European Guidelines (S1) on the use of high‐dose intravenous immunoglobulin in dermatology

Enk, Alexander; Hadaschik, Eva; Eming, Rüdiger; Fierlbeck, Gerhard; French, Lars E.; Girolomoni, Giampiero; Hertl, Michael; Jolles, Stephen; Kárpáti, Sarolta; Steinbrink, Kerstin; Stingl, Georg; Volc‐Platzer, Beatrix; Zillikens, Detlef

doi:10.1111/jdv.13725

Cited by 55 publications

(85 citation statements)

References 44 publications

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“…IVIG is thought to prevent Fas‐mediated apoptosis of keratinocytes; however, recent meta‐analyses have concluded that the use of IVIG is equivocal in its benefit, specifically with nine studies showing no survival benefit . However, current European guidelines support the use of IVIG in the absence of alternative evidence‐based therapies . If utilized, IVIG administered at a high dose (total dose ≥2 g/kg) has been shown to decrease mortality compared to low‐dose IVIG .…”

Section: Discussionmentioning

confidence: 99%

Stevens‐Johnson syndrome and toxic epidermal necrolysis: retrospective review of 10‐year experience

et al. 2019

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Background Stevens‐Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous disorders. To date, relatively few studies have looked at institutional approaches to treatment of SJS/TEN, particularly with a focus on wound care. Methods A retrospective review was conducted on patients admitted to the Hennepin County Medical Center from 2007 to 2017 with a final diagnosis of SJS or TEN. Data were obtained for demographics, causative drug, hospital course, supportive care, medical management, complications, and disposition. Results A total of 48 were diagnosed with SJS/TEN during the study period. A total of 41.7% (20/48) were men, and the mean age was 49.2 years. Sulfa antibiotics and nonsulfa antibiotics were the most common causative drug categories, each accounting for a quarter of cases. Supportive measures included intravenous fluid resuscitation in 4.2% of cases, enteral nutrition in 75%, surgical debridement in 27.1%, and porcine xenograft in 16.7%. Wound care consisted of use of a cleanser in 95.8% of patients, topical antibiotic in 95.8%, topical steroid in 20.8%, topical antifungal in 14.6%, emollient in 83.3%, nonadherent dressing in 97.9%, silver impregnated dressing in 39.6%, nonsilver impregnated dressing in 79.2%, and general wrap in 93.8%. For medical treatment, 64.6% of patients received intravenous immunoglobulin (IVIG), and 8.3% of patients received cyclosporine. Mortality rate was 12.5% overall, compared to an expected mortality rate of 25.2% as predicted by SCORTEN. Conclusions Patients treated with our current regimen of care showed a mortality rate half of that predicted by SCORTEN.

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Section: Discussionmentioning

confidence: 99%

Stevens‐Johnson syndrome and toxic epidermal necrolysis: retrospective review of 10‐year experience

et al. 2019

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“…Therefore, while IVIg has previously been an accepted therapeutic agent in TEN, its efficacy is becoming more questionable. Despite these studies, the current European guidelines on the use of IVIg in dermatology have suggested that it should be used early in the disease process in the absence of an alternative evidence-based therapeutic alternative [43], given that the potential benefits of high-dose IVIg outweigh the risks of the medication and the disease’s natural course.…”

Section: Managementmentioning

confidence: 99%

Review of Toxic Epidermal Necrolysis

Harris

Jackson

Cooper

2016

IJMS

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Toxic epidermal necrolysis (TEN) is a rare but life threatening mucocutaneous reaction to drugs or their metabolites. It is characterised by widespread keratinocyte apoptosis and sloughing of the skin, erosions of the mucous membranes, painful blistering, and severe systemic disturbance. The pathophysiology of TEN is incompletely understood. Historically, it has been regarded as a drug-induced immune reaction initiated by cytotoxic lymphocytes via a human leukocyte antigen (HLA)-restricted pathway. Several mediators have been identified as contributors to the cell death seen in TEN, including; granulysin, soluble Fas ligand, perforin/granzyme, tumour necrosis factor-α (TNF-α), and TNF-related apoptosis-inducing ligand. Currently, granulysin is accepted as the most important mediator of T cell proliferation. There is uncertainty around the accepted management of TEN. The lack of definitive management guidelines for TEN is explained in part by the rarity of the disease and its high mortality rate, which makes it difficult to conduct randomised control trials on emerging therapies. Developments have been made in pharmacogenomics, with numerous HLA alleles identified; however, these have largely been ethnically specific. These associations have translated into screening recommendations for Han Chinese.

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“…Management of patients with autoimmune diseases, requiring immunosuppressive treatment and concurrent or recent history of malignancy may be challenging and these patients are inappropriate for immunosuppressive treatment options (Enk et al, ). The relationship between cancer and autoimmunity makes IVIG treatment reasonable.…”

Section: Discussionmentioning

confidence: 99%

Intravenous immunoglobulin in resistant livedoid vasculopathy: Analysis of a case series

Özden

Özdemir

Şentürk

2020

Dermatologic Therapy

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We report a retrospective analysis the efficacy of high‐dose intravenous immunoglobulins on 9 patients with Livedoid vasculopathy for whom resistant to immunosuppressants plus anticoagulants or could not be prescribed. Intravenous immunoglobulins were used 2 g/kg per month in these patients. The treatment induced stabilization of the disease and all patients demonstrated improvement with IVIg. Complete and partial clinical response was achieved in 6 and 3 patients respectively.

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European Guidelines (S1) on the use of high‐dose intravenous immunoglobulin in dermatology

Abstract: Background The treatment of severe dermatological autoimmune diseases and toxic epidermal necrolysis (TEN) with

Cited by 55 publications

References 44 publications

Stevens‐Johnson syndrome and toxic epidermal necrolysis: retrospective review of 10‐year experience

Stevens‐Johnson syndrome and toxic epidermal necrolysis: retrospective review of 10‐year experience

Review of Toxic Epidermal Necrolysis

Intravenous immunoglobulin in resistant livedoid vasculopathy: Analysis of a case series

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