Epidermolysis bullosa acquisita

Synonym(s)

Acquired epidermolysis bullosa; dermolytic pemphigoid

Definition

Chronic autoimmune blistering disease, with lesions often occurring at sites of trauma

Pathogenesis

IgG autoantibodies specific for anchoring fibrils (type VII collagen) of the skin basement membrane causes an inflammatory process which is a contributing factor to blister formation; skin trauma a contributing factor; genetic factors possibly important, since HLA-DR2 is overrepresented in those with this condition

Clinical manifestation

Non-inflammatory bullae at sites of minor skin trauma, which heal with scars and/or milia; widespread inflammatory bullae not related to trauma; mucous membrane blisters and erosions, leading to scarring

Differential diagnosis

Epidermolysis bullosa; bullous pemphig-oid; cicatricial pemphigoid; linear IgA bul-lous dermatosis; bullous lupus erythemato-sus; porphyria cutanea tarda; bullous disease of diabetes mellitus; erythema multiforme

Therapy

Prednisone 1 mg per kg PO daily*; corticosteroid sparing agents - azathioprine; methotrexate; mycophenolate mofteil; cyclophosphamide; dapsone

References

Kirtschig G, Murrell D, Wojnarowska F, et al. (2002) Interventions for mucous membrane pemphigoid/cicatricial pemphigoid and epidermolysis bullosa acquisita: A systematic lit

214 Epidermolysis bullosa dystrophica erature review. Archives of Dermatology 138:380-384

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