Epidermolysis bullosa acquisita: Efficacy of high-dose intravenous immunoglobulins

Meier, Friedegund; Sönnichsen, Karsten; Schaumburg-Lever, Gundula; Dopfer, R.; Rassner, G.

doi:10.1016/0190-9622(93)70189-z

Cited by 70 publications

(49 citation statements)

References 19 publications

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“…Within 4 weeks after initiation of this therapy, a rapid clinical remission was achieved (d). This was accompanied by a reduction of circulating anti-Dsg 3 autoantibodies from 1,213 to 102 been treated with IVIG [24, 28, [62][63][64][65][66][67][68][69]. The response was usually favorable, but further data are needed to establish the real potential of IVIG in EBA (Table 1).…”

Section: Subepidermal Blistering Diseasesmentioning

confidence: 90%

High-Dose Intravenous Immunoglobulin (IVIG) Therapy in Autoimmune Skin Blistering Diseases

Ishii

Hashimoto

Zillikens

et al. 2009

Clinic Rev Allerg Immunol

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Treatment of autoimmune bullous skin diseases can often be challenging and primarily consists of systemic corticosteroids and a variety of immunosuppressants. Current treatment strategies are effective in most cases but hampered by the side effects of long-term immunosuppressive treatment. Intravenous immunoglobulin (IVIG) is one potential promising therapy for patients with autoimmune bullous skin diseases, and evidence of its effectiveness and safety is increasing. A number of autoimmune bullous skin diseases have been identified in which IVIG treatment may be beneficial. However, experience with IVIG in patients with autoimmune skin blistering disease is limited, where it is recommended for patients not responding to conventional therapy. The mode of action of IVIG in autoimmune diseases, including bullous diseases is far from being completely understood. We here summarize the clinical evidence supporting the notion, that IVIG is a promising therapeutic agent for the treatment of patients with autoimmune bullous skin disease. In addition, we review the proposed modes of action. In the future, randomized controlled trials are necessary to better determine the efficacy and adverse effects of IVIG in the treatment of autoimmune bullous skin diseases. In addition, insights into IVIG's mode of action might enable us to develop novel therapeutics to overcome the current shortage of IVIG.

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Section: Subepidermal Blistering Diseasesmentioning

confidence: 90%

High-Dose Intravenous Immunoglobulin (IVIG) Therapy in Autoimmune Skin Blistering Diseases

Ishii

Hashimoto

Zillikens

et al. 2009

Clinic Rev Allerg Immunol

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“…132 In one case, high-dose IVIg (Polyglobin, 400mg/kg/d intravenously for 4 days every 2 weeks for 4 courses) was used in addition to cyclosporine and prednisolone, and resulted in decreased blister formation. 148 A similar dose induced a 5-month remission in a patient after 9 cycles. 149 Lower-dose IVIg (40 mg/kg intravenously for 5 days every 3 to 4 weeks for 7 cycles) resulted in diseasefree remission for more than 10 months after multiple immunosuppressive drugs and plasma exchange failed in a patient.…”

Section: Management Of Epidermolysis Bullosa Acquisitamentioning

confidence: 97%

Management of autoimmune bullous diseases: Pharmacology and therapeutics

Mutasim

2004

Journal of the American Academy of Dermatology

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“…Intravenous immunoglobulin has been reported to be effective in some patients with EBA [54]. The mechanism by which gamma globulin might invoke a positive response in EBA is unknown.…”

Section: Treatmentmentioning

confidence: 98%

Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita

Woodley

Remington

Chen

2007

Clinic Rev Allerg Immunol

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Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune, mechanobullous disease with clinical features reminiscent of genetic dystrophic epidermolysis bullosa (DEB). EBA patients have skin fragility, blisters, scars, and milia formation. DEB is due to a genetic defect in the gene-encoding type VII collagen, which makes anchoring fibrils, structures that attach the epidermis and its underlying basement membrane zone onto the papillary dermis. DEB patients have a decrease in normally functioning anchoring fibrils. EBA patients have the same problem, but their decrease in normally functioning anchoring fibrils is because of an abnormality in their immune system in which they produce anti-type VII collagen antibodies that attack their anchoring fibrils. These IgG anti-type VII collagen antibodies are "pathogenic" because when injected into a mouse, the mouse develops an EBA-like blistering disease. EBA has several distinct clinical presentations. It can present with features similar to DEB. It can also present with features reminiscent of bullous pemphigoid, cicatricial pemphigoid, Brunsting-Perry pemphigoid, or IgA bullous dermatosis. Treatment for EBA is unsatisfactory. Some therapeutic success has been reported with colchichine, dapsone, photopheresis, infliximab, and IVIG.

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Epidermolysis bullosa acquisita: Efficacy of high-dose intravenous immunoglobulins

Cited by 70 publications

References 19 publications

High-Dose Intravenous Immunoglobulin (IVIG) Therapy in Autoimmune Skin Blistering Diseases

High-Dose Intravenous Immunoglobulin (IVIG) Therapy in Autoimmune Skin Blistering Diseases

Management of autoimmune bullous diseases: Pharmacology and therapeutics

Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita

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