Epidermolysis bullosa acquisita

Olivia Y. Lai, Michael Kasperkiewicz, Brittney K. DeClerck, Mei Chen, David T. Woodley


Epidermolysis bullosa acquisita (EBA) is a blistering skin disease that is caused by an autoantibody to type VII collagen (C7). C7 is the main component of large structures called anchoring fibrils localized at the dermal-epidermal junction (DEJ) of skin and critical for epidermal - dermal adherence. Classically, EBA presents as a mechanobullous disease with skin fragility and scarring reminiscent of hereditary dystrophic epidermolysis bullosa (DEB) or porphyria cutanea tarda. Nevertheless, if the disease is defined as autoimmunity to type VII collagen, EBA can have clinical presentations that are reminiscent of inflammatory bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), and Brunsting-Perry pemphigoid. Very rarely, EBA involving IgA autoantibodies to C7 presents clinically similar to linear IgA bullous dermatosis (LABD) with small pruritic symmetrical papulovesicles. Anecdotal reports have linked EBA to several underlying systemic diseases with the most common being inflammatory bowel disease (IBD) and systemic bullous erythematosus (SLE). Interestingly, in the gastrointestinal track there is an anchoring fibril equivalent composed of C7, and patients with Crohn's Disease and other IBDs have anti-C7 antibodies in their plasma. EBA is diagnosed using saltsplit skin indirect and direct immunofluorescence (IIF and DIF), ELISA or Western blot analysis to detect anti-C7 antibodies in the blood, and, at research centers, by immuno-electron microscopy. EBA is notoriously difficult to treat. Systemic steroids and immunosuppressant agents (azathiaprine, mycophenolate mofetil, cyclophosphamide, and methotrexate) are inconsistently effective. Colchicine and cyclosporine may be helpful, but the former cannot be used in patients who have concomitant IBD and the latter requires very high doses. Other treatments with varying success include dapsone, photophoresis, IVIG and plasmapheresis. Recently, rituximab, an anti-CD20 monoclonal antibody, has shown promise in EBA.

TitelAutoimmune Bullous Diseases: Approach and Management
Herausgeber (Verlag)Springer International Publishing
ISBN (Print)9783319267265
ISBN (elektronisch)9783319267289
PublikationsstatusVeröffentlicht - 01.01.2016


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