Successful Treatment of Intravenous Immunoglobulins in a Patient with Intractable Epidermolysis Bullosa Acquisita with Autoantibodies to Type VII Collagen and Laminin Alpha-3

Shin-Ichi Osada, Rui Yoshid

Abstract

Epidermolysis bullosa acquisita (EBA) is a blistering disease caused by autoantibodies to type VII collagen, a major component of anchoring fibrils at the dermal-epidermal junction. Here, we report a case of inflammatory EBA with a unique antibody prolife showing reactivity to laminin alpha-3 as well as type VII collagen. The patient’s cutaneous lesions were refractory to dapsone, prednisolone, betamethasone, and double filtration plasmapheresis, which led to a catheter-mediated methicillin-resistant staphylococcal aureus (MRSA) sepsis. Intravenous immunoglobulins (IVIG) initially used to resolve MRSA sepsis improved the pruritus and skin manifestations of EBA, and clinical remission of EBA was achieved after only two cycles of IVIG. The mechanism for the concurrence of antibodies to type VII collagen and laminin alpha-3 and the potential mode of action of IVIG in EBA are discussed.