Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita (EBA) is a subtype of pemphigoid that may present with scarring similar to hereditary dystrophic epidermolysis bullosa, of which the naming of EBA was derived. The clinical subtype of EBA with scarring is named mechanobullous EBA, because blisters are evoked by sudden mechanical trauma to the skin. The other subtype of EBA with erythematous lesions without scarring is named inflammatory EBA, and may look like bullous pemphigoid. The mucous membranes can be involved in both subtypes. The pathogenesis is mediated by IgG or IgA against type VII collagen, which is the component of anchoring fibrils below the lamina densa. Diagnosis is confirmed by detection of a u-serrated linear pattern of immune depositions by direct immunofluorescence microscopy of a skin biopsy. The pathogenesis of both clinical subtypes is unknown, and is not related to binding of a particular epitope of the auto-antigen. EBA is associated with systemic lupus erythematosus and colitis ulcerosa. The disease is relative refractory to treatment.