Stevens-Johnson syndrome in a patient with rheumatoid arthritis during long-term etanercept therapy

Abstract

Background: Etanercept and other anti-TNF-alpha agents have been indicated as a therapeutic option in severe drug reactions, including Stevens-Johnson syndrome and toxic epidermal necrolysis. Etanercept has been shown to quickly reduce the detachment of the epidermis and shorten healing time. Cases of etanercept-induced severe adverse drug reactions were also described. Main observations:A 27-year-old woman with a 4-year history of etanercept and sulfasalazine treatment for rheumatoid arthritis was admitted with StevensJohnson syndrome. The patient received one dose of an OTC drug containing acetaminophen, phenylephrine and pheniramine two days prior to developing fist mucocutaneous symptoms. The most probable causative agent was paracetamol. Throughout the successful routine therapy of Stevens-Johnson syndrome etanercept therapy was continued. Sulfosalazin administration was stopped and administered again after recovery with no recurrence of the skin and mucosal symptoms. Conclusions:This case indicates that there is no justification for discontinuation of long-term anti-TNF-alpha treatment in patients who develop StevensJohnson syndrome / toxic epidermal necrolysis.

Is acetaminophen associated with a risk of Stevens–Johnson syndrome and toxic epidermal necrolysis? Analysis of the French Pharmacovigilance Database

“…We were able to apply ALDEN retrospectively using available data in 12 cases. Acetaminophen causality was ‘probable’ (ALDEN score = 4) in four cases , ‘possible’ (ALDEN score = 2–3) in five cases and ‘very unlikely’ (ALDEN score <0) in three cases . The ALDEN score was inapplicable in eight cases because of a lack of data on the delay between acetaminophen intake and index day .…”

“…The two positive rechallenges were questionable because of a dubious SJS diagnosis in the first case and a conflicting immediate anaphylactoid reaction in the second case . Apart from the drug causality, a protopathic or confounding bias was possible in 15 cases (75%) due to an infectious context , including six cases with acetaminophen causality as ‘possible’ or ‘probable’. Moreover, in the twenty case reports, the diagnosis of SJS/TEN was often not clearly documented, and was even questionable, with clinical features often compatible with erythema multiforme major , which is more frequent in children and young adults, recurs regularly, has a better prognosis and can be either infectious or idiopathic in origin .…”

“…Twenty case reports of SJS/TEN related to acetaminophen are available in the international literature , two of them with a positive rechallenge. We were able to apply ALDEN retrospectively using available data in 12 cases.…”

Toxic Epidermal Necrolysis complicating Infliximab therapy in Refractory Kawasaki disease

“…There were several cases of severe skin reactions complicating TNF-α antagonist therapy in adults [3][4][5]. These cases were treated with etanercept or adalimumab for Crohn's disease or rheumatoid disease, and they were recovered after steroid treatment with discontinuation of the causative drug.…”

“…But, TNF-α antagonists had several adverse reactions, such as serious infections, optic neuritis, aplastic anemia, congestive heart failure and multiple cutaneous adverse effects such as eczema, erythema urticaria, lupus-like syndrome and even Stevens-Johnson syndrome (SJS)/Toxic epidermal necrolysis (TEN) [3]. A few numbers of cases have been reported of SJS induced by anti-TNF-α therapy in adults with Crohn's disease and rheumatoid disease [3][4][5], nevertheless, there was no reported similar cases in children. Herein, we describe a case of TEN induced by infliximab therapy in refractory KD.…”