Abstract
Background: Toxic Epidermal Necrolysis (TEN) is a dermatological emergency characterized by generalized epidermal necrolysis, causing peeling of the skin, which becomes susceptible to serious infections. It presents multisystemic involvement, with high morbidity and mortality. The management of TEN is multifaceted, and begins with identifying and removing the causative agent. Identification of the agent depends on a complete clinical history, as symptoms generally present within eight weeks of initiating drug therapy. Suspending the causative drug is fundamental in the evolution of the pathology and clinical success depends, mainly, on supportive treatment, to avoid complications, such as sepsis.
Aim: To report a single case of NET seen in our service. Method: This is a case report of a patient treated at the Hospital Universitário São Francisco na Providência de Deus – HUSF, located in the city of Bragança Paulista – SP, Brazil.
Case Report: The case reported in this paper was a 33-year-old male patient, with no known comorbidities, who presented with pruritic and painful erythematous vesicular lesions, initially in the groin region and upper limbs, with progression to the trunk and lower limbs, associated with fever, ocular hyperemia and conjunctivitis. The patient also had anemia, elevated CRP and hypoalbuminemia, but with normal renal and hepatic function. Serologies for HIV and syphilis were performed, with positive results. During hospitalization, the lesions rapidly worsened, with mucosal involvement, skin peeling and epidermal detachment, raising the suspicion of TEN. The patient was admitted to the ICU due to generalized involvement, newly diagnosed HIV and risk of sepsis. Treatment included isolation, intensive skin care, cyclosporine, benzathine penicillin due to the diagnosis of syphilis and antiretroviral therapy for HIV, leading to significant improvement. After 11 days of hospitalization, the patient was discharged from the hospital and maintained outpatient follow-up with an infectious disease specialist and dermatologist.
Conclusion: This paper highlighted a clinical case that illustrates the diversity of scenarios in which TEN can occur, emphasizing the importance of early recognition, multidisciplinary treatment and appropriate management to improve the outlook for patients, especially those who are immunosuppressed. Furthermore, awareness about TEN, especially in relation to specific medications, is crucial to ensure effective management and follow-up of patients, especially those with underlying health conditions.
References
ABE, R. Immunological response in Stevens-Johnson syndrome and toxic epidermal necrolysis. The Journal of Dermatology, v. 42, n. 1, p. 42–48, jan. 2015.
ADAM, J.; PICHLER, W. J.; YERLY, D. Delayed drug hypersensitivity: models of T-cell stimulation. British Journal of Clinical Pharmacology, v. 71, n. 5, p. 701–707, maio 2011.
ALAJMI, A. et al. A pediatric case of Stevens-Johnson syndrome/toxic epidermal necrolysis with rapid response to intravenous cyclosporine. JAAD case reports, v. 6, n. 6, p. 555–557, jun. 2020.
ALERHAND, S.; CASSELLA, C.; KOYFMAN, A. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in the Pediatric Population: A Review. Pediatric Emergency Care, v. 32, n. 7, p. 472–476, jul. 2016.
BASU, S. et al. Chronic Ocular Sequelae of Stevens-Johnson Syndrome in Children: Long-term Impact of Appropriate Therapy on Natural History of Disease. American Journal of Ophthalmology, v. 189, p. 17–28, maio 2018.
CHABY, G. et al. Incidence of and mortality from epidermal necrolysis (Stevens-Johnson syndrome/toxic epidermal necrolysis) in France during 2003-16: a four-source capture-recapture estimate. The British Journal of Dermatology, v. 182, n. 3, p. 618–624, mar. 2020.
CHARLTON, O. A. et al. Toxic Epidermal Necrolysis and Steven-Johnson Syndrome: A Comprehensive Review. Advances in Wound Care, v. 9, n. 7, p. 426–439, jul. 2020.
DUONG, T. A. et al. Severe cutaneous adverse reactions to drugs. Lancet (London, England), v. 390, n. 10106, p. 1996–2011, 28 out. 2017.
FRANTZ, R. et al. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management. Medicina (Kaunas, Lithuania), v. 57, n. 9, p. 895, 28 ago. 2021.
FREY, N. et al. The Epidemiology of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in the UK. The Journal of Investigative Dermatology, v. 137, n. 6, p. 1240–1247, jun. 2017a.
FREY, N. et al. The Epidemiology of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in the UK. The Journal of Investigative Dermatology, v. 137, n. 6, p. 1240–1247, jun. 2017b.
GALLAGHER, R. M. et al. Development and inter-rater reliability of the Liverpool adverse drug reaction causality assessment tool. PloS One, v. 6, n. 12, p. e28096, 2011.
GARCIA-DOVAL, I. et al. Toxic epidermal necrolysis and Stevens-Johnson syndrome: does early withdrawal of causative drugs decrease the risk of death? Archives of Dermatology, v. 136, n. 3, p. 323–327, mar. 2000.
GELINCIK, A.; CAVKAYTAR, O.; KUYUCU, S. An Update on the Management of Severe Cutaneous Drug Hypersensitivity Reactions. Current Pharmaceutical Design, v. 25, n. 36, p. 3881–3901, 2019.
GILBERT, M.; SCHERRER, L. A. Efficacy and safety of cyclosporine in Stevens–Johnson syndrome and toxic epidermal necrolysis. Dermatologic Therapy, v. 32, n. 1, p. e12758, 2019.
GOMULKA, J.; WILSON, B. D.; JOYCE, J. C. Toxic epidermal necrolysis due to voriconazole: case report and review. Dermatology Online Journal, v. 20, n. 9, p. 13030/qt2pt32578, 16 set. 2014.
GREGORY, D. G. New Grading System and Treatment Guidelines for the Acute Ocular Manifestations of Stevens-Johnson Syndrome. Ophthalmology, v. 123, n. 8, p. 1653–1658, ago. 2016.
GRÜNWALD, P. et al. Erythema multiforme, Stevens-Johnson syndrome/toxic epidermal necrolysis - diagnosis and treatment. Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology: JDDG, v. 18, n. 6, p. 547–553, jun. 2020.
GUVENIR, H. et al. Clinical Phenotypes of Severe Cutaneous Drug Hypersensitivity Reactions. Current Pharmaceutical Design, v. 25, n. 36, p. 3840–3854, 2019.
HASEGAWA, A.; ABE, R. Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis. F1000Research, v. 9, p. F1000 Faculty Rev-612, 2020.
HSU, D. Y. et al. Morbidity and Mortality of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in United States Adults. The Journal of Investigative Dermatology, v. 136, n. 7, p. 1387–1397, jul. 2016.
HSU, D. Y. et al. Pediatric Stevens-Johnson syndrome and toxic epidermal necrolysis in the United States. Journal of the American Academy of Dermatology, v. 76, n. 5, p. 811- 817.e4, maio 2017.
KOH, H. K. et al. Risk factors and diagnostic markers of bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: A cohort study of 176 patients. Journal of the American Academy of Dermatology, v. 81, n. 3, p. 686–693, 1 set. 2019.
LIM, V. M. et al. A decade of burn unit experience with Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: Clinical pathological diagnosis and risk factor awareness. Burns: Journal of the International Society for Burn Injuries, v. 42, n. 4, p. 836–843, jun. 2016.
LIN, C.-C. et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: risk factors, causality assessment and potential prevention strategies. Expert Review of Clinical Immunology, v. 16, n. 4, p. 373–387, abr. 2020.
LISSIA, M. et al. Toxic epidermal necrolysis (Lyell’s disease). Burns: Journal of the International Society for Burn Injuries, v. 36, n. 2, p. 152–163, mar. 2010.
MANCINELLI, M. C. et al. Toxic epidermal necrolysis in HIV-infected patients: are intravenous immunoglobulins really necessary in this subgroup? International journal of STD & AIDS, v. 32, n. 2, p. 202–204, fev. 2021.
MCCULLOUGH, M. et al. Steven Johnson Syndrome and Toxic Epidermal Necrolysis in a burn unit: A 15-year experience. Burns: Journal of the International Society for Burn Injuries, v. 43, n. 1, p. 200–205, fev. 2017.
DE MELO, M. E. F. et al. Principais classes farmacológicas relacionadas à farmacodermia. Research, Society and Development, v. 11, n. 2, p. e8011225492-e8011225492, 2022.
NOE, M. H.; MICHELETTI, R. G. Diagnosis and management of Stevens-Johnson syndrome/toxic epidermal necrolysis. Clinics in Dermatology, Adverse Cutaneous Reactions to Medications. v. 38, n. 6, p. 607–612, 1 nov. 2020.
PAULMANN, M.; MOCKENHAUPT, M. Fever in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Pediatric Cases: Laboratory Work-up and Antibiotic Therapy. The Pediatric Infectious Disease Journal, v. 36, n. 5, p. 513–515, maio 2017.
PEREIRA, F. A.; MUDGIL, A. V.; ROSMARIN, D. M. Toxic epidermal necrolysis. Journal of the American Academy of Dermatology, v. 56, n. 2, p. 181–200, fev. 2007.
PICHLER, W. J. Modes of presentation of chemical neoantigens to the immune system. Toxicology, v. 181–182, p. 49–54, 27 dez. 2002.
PRIMISAWITRI, P. P.; MAWARDI, P. The Correlation of Neutrophil–Lymphocyte Ratio and Eosinophil Count with SCORTEN in SJS/TEN. Clinical, Cosmetic and Investigational Dermatology, v. 15, p. 547–556, 30 mar. 2022.
RICHER, V. et al. Acute blistering diseases on the burn ward: Beyond Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Burns: Journal of the International Society for Burn Injuries, v. 39, n. 6, p. 1290–1296, set. 2013.
SASSOLAS, B. et al. ALDEN, an algorithm for assessment of drug causality in Stevens-Johnson Syndrome and toxic epidermal necrolysis: comparison with case-control analysis. Clinical Pharmacology and Therapeutics, v. 88, n. 1, p. 60–68, jul. 2010.
SEKULA, P. et al. Comprehensive survival analysis of a cohort of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. The Journal of Investigative Dermatology, v. 133, n. 5, p. 1197–1204, maio 2013.
SHANBHAG, S. S. et al. Multidisciplinary care in Stevens-Johnson syndrome. Therapeutic Advances in Chronic Disease, v. 11, p. 2040622319894469, 2020.
SINGH, N.; PHILLIPS, M. Toxic Epidermal Necrolysis: A Review of Past and Present Therapeutic Approaches. Skin Therapy Letter, v. 27, n. 5, p. 7–13, set. 2022.
TCHETNYA, X. et al. Severe eye complications from toxic epidermal necrolysis following initiation of Nevirapine based HAART regimen in a child with HIV infection: a case from Cameroon. BMC pediatrics, v. 18, n. 1, p. 108, 13 mar. 2018.
TUNCER, B. et al. Anesthetic management of toxic epidermal necrolysis: a report of two cases. Anaesthesia, Pain & Intensive Care, v. 27, n. 1, p. 135–138, 2023.
YANG, M.-S. et al. Incidence of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Nationwide Population-Based Study Using National Health Insurance Database in Korea. PloS One, v. 11, n. 11, p. e0165933, 2016.

This work is licensed under a Creative Commons Attribution 4.0 International License.