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

doi:10.4172/2155-9554.1000200

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.

Highlights

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
Indirect immunofluorescence (IIF) demonstrated circulating IgG anti-basement membrane zone (BMZ) antibodies at a titer of 1:160, which bound to the dermal side of 1M NaCl-split normal human skin
Gradual increase in levels of liver transglutaminases led to cessation of dapsone. Because both pruritus and skin lesions were refractory to subsequent oral prednisolone (40 mg/day) or betamethasone (6 mg/day), double filtration plasmapheresis (DFPP) was performed

Summary

Introduction

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. Indirect immunofluorescence (IIF) demonstrated circulating IgG anti-BMZ antibodies at a titer of 1:160, which bound to the dermal side of 1M NaCl-split normal human skin (data not shown). Immunoblot (IB) analysis of normal human dermal extracts revealed that IgG antibodies in the patient serum reacted with a 290-kDa protein band with the same mobility as an epidermolysis bullosa acquisita (EBA) antigen (type VII collagen; Figure 2d).

Results

Conclusion