20 April 2020
: Case report
Epidermolysis Bullosa Acquisita: A Case Report
Challenging differential diagnosis, Rare disease
Erik A. Kumetz1BCDEF*, Jon H. Meyerle2DEF, Shayna C. Rivard3ACDEFDOI: 10.12659/AJCR.919432
Am J Case Rep 2020; 21:e919432
Abstract
BACKGROUND: Epidermolysis bullosa acquisita is a rare, subepithelial bullous disorder, which is distinguished from other autoimmune blistering diseases by the production of antibodies against type VII collagen.
CASE REPORT: Here, we describe the case of a 79-year-old male resident of the Northern Mariana Islands who presented to the clinic with multiple blistering skin lesions.
CONCLUSIONS: The primary focus of treatment is to prevent disease progression and serious complications of scarring (including blindness and respiratory obstruction) by avoiding physical trauma and suppressing the immune systems with agents, including corticosteroids, colchicine, dapsone, methotrexate, and cyclophosphamide. Successful treatment of the condition should involve a multidisciplinary team of medical professionals with regular monthly follow-ups during periods of active disease.
Keywords: Collagen Type VII, Epidermolysis Bullosa Acquisita, Micronesia, Abscess, Aged, Anti-Inflammatory Agents, Autoimmune Diseases, Prednisone, Staphylococcal Infections
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