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Use of Intravenous Immunoglobulin in Toxic Epidermal Necrolysis

"In a Patient With Toxic Epidermal Necrolysis, ...?"

Laurence Feldmeyer, MD, PhD; Francisco A. Kerdel, MD; Lars E. French, MD Arch Dermatol. 2011;147(12):1440-1441.

Toxic epidermal necrolysis (TEN) is a rare, life-threatening, adverse cutaneous drug reaction with a reported mortality rate ranging from 25% to 35%.1

1.  Based on the reported inhibition of Fas-mediated apoptosis of keratinocytes by intravenous (IV) immunoglobulin (IVIg) in vitro, IVIg has since been considered as a potential therapeutic modality in TEN.

2. To date, 15 studies including more than 10 patients per study and analyzing the effect of IVIg in TEN have been reported in the literature. They all suffer their imperfect study designs and limitations, and none are of a randomized, controlled design.

The problem with TEN to date is that a specific therapy for Stevens-Johnson syndrome/TEN that has shown efficacy in controlled clinical trials unfortunately does not exist.

In this issue of the Archive s, Wootton et al3 illustrate perfectly the practice gap encountered by dermatologists and . . . [Full Text of this Article]

 

Cover PDF In a Patient With Toxic Epidermal Necrolysis, Does Intravenous Immunoglobulin Improve... Wootton et al. Arch Dermatol.2011; 147: 1437-1440.

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Vol. 147 No. 12, December 2011

Use of Intravenous Immunoglobulin in Toxic Epidermal Necrolysis Comment on "In a Patient With Toxic Epidermal Necrolysis, Does Intravenous Immunoglobulin Improve Survival Compared With Supportive Care?" Laurence Feldmeyer, MD, PhD; Francisco A. Kerdel, MD; Lars E. French, MD Arch Dermatol. 2011;147(12):1440-1441. doi:10.1001/archdermatol.2011.1610

Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. Toxic epidermal necrolysis (TEN) is a rare, life-threatening, adverse cutaneous drug reaction with a reported mortality rate ranging from 25% to 35%.

1 Based on the reported inhibition of Fas-mediated apoptosis of keratinocytes by intravenous (IV) immunoglobulin (IVIg) in vitro, IVIg has since been considered as a potential therapeutic modality in TEN.

2 To date, 15 studies including more than 10 patients per study and analyzing the effect of IVIg in TEN have been reported in the literature. They all suffer their imperfect study designs and limitations, and none are of a randomized, controlled design. The problem with TEN to date is that a specific therapy for Stevens-Johnson syndrome/TEN that has shown efficacy in controlled clinical trials unfortunately does not exist.

Use of Intravenous Immunoglobulin in Toxic Epidermal Necrolysis Archives of Dermatology FULL TEXT | WEB OF SCIENCE | PUBMED 11. Yang Y, Xu J, Li F, Zhu X.

Combination therapy of intravenous immunoglobulin and corticosteroid in the treatment of toxic epidermal necrolysis and Stevens-Johnson syndrome: a retrospective comparative study in …

Title: Stevens-Johnson syndrome and toxic epidermal necrolysis: efficacy of intravenous immunoglobulin and a review of treatment options.

Published: 2009 Jan in Singapore medical journal

Patients:6

Demographics:Adult, 19 to 44 Aged, 65 to 79 Aged, 80 and older Middle Aged, 45 to 64

Conditions:Lyell's Syndrome, Stevens-Johnson Syndrome

Treatments:Cyclosporine (Sandimmune)Drug Administration Schedule Immunoglobulins, Intravenous (Gammagard) Institution:Changi General HospitalExperts:Kwok, CLiu, T TTay, Y KTeo, L Full Abstract

Conditions Treatments Experts Symptoms Demographics Toxic epidermal necrolysis (TEN) is a rare, severe adverse drug reaction.

Steven-Johnson syndrome (SJS) represents the milder end of the spectrum. The exact pathogenesis of TEN and SJS is still unknown and many drugs, including prednisolone, cyclosporin and intravenous immunoglobulin (IVIG), have been used in an attempt to halt the disease process. The use of IVIG in particular is controversial. We share our experience with the use of IVIG in six patients with TEN. We will also review the various proposed mechanisms underlying TEN, the mechanism of action of IVIG in TEN and summarise useful treatment options.

 

Outcome: This anti-apoptotic action of IVIG helps explain how IVIG works in severe, life threatening dermatologic conditions that are resistant to traditional systemic treatments, such as toxic epidermal necrolysis and Stevens-Johnson syndrome.

Thus, the actions of IVIG are varied and complex, and the primary mechanisms of action may be different in different diseases.

Title:Novel mechanism for therapeutic action of IVIG in autoimmune blistering dermatoses.Published: 2008 in Current directions in autoimmunity

Conditions:Autoimmune Disease

Lyell's Syndrome

Stevens-Johnson Syndrome

Treatments: Immunoglobulins, Intravenous (Gammagard)Treatments Institution: University of California

Experts:Grando, Sergei A Michael, Daniel Full Abstract

The mode of action of intravenous immunoglobulin (IVIG) is complex. An ongoing research continues to elaborate and identify novel mechanisms. Recent advances have demonstrated that IVIG has direct effect on keratinocytes, the target cells of autoimmune blistering diseases. IVIG protects keratinocytes from pathogenic autoantibodies by preventing the autoantibody-induced of apoptosis and oncosis. This anti-apoptotic action of IVIG helps explain how IVIG works in severe, life threatening dermatologic conditions that are resistant to traditional systemic treatments, such as toxic epidermal necrolysis and Stevens-Johnson syndrome. Thus, the actions of IVIG are varied and complex, and the primary mechanisms of action may be different in different diseases.

 

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis are the most severe cutaneous reactions that occur in children.

Off-label use of human intravenous immunoglobulin (IVIG) has been reported in a number of autoimmune and cell-mediated blistering disorders of the skin, including severe cutaneous drug reactions.

We review 28 previous reports in which IVIG was used in pediatric patients with SJS and toxic epidermal necrolysis and discuss our experience in 7 children with SJS, in whom no new blisters developed within 24 to 48 hours after IVIG administration and rapid recovery ensued.IVIG seems to be a useful and safe therapy for children with severe cutaneous drug reactions.

Well-controlled, prospective, multicenter clinical trials are needed to determine optimal dosing guidelines and to compare the efficacy and safety of IVIG with other potentially effective modalities.

Title: Use of intravenous immunoglobulin in children with stevens-johnson syndrome and toxic epidermal necrolysis: seven cases and review of the literature.

Published: 2003 Dec in Pediatrics Patients:7

Demographics: Child, 6 to 12 Infant, 1 to 23 months Adolescent, 13 to 18 Conditions: Lyell's Syndrome

Stevens-Johnson Syndrome

Treatments:Immunoglobulins, Intravenous (Gammagard)Off-Label Use

Institution:Texas Children's Hospital

Experts:Jung, PeterLevy, Moise LMetry, Denise W

Full Abstract Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis are the most severe cutaneous reactions that occur in children. Off-label use of human intravenous immunoglobulin (IVIG) has been reported in a number of autoimmune and cell-mediated blistering disorders of the skin, including severe cutaneous drug reactions.

We review 28 previous reports in which IVIG was used in pediatric patients with SJS and toxic epidermal necrolysis and discuss our experience in 7 children with SJS, in whom no new blisters developed within 24 to 48 hours after IVIG administration and rapid recovery ensued. IVIG seems to be a useful and safe therapy for children with severe cutaneous drug reactions.

Well-controlled, prospective, multicenter clinical trials are needed to determine optimal dosing guidelines and to compare the efficacy and safety of IVIG with other potentially effective modalities.