epidermolysis bullosa acquisita Flashcards

1
Q

Achronicsubepidermalbullousdiseaseassociatedwithautoimmunitytoth

A

type 7 collagen

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2
Q

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.

A

classic mechanobullous presentation

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3
Q

Widespreadin ammatoryvesiculobullouseruptionassoci- atedwitherythematousorurticarialskinlesionsinvolvingthetrunk,skin olds,andinadditionthe extremities

A

Bullouspemphigoid–likepresentation

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4
Q

hasprominentmucosalinvolvement—erosionsandscarring in the mouth, esophagus, conjunctiva, anus, and vagina

A

Cicatricialpemphigoid–likepresentation

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5
Q

shows vesicles arranged in an annular ashion, reminiscento linearIgAbullousdermatosis,DH,orCBDC

A

The linear IgAbullous dermatosis–like presentation

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6
Q

histo

A

Subepidermal blisters

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7
Q

IP

A

linearIgG(plusIgA,gM, actorB,andproperdin)atthedermal–epidermaljunction.
■ Antibodiesinserabindtoa290-kDabandinWesternblotscontainingtypeVIIcollagen.ELISA
speci c or antibodies to type VIIcollagen.

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8
Q

tx

A

SomeEBApatientsimproveondapsoneandhigh doseso colchicine.Supportivetherapy

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