Early Cyclosporine Treatment of Incipient Toxic Epidermal Necrolysis Induced by Concomitant Use of Lamotrigine and Sodium Valproate

Hashim, Natasha; Bandara, Dalmianti; Tan, E.; Ilchyshyn, A.

doi:10.1080/00015550310005906

Cited by 26 publications

(11 citation statements)

References 8 publications

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“…A small case series with three TEN patients treated initially with high-doses of intravenous dexamethasone followed by CsA showed a stop in disease progression within 72 h [87]. Other single case reports also reported a positive effect of the use of CsA in TEN [88,89]. Recently, Valeyrie-Allanore L conducted an open, phase II trial to determine the safety and possible benefit of ciclosporin [90].…”

Section: Management and Therapymentioning

confidence: 99%

Toxic epidermal necrolysis and Stevens-Johnson syndrome

Harr

French

2010

Orphanet J Rare Dis

512

556

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Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. Both are rare, with TEN and SJS affecting approximately 1or 2/1,000,000 annually, and are considered medical emergencies as they are potentially fatal. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Currently, TEN and SJS are considered to be two ends of a spectrum of severe epidermolytic adverse cutaneous drug reactions, differing only by their extent of skin detachment. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. Diagnosis relies mainly on clinical signs together with the histological analysis of a skin biopsy showing typical full-thickness epidermal necrolysis due to extensive keratinocyte apoptosis. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP), disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS). Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, evaluation of the prognosis using SCORTEN, identification and interruption of the culprit drug, specialized supportive care ideally in an intensive care unit, and consideration of immunomodulating agents such as high-dose intravenous immunoglobulin therapy. SJS and TEN are severe and life-threatening. The average reported mortality rate of SJS is 1-5%, and of TEN is 25-35%; it can be even higher in elderly patients and those with a large surface area of epidermal detachment. More than 50% of patients surviving TEN suffer from long-term sequelae of the disease.

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Section: Management and Therapymentioning

confidence: 99%

Toxic epidermal necrolysis and Stevens-Johnson syndrome

Harr

French

2010

Orphanet J Rare Dis

512

556

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“…Several case reports and case series revealed encouraging result of use of cyclosporine in stopping disease progression and to prevent the mortality. [11,[28][29][30][31][32][33][34] In Indian subcontinent, systemic steroids have traditionally been used to manage this condition due to its experience of use, easy availability, and cost-effectiveness despite having multiple complications. This study was designed to evaluate the efficacy of cyclosporine and compare the results with patients who were managed by systemic steroids in tertiary health-care setting.…”

Section: Introduction Introductionmentioning

confidence: 99%

Cyclosporine in Stevens Johnson syndrome and toxic epidermal necrolysis and retrospective comparison with systemic corticosteroid

Singh¹,

Chatterjee²,

Verma³

2013

Indian J Dermatol Venereol Leprol

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“…In all the included studies, the adverse event starts with dermatological manifestation like generalized reddish pruritic rashes over face, neck, chest, and upper extremities, erythematous maculopapular eruptions, bullous rash, skin lesions, ulceration and hemorrhagic crusts on lip and nose, and exulcerations in the genital region that worsened and resulted in more than 40% epidermal detachment and extensive exfoliation of skin. Positive Nikolsky's sign (shearing off of the epidermis with lateral pressure), which is slight rubbing of skin resulting in exfoliation of the outermost layers, has been reported in four cases . Opened lesions and erythematous skin adjacent to the blistering indicates positive Nikolsky's sign.…”

Section: Resultsmentioning

confidence: 94%

Valproic acid and Stevens‐Johnson syndrome: a systematic review of descriptive studies

Rashid¹,

Kashyap

Undela

2019

Int J Dermatology

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Valproic acid (VPA) is a commonly used antiepileptic drug (AED). Stevens‐Johnson syndrome (SJS) and toxic epidermal necrolysis are the rare but fatal cutaneous adverse drug reactions for VPA. We aimed to identify and critically evaluate all the literature of SJS in association with VPA and to describe the clinical features of the condition. A literature search was conducted in MEDLINE/PubMed from inception to December 2017 and updated in July 2018 without any restrictions, and the references of included studies were also searched. The descriptive studies discussing any patients who experienced SJS followed by the use of VPA alone or along with any other AED for its main indication were included. Two authors were independently involved in the study selection and data extraction. Disagreements were resolved by consensus or by the discussion with a third reviewer. A total of 19 studies including 17 case reports and two case series of 98 were included in the review. In all studies, the dose of VPA ranged from 100 mg/day to a maximum dose of 1,000 mg/day and was administered for indications including epilepsy, seizures, schizophrenic affective disorders, and bipolar disorder. The mortality seen with severe cutaneous adverse drug reactions can be decreased by immediate withdrawal of opposing medications, providing symptomatic relief, and offering supportive care, all of which are the mainstay of controlling the condition. It is essential for healthcare professionals to be aware of the importance of monitoring SJS or any other cutaneous reactions followed by the use of valproic acid even though the incidence is low, but it is injurious to the patient.

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Early Cyclosporine Treatment of Incipient Toxic Epidermal Necrolysis Induced by Concomitant Use of Lamotrigine and Sodium Valproate

Cited by 26 publications

References 8 publications

Toxic epidermal necrolysis and Stevens-Johnson syndrome

Toxic epidermal necrolysis and Stevens-Johnson syndrome

Cyclosporine in Stevens Johnson syndrome and toxic epidermal necrolysis and retrospective comparison with systemic corticosteroid

Valproic acid and Stevens‐Johnson syndrome: a systematic review of descriptive studies

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