Clinical features of Stevens — Johnson syndrome and toxic epidermal necrolysis: An observational cohort retrospective study

Background. Stevens — Johnson syndrome and toxic epidermal necrolysis are severe diseases featuring lesions of the mucous membranes and skin, with a possible lethal outcome. These diseases are most often triggered by various infections and medications, e.g., nonsteroidal anti-inflammatory drugs, antibacterial drugs, B vitamins, antiepileptic drugs, etc. Early diagnosing is therefore critical for immediate discontinuation of the inducing agent and initiation of treatment.

Objectives. To study etiologic factors, the severity level and mortality rate in hospitalized patients with Stevens — Johnson syndrome and toxic epidermal necrolysis.

Methods. An observational cohort retrospective study was conducted to analyze the case histories of 229 patients with Stevens — Johnson syndrome and toxic epidermal necrolysis hospitalized in the Allergology Department of the Municipal Clinical Hospital No. 21 in Ufa, in 2014–2023. The diagnoses of Stevens — Johnson syndrome and toxic epidermal necrolysis were established by complex investigations. The clinical criteria for Stevens — Johnson syndrome were acute conditions characterized by mucous membrane erosions and skin lesions (atypical michenoid lesions, bullae, and/or erosions) with maximum epidermal detachment of less than 10% of the total body surface area; for toxic epidermal necrolysis, maximum epidermal detachment of more than 30% of the total body surface area in addition to the above symptoms. Cases with maximum epidermal detachment of 10–30% of the total body surface area were classified as overlapping Stevens — Johnson syndrome and toxic epidermal necrolysis. The study group comprised 229 patients, including 44.1% men and 55.9% women, aged 45 (34; 61) years. Patients were divided into three groups: Group 1 (n = 83/229, 36%) had Stevens — Johnson syndrome, Group 2 (n = 41/229; 18%) had a combination of Stevens — Johnson syndrome and toxic epidermal necrolysis, and Group 3 (n = 105/229; 46%) had toxic epidermal necrolysis. The data analyzed in patients in this study included demographic information (age and sex), medical history and comorbid conditions, prior medication use, and the time between the first administration of the causative drug and the onset of symptoms of all disease groups studied. The SCORTEN (Score of Toxic Epidermal Necrosis) scale was used to assess the severity of course and prognosis in patients with toxic epidermal necrolysis. Hospital mortality was also studied in this group of patients. Differences were considered significant if the p-level of rejection of the null hypothesis of their absence did not exceed 0.05.

Results. The leading causes of disease development were medications, with a total of 127/229 (55%), among which antibiotics prevailed with 52/127 (41%), non-steroidal anti-inflammatory drugs with 31/127 (24.4%), anticonvulsants, antiretrovirals, and B vitamins with 6/127 (4.7%) each. The second frequently registered trigger was viral infections, 34/229 (14.8%) including herpes simplex virus types 1 and 2, human immunodeficiency virus, Epstein — Barr, and hepatitis viruses. The etiologic factor could not be established in almost one third of patients, 66/229 (28.8%). Manifestation of symptoms depended on the type of reaction, with toxic epidermal necrolysis developing more often in the first week and Stevens — Johnson syndrome manifesting predominantly in the fourth week. Most patients experienced the diseases for the first time; recurrent manifestations were registered only in 21/229 (9.2%) patients. The clinical presentation included skin lesions 229/229 (100%), hyperthermia 155/229 (67.7%,), oral mucosal erosions 201/229 (87.8%) and genital erosions 142/229 (62%), and conjunctivitis 162/229 (70.7%). Toxic epidermal necrolysis was associated with a more severe course. The calculated mortality for 105 patients with toxic epidermal necrolysis according to the SCORTEN scale was 21.38% (22 cases). The mean SCORTEN score was 2.39 ± 0.59. However, 4 patients died (mortality rate 4%). All patients with lethal outcome were scored 4 on the SCORTEN scale.

Conclusion. Drug exposure and viral infection were more frequent causes of toxic epidermal necrolysis than Stevens — Johnson syndrome and the combination of Stevens-Johnson syndrome and toxic epidermal necrolysis. In toxic epidermal necrolysis, symptoms more frequently developed in the 1st week after drug administration. However, the mortality rate of patients amounted to 4%, which was significantly lower than the estimated (21.38%) according to the SCORTEN scale. This may be due to early diagnosis of the disease and timely provision of medical care in the hospital.

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