdiseaseassociatedwithautoimmunitytoth
type 7 collagen
what type of eba…. is a nonin ammatory, blistering eruption with acral distribution that heals with scarring and milia ormation. It is a mechanobullous disease marked by skin
ragility.Scarsintraumatizedregionssuchasthedorsao thehands,knuckles,elbows,knees,sacral area, and toes. Resembling porphyria cutanea tarda (see Section 10) or hereditary epidermolysis bullosa.
classic mechanobullous presentation
Widespreadin ammatoryvesiculobullouseruptionassoci- atedwitherythematousorurticarialskinlesionsinvolvingthetrunk,skin olds,andinadditionthe extremities
Bullouspemphigoid–likepresentation
hasprominentmucosalinvolvement—erosionsandscarring in the mouth, esophagus, conjunctiva, anus, and vagina
Cicatricialpemphigoid–likepresentation
shows vesicles arranged in an annular ashion, reminiscento linearIgAbullousdermatosis,DH,orCBDC
The linear IgAbullous dermatosis–like presentation
histo
Subepidermal blisters
IP
linearIgG(plusIgA,gM, actorB,andproperdin)atthedermal–epidermaljunction.
■ Antibodiesinserabindtoa290-kDabandinWesternblotscontainingtypeVIIcollagen.ELISA
speci c or antibodies to type VIIcollagen.
tx
SomeEBApatientsimproveondapsoneandhigh doseso colchicine.Supportivetherapy