A case of toxic epidermal necrolysis with lesions mostly on sun‐exposed skin

At, Mansur; Aydıngöz, İkbal Esen

doi:10.1111/j.1600-0781.2005.00149.x

Cited by 14 publications

(17 citation statements)

References 4 publications

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“…4 Two case of TEN have been reported in which the bullae and erosions occurred in sun-exposed areas. 53,54 These patients presented with typical features of fever, mucositis, and erosions, but the erosions were limited to light-exposed skin. Subsequent photopatch tests were negative.…”

Section: Clinical Aspects Of Sjs/tenmentioning

confidence: 98%

Toxic epidermal necrolysis

Pereira

Mudgil

Rosmarin

2007

Journal of the American Academy of Dermatology

287

237

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Section: Clinical Aspects Of Sjs/tenmentioning

confidence: 98%

Toxic epidermal necrolysis

Pereira

Mudgil

Rosmarin

2007

Journal of the American Academy of Dermatology

287

237

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“…Case reports exist for hydroxchloroquine [58], naproxene [59] and clobazam [60]. An often addressed issue is the induction of TEN or SJS after vaccination.…”

Section: Etiology and Pathogenesismentioning

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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“…1 There have been four published case reports associated with naproxen. 1,[14][15][16][17] There were also four published cases of ibuprofen resulting in SJS/TEN, which also resulted in liver involvement. 1,2,[18][19][20] A review article by Ward et al 1 reported that older patients, women, and patients within the first month of treatment with NSAIDS may have the highest risk for SJS/TEN.…”

Section: Discussionmentioning

confidence: 99%