P043 A scoping review of patient metrics, aetiology, management and complications in Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)
Stevens–Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) is a rare, life-threatening mucocutaneous adverse reaction. Limited clinical trials have been undertaken, and clinicians are reliant on case reports and series to guide clinical care. However, these case report and series have never been summarized. Our objective was to scrutinize the clinical characteristics (patient metrics, aetiology, management and complications) reported in case reports and series of SJS/TEN. The OVID MEDLINE and Embase databases were searched from inception to 2023 (updated in 2024). Case reports and series of patients with SJS/TEN of all ages were included. Articles had to be published in English, with keyword data that could be extracted from the abstract or title. Screening and data extraction were conducted by paired researchers independently. Clinical characteristics were categorized thematically; patients could be in more than one category. Patient and clinical characteristics were described using percentages. From 1847 articles identified, 1591 were included, of which 1166 (73%) abstracts were available; 204 (13%) were case series. In total, 1684 patients were included; 56% (484 of 869) were female; age 0–18 years was the commonest age group (18%, 143 of 814); and 1% (17 of 1684) were pregnant. The most common medical histories reported were HIV (17%, 61 of 364), neurological conditions (16%, 59 of 364) and mental health conditions (8%, 30 of 364). The most frequent cancer histories were haematological (28%, 54 of 196), lung (21%, 42 of 196), gastrointestinal/colorectal, and neurological (both 12%, 24 of 196). Erythema multiforme major (24%, 16 of 67) was the most common differential diagnosis. Overall, 137 causative drug classes were reported; the three most common were anticonvulsants (20%, 247 of 1262), nonsteroidal anti-inflammatories (7%, 83 of 1262) and immune checkpoint inhibitors (6%, 71 of 1262). The most common causative individual drugs were lamotrigine (n = 76), carbamazepine (n = 71), phenytoin (n = 45), co-trimoxazole (n = 36) and pembrolizumab (n = 28). The most prevalent causative infections were mycoplasma (31%, 33 of 108), COVID-19 (19%, 21 of 108) and herpes simplex virus (6%, 6 of 108). Investigations undertaken included human leucocyte antigen testing (15%, 34 of 232), lymphocyte transformation test (11%, 26 of 232) and drug patch testing (7%, 17 of 232). The most common treatments were steroid (any route; 53%, 382 of 718), intravenous immunoglobulins (26%, 184 of 718), antibiotics (14%, 98 of 718) and ciclosporin (10%, 70 of 718). The most frequent complications were ocular (other than conjunctivitis, symblepharon, corneal ulcer; 25%, 100 of 405), gastrointestinal (other than dysphagia, liver failure, vanishing bile duct syndrome; 14%, 55 of 405) and skin (13%, 54 of 405). Overall, 7% (111 of 1684) died. This is the first scoping review summarizing all case reports and series of SJS/TEN. We have highlighted reported medication and infection triggers, which may help healthcare professionals to identify a cause and be aware of those at risk. Regarding treatments, steroids are the mainstay, alongside intravenous immunoglobulin and ciclosporin. The range of complications emphasizes the need for a multidisciplinary management approach both in the short- and longer-term care of patients.
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