Epidermolysis bullosa acquisita.

نویسندگان

N R Dar

A Hameed

A A Khan

چکیده

Autoimmunity to type-WI collagen is characterized by autoantibodies predominantly of lgG class to the non-collagenous domain of type-WI collagen present in the anchoring fibrils which bind basement membrane lamina densa to the anchoring plaques in the dermis. This results in a sublami.na densa split with a blister formation. Type WI collagen autoim.munity is heterogenous in its clinical spectrum presenting as epiderrnaolysis bullosa acquisite (EBA) or a subtype of bullous lupus eiythematosus. . EBA, the commoner of the presentations, was first described by Elliot in 1895. In its classical form it is characterized by increased skin fragility and non-inflammatory blisters that heal with scarring and milia, occurring predominantly over the trauma prone sites.In addition to its classical form it may clinically mimic bullous pemphigoid or cicatricial pemphigoid. Histologically there is a subepidermal blister with a variable infiltrate. Direct immunoflourescence (DIF) and indirect immunofluorescence (1W) of perilesional salt split skin (SSS), or the substrate respectively demonstrate linear deposition of IgG and sometimes IgA and IgM on the dermal side of the blister. Immunoelectron microscopy shows the imrnunoreactants to be in the subla.mina densa zone. We describe a case of EBA whom we believe is the first confirmed case from Pakistan.

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