High‐Dose Intravenous Immunoglobulins in the Treatment of Toxic Epidermal Necrolysis: An Asian Series
59
Citation
15
Reference
10
Related Paper
Citation Trend
Abstract:
Abstract Toxic epidermal necrolysis (TEN) is a severe, immune‐mediated, mucocutaneous reaction resulting in extensive keratinocyte apoptosis. High‐dose human intravenous immunoglobulins (IVIG) have been proposed as an effective treatment for TEN. Retrospective data from 8 patients with TEN and 4 patients with Stevens‐Johnson syndrome‐toxic epidermal necrolysis (SJS‐TEN) overlap treated with high‐dose IVIG were analysed. The total dose of IVIG administered was 2 g/kg body weight, with the exception of 2 patients who received a total dose of 1.5 g/kg body weight. Their mean age was 49.9 ± 18.8 years (range, 19 to 70 years). The mean time from the first sign of skin lesion or mucosal or epidermal detachment to commencement of IVIG was 8.7 ± 5.5 days (range, 3 to 22 days). Of the 11 patients who survived, the mean time to objective response was 3.6 ± 1.9 days (range, 2 to 8 days). The length of stay (LOS) in hospital was 20.4 ± 8.0 days (range, 10 to 37 days). The survival rate was 91.6%. One patient developed permanent mucocutaneous sequelae following TEN. There were no adverse reactions to IVIG. We conclude that high‐dose IVIG may be a safe and effective therapy for Asian patients with TEN.Keywords:
Toxic Epidermal Necrolysis
Mucocutaneous zone
Dose
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous responses caused by drugs that are characterized by widespread necrosis and epidermal detachment. It is essential to know the risk factors and those agents involved in the development of this pathology in order to use them with caution in susceptible patients and to have a high index of suspicion.
Toxic Epidermal Necrolysis
Mucocutaneous zone
Cite
Citations (0)
ABSTRACT Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) represents a spectrum of rare but severe mucocutaneous drug reactions. Gastrointestinal involvement of SJS/TEN is associated with high morbidity and mortality and often presents 2–3 weeks after the initial appearance of skin lesions. There are no evidence-based treatment algorithms for the management of SJS/TEN. We report a case of life-threatening gastrointestinal bleeding from colonic involvement of SJS/TEN and discuss potential therapeutic options.
Toxic Epidermal Necrolysis
Gastrointestinal bleeding
Cite
Citations (3)
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered a spectrum of acute life-threatening mucocutaneous reactions that differ only in severity. Both diseases are characterized by mucous membrane and skin involvement, are often caused by medications, and are collectively known as epidermal necrolysis (EN).A severity of illness score has been devised to predict prognosis in patients with EN. The scoring system addresses 7 prognostic factors.Patients with EN require supportive care. Those with extensive skin involvement should be admitted to an intensive care unit or burn unit if possible. Suspected, as well as unnecessary, medications should be discontinued. Baseline laboratory tests, imaging, cultures, and biopsies should be obtained. Intravenous access should be established and hydration and nutritional support begun. Daily oral care, wound care, pain control, and early physician consultation are also important aspects of treatment.EN requires early diagnosis, appropriate workup, and appropriate treatment to minimize potential morbidity and mortality. In many clinicians' experience, EN is rare; therefore, education and improved understanding of the potential causes and appropriate treatment regimens are vital when confronted with such a patient.
Toxic Epidermal Necrolysis
Mucocutaneous zone
Mucous membrane
Cite
Citations (9)
Institute of Pharmaceutical Chemistry, GoetheUniversity, Frankfurt/Main, GermanyA rather wide range of drugs is able to cause severecutaneous adverse reactions. There is a variety ofsometimes life-threatening mucocutaneous reactionsincluding Stevens-Johnson syndrome (SJS) and ToxicEpidermal Necrolysis (TEN) which are regarded as twostages of a single disease. They are characterised bycutaneous erythema with blister formation of varyingextent and haemorrhagic erosions of mucousmembranes. SJS is characterised by skin detachmentfrom up to 10% of the body surface area (BSA) whileTEN, per definition, needs more than 30% skin affected.An overlap group of SJS/TEN has been defined withblisters and erosions between 10 and 30% of the bodysurface.
Toxic Epidermal Necrolysis
Mucocutaneous zone
Erythema
Drug reaction
Erythema multiforme
Body surface area
Cite
Citations (0)
Recent reports suggest that acquired immunodeficiency syndrome (AIDS) patients are at higher risk of developing mucocutaneous reactions such as toxic epidermal necrolysis and Stevens-Johnson syndrome (SJS). Resultant dry eye may be further exacerbated by human immunodeficiency virus (HIV) related lacrimal gland dysfunction and lead to a chronic keratoconjunctivitis. We report one patient with AIDS and toxic epidermal necrolysis and two patients with AIDS and SJS who developed severe dry eye misdiagnosed as infectious keratoconjunctivitis. Cicatrizing mucocutaneous reactions should be suspected in AIDS patients and the dry eye treated to control symptoms and prevent complications.
Toxic Epidermal Necrolysis
Mucocutaneous zone
Keratoconjunctivitis
Cite
Citations (26)
Toxic Epidermal Necrolysis
Mucocutaneous zone
Sloughing
Cite
Citations (81)
Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe adverse reactions to drugs that cause a life‐threatening eruption of mucocutaneous blistering and epithelial sloughing. While the acute complications of SJS/TEN are well described, it is increasingly recognized that survivors may develop delayed sequelae, some of which can be associated with significant morbidity. Studies of long‐term SJS/TEN outcomes mostly focus on mucocutaneous and ocular complications. However, other internal organs, such as the respiratory tract and gastrointestinal tract, can be affected. Psychological sequelae are also frequent following the trauma of widespread epidermal necrolysis. An appreciation of the 'chronic' phase of SJS/TEN is needed by clinicians caring for individuals who have survived the acute illness. This review aims to provide an update on the breadth and range of sequelae that can affect patients in the months and years following an acute episode of SJS/TEN.
Toxic Epidermal Necrolysis
Broad spectrum
Cite
Citations (134)
Abstract Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe dermatologic reactions with mucocutaneous involvement that carry elevated mortality rates. They differ along a spectrum of severity based upon body surface area affected. These conditions, usually caused by a drug or infection, are believed to result from cell-mediated and often drug-specific cytotoxic reactions against keratinocytes, leading to widespread dermal-epidermal detachment. Studies attempting to identify potential curative therapies such as intravenous immune globulin (IVIG) and corticosteroids remain inconclusive. However, improved outcomes have been demonstrated by early withdrawal of offending medications, early transfer to an intensive care unit or burn unit, and aggressive supportive care. Due to the rare incidence of SJS and TEN, its recurrence among survivors hints at future vulnerability for these patients, and notorious offending medications should thus be avoided. This clinical review will highlight the diagnostic and therapeutic challenges posed by SJS and TEN, while emphasizing the need to maintain them high on the emergency medicine physician's differential. The review will also detail the supportive measures to take for preventing the rapid progression of mucocutaneous complications and subsequent sepsis-related mortality.
Toxic Epidermal Necrolysis
Mucocutaneous zone
Erythroderma
Body surface area
Cite
Citations (2)
Toxic Epidermal Necrolysis
Mucocutaneous zone
Erythema multiforme
Erythema
Erythroderma
Cite
Citations (1)