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Principles of Clinical Diagnosis
Published in Susan Bayliss Mallory, Alanna Bree, Peggy Chern, Illustrated Manual of Pediatric Dermatology, 2005
Susan Bayliss Mallory, Alanna Bree, Peggy Chern
Major pointsStevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are on a spectrum and have considerable overlap (see Chapter 13)Severe cutaneous reaction in which the individual appears acutely illRare in infants and children compared to teens and adultsCombined incidence of SJS and TEN is approximately 1.5–2 cases per million per year in the general populationStevens–Johnson syndrome (erythema multiforme major):Drugs are the major precipitating factorMycoplasma pneumoniae infection associated in children and teensClinical features:1–14 day prodrome of high fever, sore throat, malaiseRapid onset of cutaneous blistering with epidermal detachment beginning with target lesions (Figures 11.4–11.6)
Descriptions of important reactions
Published in Jerome Z. Litt, Neil H. Shear, Litt's Drug Eruption & Reaction Manual, 2017
The Stevens-Johnson syndrome (erythema multiforme major), a severe and occasionally fatal variety of erythema multiforme, has an abrupt onset and is accompanied by any or all of the following: fever, myalgia, malaise, headache, arthralgia, ocular involvement, with occasional bullae and erosions covering less than 10% of the body surface. Painful stomatitis is an early and conspicuous symptom. Hemorrhagic bullae may appear over the lips, mouth and genital mucous membranes. Patients are often acutely ill with high fever. The course from eruption to the healing of the lesions may extend up to six weeks.
Descriptions of Important Reactions
Published in Jerome Z. Litt, Neil H. Shear, Litt's Drug Eruption & Reaction Manual, 2017
The Stevens–Johnson syndrome (erythema multiforme major), a severe and occasionally fatal variety of erythema multiforme, has an abrupt onset and is accompanied by any or all of the following: fever, myalgia, malaise, headache, arthralgia, ocular involvement, with occasional bullae and erosions covering less than 10% of the body surface. Painful stomatitis is an early and conspicuous symptom. Hemorrhagic bullae may appear over the lips, mouth and genital mucous membranes. Patients are often acutely ill with high fever. The course from eruption to the healing of the lesions may extend up to six weeks.
Stevens-Johnson syndrome and toxic epidermal necrolysis: risk factors, causality assessment and potential prevention strategies
Published in Expert Review of Clinical Immunology, 2020
Chu-Chi Lin, Chun-Bing Chen, Chuang-Wei Wang, Shuen-Iu Hung, Wen-Hung Chung
A similar skin reaction, erythema multiforme major (EMM) with clinical characteristic features of typical target lesions on acral parts, is majorly caused by microbial infection such as Mycoplasma pneumonia and herpes simplex virus infection, rather than drugs. Studies have shown that EMM has a different pathophysiological mechanism to cause necrosis below the epidermis [18]. EMM and SJS/TEN could be distinguished from each other by different demographic symptoms, clinical presentation and risk factors [19]. The case–control surveillance cohort study by the European SCAR group (RegiSCAR) have already identified the risk medications related to SJS/TEN [20]. The studies showed that sulfonamides, aromatic anticonvulsants, allopurinol, oxicam, nonsteroidal anti-inflammatory drugs, and anti-retroviral drugs could account for most proportion of SJS/TEN [20]. Newly developed immune checkpoint blockade and anti-cancer target therapies also have been reported to induce SJS/TEN [21]. SJS/TEN triggered by immune checkpoints blockade with anti-programmed death-1 (PD-1) (such as nivolumab, and pembrolizumab) and anti-cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) (ipilimumab) have been reported [22]. Proton pump inhibitors, known to induce type I hypersensitivity reactions, have also been found to induce life-threatening SCARs in our previous study [23]. Studies showed that SJS has a mortality of 10% and TEN has as high as 30-40% in patients around the global world [24]. The mortality of SJS/TEN can be affected by patients’ underlying risk factors during the acute stage of disease, such as old age, poor renal function, increased heart rate, associated malignancy, extensive skin detachment (>10%), decreased serum bicarbonate and increased serum glucose [25].