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Bourke CM, Cummings BK, Hurley DJ, Murphy CC, Chamney S. Isolated Ocular Stevens-Johnson Syndrome Caused by Lymecycline in a Patient with Underlying Ulcerative Colitis. J Clin Med 2023; 12:5259. [PMID: 37629300 PMCID: PMC10456061 DOI: 10.3390/jcm12165259] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2023] [Revised: 08/03/2023] [Accepted: 08/10/2023] [Indexed: 08/27/2023] Open
Abstract
Stevens-Johnson syndrome (SJS) and the more severe variant, toxic epidermal necrolysis (TEN), are a spectrum of mucocutaneous reactions with potentially devastating ocular consequences. Ocular complications occur in about 70% of patients with Stevens-Johnson syndrome, and 35% continue with chronic disease. We report an unusual presentation of isolated ocular Stevens-Johnson syndrome in a patient with recently diagnosed ulcerative colitis being treated with Infliximab. The case had an insidious and atypical onset and represented a diagnostic dilemma. The diagnosis was more difficult, due to the fact that the inciting agent had long been stopped. Severe bacterial conjunctivitis such as that caused by Chlamydia Trachomatis, Corynebacterium diphtheria, and Neisseria Gonorrhea can cause forniceal shortening and symblepharon; this diagnosis was ruled out with microbiological swabs. A conjunctival biopsy was the key to diagnosis. Treatment involved high-dose IV steroids and dual immunosuppression with Infliximab and mycophenolate mofetil. We sought to employ interventions with the greatest impacts on our patient's condition. Our experience contributes to the growing evidence supporting intensive ophthalmic management of SJS to prevent long-term vision loss.
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Affiliation(s)
- Christine M. Bourke
- Department of Ophthalmology, Children’s Health Ireland at Crumlin, Cooley Road, Crumlin, D12 N512 Dublin, Ireland;
| | - Brendan K. Cummings
- Department of Ophthalmology, Royal Victoria Eye and Ear Hospital, Adelaide Road, D02 XK51 Dublin, Ireland; (D.J.H.); (C.C.M.)
| | - Daire J. Hurley
- Department of Ophthalmology, Royal Victoria Eye and Ear Hospital, Adelaide Road, D02 XK51 Dublin, Ireland; (D.J.H.); (C.C.M.)
| | - Conor C. Murphy
- Department of Ophthalmology, Royal Victoria Eye and Ear Hospital, Adelaide Road, D02 XK51 Dublin, Ireland; (D.J.H.); (C.C.M.)
| | - Sarah Chamney
- Department of Ophthalmology, Children’s Health Ireland at Crumlin, Cooley Road, Crumlin, D12 N512 Dublin, Ireland;
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Lehloenya RJ. Disease severity and status in Stevens–Johnson syndrome and toxic epidermal necrolysis: Key knowledge gaps and research needs. Front Med (Lausanne) 2022; 9:901401. [PMID: 36172538 PMCID: PMC9510751 DOI: 10.3389/fmed.2022.901401] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2022] [Accepted: 07/18/2022] [Indexed: 11/26/2022] Open
Abstract
Stevens–Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are on a spectrum of cutaneous drug reactions characterized by pan-epidermal necrosis with SJS affecting < 10% of body surface area (BSA), TEN > 30%, and SJS/TEN overlap between 10 and 30%. Severity-of-illness score for toxic epidermal necrolysis (SCORTEN) is a validated tool to predict mortality rates based on age, heart rate, BSA, malignancy and serum urea, bicarbonate, and glucose. Despite improved understanding, SJS/TEN mortality remains constant and therapeutic interventions are not universally accepted for a number of reasons, including rarity of SJS/TEN; inconsistent definition of cases, disease severity, and endpoints in studies; low efficacy of interventions; and variations in treatment protocols. Apart from mortality, none of the other endpoints used to evaluate interventions, including duration of hospitalization, is sufficiently standardized to be reproducible across cases and treatment centers. Some of the gaps in SJS/TEN research can be narrowed through international collaboration to harmonize research endpoints. A case is made for an urgent international collaborative effort to develop consensus on definitions of endpoints such as disease status, progression, cessation, and complete re-epithelialization in interventional studies. The deficiencies of using BSA as the sole determinant of SJS/TEN severity, excluding internal organ involvement and extension of skin necrosis beyond the epidermis, are discussed and the role these factors play on time to healing and mortality beyond the acute stage is highlighted. The potential role of artificial intelligence, biomarkers, and PET/CT scan with radiolabeled glucose as markers of disease status, activity, and therapeutic response is also discussed.
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Affiliation(s)
- Rannakoe J. Lehloenya
- Division of Dermatology, Department of Medicine, University of Cape Town, Cape Town, South Africa
- Combined Drug Allergy Clinic, Groote Schuur Hospital, Cape Town, South Africa
- *Correspondence: Rannakoe J. Lehloenya, ; orcid.org/0000-0002-1281-1789
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3
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Vunnam N, Hansen S, Williams DC, Been M, Lo CH, Pandey AK, Paulson CN, Rohde JA, Thomas DD, Sachs JN, Wood DK. Fluorescence Lifetime Measurement of Prefibrillar Sickle Hemoglobin Oligomers as a Platform for Drug Discovery in Sickle Cell Disease. Biomacromolecules 2022; 23:3822-3830. [PMID: 35944154 PMCID: PMC9472799 DOI: 10.1021/acs.biomac.2c00671] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/26/2022] [Revised: 07/22/2022] [Indexed: 11/30/2022]
Abstract
The molecular origin of sickle cell disease (SCD) has been known since 1949, but treatments remain limited. We present the first high-throughput screening (HTS) platform for discovering small molecules that directly inhibit sickle hemoglobin (HbS) oligomerization and improve blood flow, potentially overcoming a long-standing bottleneck in SCD drug discovery. We show that at concentrations far below the threshold for nucleation and rapid polymerization, deoxygenated HbS forms small assemblies of multiple α2β2 tetramers. Our HTS platform leverages high-sensitivity fluorescence lifetime measurements that monitor these temporally stable prefibrillar HbS oligomers. We show that this approach is sensitive to compounds that inhibit HbS polymerization with or without modulating hemoglobin oxygen binding affinity. We also report the results of a pilot small-molecule screen in which we discovered and validated several novel inhibitors of HbS oligomerization.
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Affiliation(s)
- Nagamani Vunnam
- Department
of Biomedical Engineering, University of
Minnesota, Minneapolis, Minnesota 55455, United States
| | - Scott Hansen
- Department
of Biomedical Engineering, University of
Minnesota, Minneapolis, Minnesota 55455, United States
| | - Dillon C. Williams
- Department
of Biomedical Engineering, University of
Minnesota, Minneapolis, Minnesota 55455, United States
| | - MaryJane
Olivia Been
- Department
of Biomedical Engineering, University of
Minnesota, Minneapolis, Minnesota 55455, United States
| | - Chih Hung Lo
- Department
of Biomedical Engineering, University of
Minnesota, Minneapolis, Minnesota 55455, United States
| | - Anil K. Pandey
- Department
of Biomedical Engineering, University of
Minnesota, Minneapolis, Minnesota 55455, United States
| | - Carolyn N. Paulson
- Department
of Biomedical Engineering, University of
Minnesota, Minneapolis, Minnesota 55455, United States
| | - John A. Rohde
- Department
of Biochemistry, Molecular Biology, and Biophysics, University of Minnesota, Minneapolis, Minnesota 55455, United States
| | - David D. Thomas
- Department
of Biochemistry, Molecular Biology, and Biophysics, University of Minnesota, Minneapolis, Minnesota 55455, United States
| | - Jonathan N. Sachs
- Department
of Biomedical Engineering, University of
Minnesota, Minneapolis, Minnesota 55455, United States
| | - David K. Wood
- Department
of Biomedical Engineering, University of
Minnesota, Minneapolis, Minnesota 55455, United States
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4
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Lee KCH, Ko JP, Oh CC, Sewa DW. Managing respiratory complications in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Int J Dermatol 2021; 61:660-666. [PMID: 34494255 DOI: 10.1111/ijd.15888] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/20/2021] [Revised: 07/29/2021] [Accepted: 08/13/2021] [Indexed: 11/28/2022]
Abstract
In the recently published guidelines by the Society of Dermatology Hospitalists on the management of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), a brief section was included on airway management. These recommendations provide an easy reference on how to manage respiratory complications of the disease. Understanding the evidence that underlies these recommendations would offer physicians greater clarity on the considerations behind every decision and treatment offered. We present a review of the literature on respiratory manifestations associated with SJS and TEN. In addition, we aim to address specific concerns regarding the respiratory management of these patients. These include issues such as the indications and optimal timing of intubation, tracheostomy, role of flexible nasoendoscopy, bronchoscopy, ventilation strategies, and management of chronic respiratory complications.
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Affiliation(s)
- Ken Cheah Hooi Lee
- Department of Respiratory and Critical Care Medicine, Singapore General Hospital, Singapore, Singapore
| | - Joanna Phone Ko
- Nursing Division (Specialty Nursing), Singapore General Hospital, Singapore, Singapore
| | - Choon Chiat Oh
- Department of Dermatology, Singapore General Hospital, Singapore, Singapore
| | - Duu Wen Sewa
- Department of Respiratory and Critical Care Medicine, Singapore General Hospital, Singapore, Singapore
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Bechek S, Garcia M, Chiou H. Severe Gastrointestinal Involvement in Pediatric Stevens-Johnson Syndrome: A Case Report and Review of the Literature. Clin Exp Gastroenterol 2020; 13:377-383. [PMID: 33061516 PMCID: PMC7533238 DOI: 10.2147/ceg.s269349] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/25/2020] [Accepted: 08/11/2020] [Indexed: 12/11/2022] Open
Abstract
Stevens-Johnson syndrome and toxic epidermal necrolysis form a rare but severe disease spectrum characterized by widespread epidermal detachment. Gastrointestinal manifestations of the disease, however, are rarely described in the pediatric literature and have a high mortality among adults. There are limited data on the treatment of these cases, with conflicting evidence regarding the benefit of steroids, IVIG, or other immunosuppressive agents. We review previous instances of gastrointestinal involvement in children and report the case of a previously healthy 13-year-old who presented with the typical ocular and skin findings of Stevens-Johnson syndrome, subsequently developed severe life-threatening diarrhea, and was found to have severe esophagitis, duodenitis, and colitis on endoscopic evaluation. Treatment was initiated with an immediate, short course of steroids along with early introduction of an enteral diet via nasogastric tube, and resulted in full gastrointestinal recovery. This case highlights successful medical treatment of the first reported pediatric case of SJS/TEN with both upper and lower gastrointestinal tract involvement.
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Affiliation(s)
- Sophia Bechek
- Stanford University School of Medicine, Stanford, CA, USA
| | - Manuel Garcia
- Division of Pediatric Gastroenterology, Stanford University School of Medicine, Stanford, CA, USA
| | - Howard Chiou
- Department of Medicine, Santa Clara Valley Medical Center, San Jose, CA, USA
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Jha AK, Suchismita A, Jha RK, Raj VK. Spectrum of gastrointestinal involvement in Stevens - Johnson syndrome. World J Gastrointest Endosc 2019; 11:115-123. [PMID: 30788030 PMCID: PMC6379748 DOI: 10.4253/wjge.v11.i2.115] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/17/2018] [Revised: 01/29/2019] [Accepted: 02/13/2019] [Indexed: 02/06/2023] Open
Abstract
Stevens - Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) is a severe adverse drug reaction associated with involvement of skin and mucosal membranes, and carries significant risk of mortality and morbidity. Mucus membrane lesions usually involve the oral cavity, lips, bulbar conjunctiva and the anogenitalia. The oral/anal mucosa and liver are commonly involved in SJS or TEN. However, intestinal involvement is distinctly rare. We herein review the current literature regarding the gastrointestinal involvement in SJS or TEN. This review focuses mainly on the small bowel and colonic involvement in patients with SJS or TEN.
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Affiliation(s)
- Ashish Kumar Jha
- Department of Gastroenterology, Indira Gandhi Institute of Medical Science, Sheikhpura, Patna 800014, India
| | - Arya Suchismita
- Department of Pediatrics, Indira Gandhi Institute of Medical Science, Sheikhpura, Patna 800014, India
| | | | - Vikas Kumar Raj
- Health Center, National Institute of Technology, Patna 800014, India
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Toxic epidermal necrolysis data from the CELESTE multinational registry. Part II: Specific systemic and local risk factors for the development of infectious complications. Burns 2018; 44:1561-1572. [PMID: 29903602 DOI: 10.1016/j.burns.2018.03.006] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/06/2017] [Revised: 03/13/2018] [Accepted: 03/19/2018] [Indexed: 12/20/2022]
Abstract
The aim of the study was to identify the most important systemic and local risk factors for the development of infectious complications in patients with toxic epidermal necrolysis (TEN). MATERIAL AND METHODOLOGY This is a multicentric study that included all patients with TEN who were hospitalized between 2000-2015 in specialized centres in the Czech Republic and Slovakia. The total catchment area included a population of over 12.5 million inhabitants. The actual implementation of the project was carried out using data obtained from the CELESTE (Central European LyEll Syndrome: Therapeutic Evaluation) registry, wherein specific parameters related to epidemiological indicators and infectious complications in patients with TEN were evaluated as a retrospective analysis. RESULTS A total of 38 patients (97%) of the group were treated with corticosteroids. The comparison of patients with different doses of corticosteroids did not exhibit a statistically significant effect of corticosteroid administration on the development of infectious complications (p=0.421). There was no effect of the extent of the exfoliated area on the development of infectious complications in this area. The average extent of the exfoliated area was 66% TBSA (total body surface area) in patients with reported infectious complications and 71% TBSA (p=0.675) in patients without infectious complications. In the case of the development of an infectious complication in the bloodstream (BSI), the increasing effect of the SCORTEN (SCORe of Toxic Epidermal Necrosis) value was monitored during hospitalization. Within 5days from the beginning of the hospitalization, the average SCORTEN value was 2.7 in 6 patients with BSI and 3.0 in 32 patients without BSI (p=0.588). In the period after the 15th day of hospitalization, 7 patients with BSI had an average SCORTEN value of 3.4, and 16 patients without BSI had an average SCORTEN value of 2.5 (p=0.079). In the case of low respiratory tract infection (LRTI), the effects of the necessity for artificial pulmonary ventilation and the presence of tracheostomy were monitored. The statistically significant effect of mechanical ventilation on the development of LRTI occurred only during the period of 11-15days from the beginning of the hospitalization (p=0.016). The effect of the tracheostomy on the development of LRTI was proven to be more significant. CONCLUSION We did not find any statistically significant correlation between the nature of immunosuppressive therapy and the risk of developing infectious complications. We failed to identify statistically significant risk factors for the development of BSI. Mechanical ventilation and tracheostomy increase the likelihood of developing LRTIs in patients with TEN.
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8
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Toxic Epidermal Necrolysis with Gastrointestinal Involvement: A Case Report and Review of the Literature. J Burn Care Res 2018; 38:e450-e455. [PMID: 27058583 DOI: 10.1097/bcr.0000000000000336] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
Gastrointestinal involvement is a rare complication of toxic epidermal necrolysis syndrome (TENS) that results in sloughing of the intestinal epithelium. Prior case reports have noted the potential susceptibility of the entire gastrointestinal tract, from oropharynx and esophagus to sigmoid colon and rectum. Given its infrequency, the effect of gastrointestinal involvement on the treatment and prognosis of TENS is poorly understood. Here, the authors report a case of gastrointestinal symptoms in a patient diagnosed with toxic epidermal necrolysis, likely representing gastrointestinal involvement. In addition, the authors describe the histopathologic and endoscopic characteristics of the involved mucosa, clinical course, and present a review of the literature of this rare but potentially impactful complication of TENS.
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9
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Saeed H, Mantagos IS, Chodosh J. Complications of Stevens–Johnson syndrome beyond the eye and skin. Burns 2016; 42:20-27. [DOI: 10.1016/j.burns.2015.03.012] [Citation(s) in RCA: 39] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/26/2014] [Revised: 03/01/2015] [Accepted: 03/20/2015] [Indexed: 11/27/2022]
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10
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Williams R, Hodge J, Ingram W. Indications for intubation and early tracheostomy in patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Am J Surg 2016; 211:684-688.e1. [PMID: 26860621 DOI: 10.1016/j.amjsurg.2015.12.011] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/31/2015] [Revised: 12/04/2015] [Accepted: 12/08/2015] [Indexed: 11/30/2022]
Abstract
BACKGROUND Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) result in epidermal sloughing and mucositis. There are no published guidelines for intubation and early tracheostomy in this patient population. METHODS A retrospective chart review of 40 patients admitted from 2010 to 2015 with SJS and TEN was conducted. Descriptive statistics and significance were calculated. RESULTS Of the 43% of patients who underwent early tracheostomy, 100% had oral involvement while the initial total body surface area (TBSA) was 70% or more in 41% of patients (P < .05). TBSA progressed 15% or more in 53% of patients with 6% having airway involvement and a neurologic diagnosis mandating intubation. Mortality was 17%. CONCLUSIONS Indications for intubation and early tracheostomy for SJS and TEN are documented oral involvement plus one of the following: initial TBSA 70% or more; progression of TBSA involved from hospital day 1 to hospital day 3, 15% TBSA or more; underlying neurologic diagnosis preventing airway protection; and documented airway involvement on direct laryngoscopy.
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Affiliation(s)
- Rachael Williams
- Emory University School of Medicine and Grady Health System, 69 Jesse Hill Jr. Drive, SE, Atlanta, GA, 30303, USA.
| | - Juvonda Hodge
- Emory University School of Medicine and Grady Health System, 69 Jesse Hill Jr. Drive, SE, Atlanta, GA, 30303, USA
| | - Walter Ingram
- Emory University School of Medicine and Grady Health System, 69 Jesse Hill Jr. Drive, SE, Atlanta, GA, 30303, USA
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11
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Mesenteric ischemia secondary to toxic epidermal necrolysis: case report and review of the literature. J Burn Care Res 2015; 35:e346-52. [PMID: 24496304 DOI: 10.1097/bcr.0000000000000006] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
A 28-year-old otherwise healthy man was admitted to the burn center for treatment of toxic epidermal necrolysis (TEN) involving 90% of the TBSA and oropharynx. On hospital day 8, his cutaneous lesions were healing well, but he developed respiratory distress, fever, and abdominal distension. Computerized tomography demonstrated distended bowel, pneumatosis intestinalis, and portal venous gas. He underwent emergent celiotomy. Patchy areas of nonperforated necrosis along the jejunum and ileum were present. No mechanical or embolic source of ischemia could be identified. A 120-cm segment of ischemic small bowel was resected and the abdomen was closed temporarily. On planned "second look" the following day, no further disease was encountered and intestinal continuity was restored. Tube feeds were then initiated and the patient's recovery was uneventful thereafter. Although traditionally considered a skin disorder, TEN may be more accurately described as a disorder affecting the junction of an epithelium and its supporting tissue. It is most prominently manifested at the epidermal-dermal junction, but epithelial-submucosal junctions are also affected. The ocular, respiratory, genitourinary, and gastrointestinal manifestations of TEN are variable and incompletely understood. This disease is rooted in immunological dysfunction and the small bowel is rich in immunologically active tissue; Peyer patches and lymph nodes abound. Clinicians should be vigilant for gastrointestinal tract involvement, which is potentially treatable with resection of the ischemic bowel. The authors suspect that, given the critical condition of many TEN patients, bowel symptoms may be incorrectly attributed to global hypoperfusion and sepsis.
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12
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Heye P, Descloux A, Singer G, Rosenberg R, Kocher T. Perforated sigmoid diverticulitis in the presence of toxic epidermal necrolysis. Case Rep Dermatol 2014; 6:49-53. [PMID: 24707250 PMCID: PMC3975207 DOI: 10.1159/000360129] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022] Open
Abstract
Even though the incidence of toxic epidermal necrolysis (TEN) is low, it is also associated with a high mortality rate. The condition predominantly affects the skin, but may also affect the gastrointestinal tract, dramatically increasing mortality. We present a case of perforated sigmoid diverticulitis in the presence of TEN. The patient was taking medication, known to be a risk factor, and presented an affected total body surface area and temporal development similar to previously reported cases of TEN. Characteristic abdominal symptoms, however, were missing. Gastrointestinal involvement in TEN appears to be a poor prognostic factor; medical staff must therefore be alert to patients with TEN who complain of abdominal discomfort. The exact pathogenesis, however, remains unclear.
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Affiliation(s)
- P Heye
- Department of Surgery, Kantonsspital Baden, Baden, Switzerland
| | - A Descloux
- Department of Surgery, Kantonsspital Baden, Baden, Switzerland
| | - G Singer
- Department of Pathology, Kantonsspital Baden, Baden, Switzerland
| | - R Rosenberg
- Department of Surgery, Kantonsspital Baden, Baden, Switzerland
| | - T Kocher
- Department of Surgery, Kantonsspital Baden, Baden, Switzerland
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Bouziri A, Khaldi A, Hamdi A, Borgi A, Ghorbel S, Kharfi M, Hadj SB, Menif K, Ben Jaballah N. Toxic epidermal necrolysis complicated by small bowel intussusception: a case report. J Pediatr Surg 2011; 46:e9-e11. [PMID: 21292071 DOI: 10.1016/j.jpedsurg.2010.09.011] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/14/2010] [Revised: 08/30/2010] [Accepted: 09/01/2010] [Indexed: 12/19/2022]
Abstract
Intestinal involvement in toxic epidermal necrolysis (TEN) has been identified only rarely. We report a case of TEN complicated by small bowel intussusception. The patient was a previously healthy 8-year-old boy who presented with TEN and extensive lesions, including up to 40% of the body surface area as well as conjunctival, oropharyngeal, respiratory, and genital mucosa. Rapidly after the onset of a constant rate of enteral feeding, he developed bilious vomiting, diarrhea, and significant abdominal distension. Abdominal sonography showed a small bowel intussusception. At abdominal exploration, an ileoileal intussusception was observed with a viable but inflamed bowel wall. Manual reduction was performed. During the postoperative clinical course, the patient was managed with total parenteral nutrition and local care of the skin and mucous membranes. Enteral feeding was introduced on the sixth postoperative day, and the child left the hospital 15 days after his admission. The association of TEN and small bowel intussusception has not been previously reported in the literature.
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Affiliation(s)
- Asma Bouziri
- Paediatric Intensive Care Unit, Children's Hospital of Tunis, Tunis, Tunisia.
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Ward KE, Archambault R, Mersfelder TL. Severe adverse skin reactions to nonsteroidal antiinflammatory drugs: A review of the literature. Am J Health Syst Pharm 2010; 67:206-13. [DOI: 10.2146/ajhp080603] [Citation(s) in RCA: 59] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022] Open
Affiliation(s)
- Kristina E. Ward
- College of Pharmacy, University of Rhode Island, Kingston. LT USN MSC
| | - Raoul Archambault
- Department of Pharmacy, Robert E. Bush Naval Hospital, Twentynine Palms, CA; at the time of writing he was a student, College of Pharmacy, University of Rhode Island
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Sakai N, Yoshizawa Y, Amano A, Higashi N, Aoki M, Seo T, Suzuki K, Tanaka S, Tsukui T, Sakamoto C, Arai M, Yamamoto Y, Kawana S. Toxic epidermal necrolysis complicated by multiple intestinal ulcers. Int J Dermatol 2008; 47:180-2. [PMID: 18211494 DOI: 10.1111/j.1365-4632.2008.03389.x] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Affiliation(s)
- Noriyasu Sakai
- Departments of Dermatology and Digestive Medicine, and Center of Emergency Medicine, Nippon Medical School, Tokyo, Japan.
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16
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Saxena R, Loghmanee F. Fatal Drug Reaction Due to Allopurinol Therapy in a 72-Year-Old Man. Arch Pathol Lab Med 2005; 129:e183-4. [PMID: 16048416 DOI: 10.5858/2005-129-e183-fdrdta] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Affiliation(s)
- Rakhee Saxena
- Department of Pathology, State University of New York, Buffalo, NY, USA.
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17
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Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS. Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. Ann Allergy Asthma Immunol 2005; 94:419-36; quiz 436-8, 456. [PMID: 15875523 DOI: 10.1016/s1081-1206(10)61112-x] [Citation(s) in RCA: 198] [Impact Index Per Article: 9.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
Abstract
OBJECTIVE To perform a comprehensive review of Stevens-Johnson syndrome and toxic epidermal necrolysis. DATA SOURCES A MEDLINE search was performed for the years 1975 to 2003 using the keywords Stevens-Johnson syndrome and toxic epidermal necrolysis to identify relevant articles published in English in peer-reviewed journals. STUDY SELECTION All clinical studies that reported on 4 or more patients, review articles, and experimental studies that concerned disease mechanisms were selected and further analyzed. Clinical reports that included fewer than 4 patients were selected only if they were believed to carry a significant message about disease mechanism or therapy. RESULTS Stevens-Johnson syndrome and toxic epidermal necrolysis seem to be variants of the same disease with differing severities. A widely accepted consensus regarding diagnostic criteria and therapy does not exist at present. Despite the recent experimental studies, the pathogenic mechanisms of these diseases remain unknown. Although progress in survival through early hospitalization in specialized burn units has been made, the prevalence of life-long disability from the ocular morbidity of Stevens-Johnson syndrome and toxic epidermal necrolysis has remained unchanged for the past 35 years. Further progress depends on modification of the acute phase of the disease rather than continuation of supportive care. The available published evidence indicates that a principal problem in the pathogenesis is immunologic and that immunomodulatory intervention with short-term, high-dose intravenous steroids or intravenous immunoglobulin holds the most promise for effective change in survival and long-term morbidity. CONCLUSIONS The results of this review call for a widely accepted consensus on diagnostic criteria for Stevens-Johnson and toxic epidermal necrolysis and multicenter collaboration in experimental studies and clinical trials that investigate disease mechanisms and novel therapeutic interventions, respectively.
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Affiliation(s)
- Erik Letko
- Department of Ophthalmology, Uveitis and Immunology Service, The Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts 02114, USA
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Carneiro M, Lescano M, Romanello L, Modena J, Carneiro F, Ramalho L, Martinelli A, Franca A. ACUTE ESOPHAGEAL NECROSIS. Dig Endosc 2005. [DOI: 10.1111/j.1443-1661.2005.00464.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/23/2023]
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García Fernández D, García-Patos Briones V, Castells Rodellas A. Síndrome de Stevens-Johnson/necrólisis epidérmica tóxica. ACTA ACUST UNITED AC 2001. [DOI: 10.1016/s0213-9251(01)72498-7] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
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20
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Affiliation(s)
- J T Stutts
- Department of Pediatrics, Division of Gastroenterology and Nutrition and Department of Pathology, Vanderbilt University School of Medicine, Nashville, Tennessee, USA
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21
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Sugimoto Y, Mizutani H, Sato T, Kawamura N, Ohkouchi K, Shimizu M. Toxic epidermal necrolysis with severe gastrointestinal mucosal cell death: a patient who excreted long tubes of dead intestinal epithelium. J Dermatol 1998; 25:533-8. [PMID: 9769600 DOI: 10.1111/j.1346-8138.1998.tb02450.x] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
TEN is a severe inflammatory disease which is characterized by generalized epithelial destruction. The epidermis is the most common target of TEN, however, any epithelium can be involved. We report a toxic epidermal necrolysis (TEN) patient who excreted long tubes of dead intestinal epithelium. Epidermal keratinocytes and intestinal epithelium were found to undergo extensive apoptosis by TUNEL method. Drugs were speculated as the causative agents for this case, the causative drug has not been identified. In contrast to marked improvement of cutaneous manifestation and hepatic function by methyl prednisolone pulse therapy, the gastrointestinal symptoms did not respond to therapies, and the patient died by heart failure. Present case suggested a pathogenetic mechanism targeting antigens commonly expressed on the gastrointestinal epithelium and epidermis.
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Affiliation(s)
- Y Sugimoto
- Department of Dermatology, Mie University, Faculty of Medicine, Japan
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22
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Lebargy F, Wolkenstein P, Gisselbrecht M, Lange F, Fleury-Feith J, Delclaux C, Roupie E, Revuz J, Roujeau JC. Pulmonary complications in toxic epidermal necrolysis: a prospective clinical study. Intensive Care Med 1997; 23:1237-44. [PMID: 9470079 PMCID: PMC7095164 DOI: 10.1007/s001340050492] [Citation(s) in RCA: 140] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
OBJECTIVE To evaluate the incidence, clinical features, and prognosis of pulmonary complications associated with toxic epidermal necrolysis DESIGN Prospective study. SETTING Dermatology intensive care unit in Mondor Hospital, France. PATIENTS 41 consecutive patients. INTERVENTIONS On admission, then daily, respiratory evaluation was based on clinical examination, chest X-ray, and arterial blood gas analysis. When clinical symptoms, X-ray abnormalities, or hypoxemia [partial pressure of oxygen (PO2) < 80 mm Hg] were present, fiberoptic bronchoscopy was performed. RESULTS 10 patients presented early manifestations: dyspnea (n = 10), bronchial hypersecretion (n = 7), marked hypoxemia (n = 10) (PO2 = 59 +/- 8 mm Hg). Chest X-ray was normal (n = 8) or showed interstitial infiltrates (n = 2). In these 10 patients, fiberoptic bronchoscopy demonstrated sloughing of bronchial epithelium in proximal airways. Delayed pulmonary complications occurred in 6 of these 10 patients from day 7 to day 15: pulmonary edema (n = 2), atelectasis (n = 1), bacterial pneumonitis (n = 4). Mechanical ventilation was required in 9 patients. A fatal outcome occurred in 7 patients. Seven patients did not develop early pulmonary manifestations (PO2 on admission 87 +/- 6 mm Hg) but only delayed pulmonary symptoms related to atelectasis (n = 1), pulmonary edema (n = 4), and bacterial pneumonitis (n = 3); bronchial epithelial detachment was not observed. None of them required mechanical ventilation and all recovered with appropriate therapy. CONCLUSIONS "Specific" involvement of bronchial epithelium was noted in 27% of cases and must be suspected when dyspnea, bronchial hypersecretion, normal chest X-ray, and marked hypoxemia are present during the early stages of toxic epidermal necrosis. Bronchial injury seems to indicate a poor prognosis, as mechanical ventilation was required for most of these patients and was associated with a high mortality.
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Affiliation(s)
- F Lebargy
- Hôpital Henri-Mondor, Créteil, France
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Affiliation(s)
- J C Roujeau
- Department of Dermatology, Henri Mondor Hospital, University of Paris XII, Creteil, France
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Michel P, Joly P, Ducrotte P, Hemet J, Leblanc I, Lauret P, Lerebours E, Colin R. Ileal involvement in toxic epidermal necrolysis (Lyell syndrome). Dig Dis Sci 1993; 38:1938-41. [PMID: 8404419 DOI: 10.1007/bf01296123] [Citation(s) in RCA: 21] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/30/2023]
Abstract
Intestinal involvement in toxic epidermal necrolysis (TEN) has been identified only rarely. This report describes a case of TEN with ileal manifestations characterized by a profuse diarrhea with malabsorption, protein-losing enteropathy, and radiologically by multiple stenosis. After healing of the cutaneous lesions, total parenteral nutrition was initiated, resulting in a decreased diarrhea. However, after one month of total parenteral nutrition, malabsorption and protein-losing enteropathy continued and the radiological lesions were still present with an aspect consistent with a sclerotic process. A surgical resection of the pathological ileal segment was performed with end-to-end anastomosis. Pathological examination of the resected segment showed a necrosis of the ileal mucosa with a pattern similar to that of the epidermal necrosis. No sclerosis was observed. It seems that a prolonged total parenteral nutrition could have induced a complete healing of intestinal lesions. This case report is the first clinical, radiological, and histological study of an ileal involvement in TEN.
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Affiliation(s)
- P Michel
- Service des maladies de l'Appareil digestif et de la Nutrition, Hôpital Charles Nicolle, Rouen, France
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25
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Affiliation(s)
- J C Roujeau
- Dermatology Service, Henri Mondor Hospital, University of Paris XII, France
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26
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Carter FM, Mitchell CK. Toxic epidermal necrolysis--an unusual cause of colonic perforation. Report of a case. Dis Colon Rectum 1993; 36:773-7. [PMID: 8348869 DOI: 10.1007/bf02048370] [Citation(s) in RCA: 30] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/30/2023]
Abstract
Toxic epidermal necrolysis (TEN) is a rare and severe reaction to certain drugs that results in full-thickness epidermal necrosis of the skin. Other mucosal surfaces such as the oropharynx, esophagus, conjunctiva, and genitalia may also be affected. Specific involvement of the colon is distinctly unusual. A case of TEN that resulted in complete small bowel necrosis and colonic perforation is reported. Early and aggressive surgical resection dealt successfully with this complication and, along with supportive care, allowed resolution of the systemic condition to occur. Five additional cases of colonic necrosis complicating TEN are reviewed.
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Affiliation(s)
- F M Carter
- Department of Surgery, East Carolina University School of Medicine, Greenville, North Carolina 27858
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27
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Affiliation(s)
- A Mahé
- Institut Marchoux, Bamako, Mali
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28
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29
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de la Cotera FJ, Kuo PC. Toxic epidermal necrolysis: report of a case. J Oral Maxillofac Surg 1992; 50:638-42. [PMID: 1593330 DOI: 10.1016/0278-2391(92)90450-e] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
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30
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Roujeau JC. Toxic epidermal necrolysis (Lyell syndrome): more than "acute skin failure". Intensive Care Med 1992; 18:4-5. [PMID: 1578046 DOI: 10.1007/bf01706417] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
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31
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Timsit JF, Mion G, Rouyer N, Le Gulluche Y, Carsin H. Bronchopulmonary distress associated with toxic epidermal necrolysis. Intensive Care Med 1992; 18:42-4. [PMID: 1578047 DOI: 10.1007/bf01706425] [Citation(s) in RCA: 26] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
We describe here a patient with severe TEN and respiratory distress and we review the subject of bronchopulmonary symptoms in TEN. Even if pseudostratified ciliated involvement is uncommon, bronchial lesions in the absence of other known causes, should be specifically related to TEN. The mechanisms of pulmonary involvement and ARDS associated with TEN are discussed.
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Affiliation(s)
- J F Timsit
- Burns Center, Hôpital d'Instruction des Armées Percy, Clamart, France
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32
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Chosidow O, Delchier JC, Chaumette MT, Wechsler J, Wolkenstein P, Bourgault I, Roujeau JC, Revuz J. Intestinal involvement in drug-induced toxic epidermal necrolysis. Lancet 1991; 337:928. [PMID: 1673016 DOI: 10.1016/0140-6736(91)90273-r] [Citation(s) in RCA: 59] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
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Abstract
Toxic epidermal necrolysis is perhaps the most formidable disease encountered by dermatologists. Uncommon but not rare, toxic epidermal necrolysis occurs in 60 to 70 persons per year in France. It remains as puzzling a disorder as it was 34 years ago, when described by Lyell. Whether or not toxic epidermal necrolysis is the most severe form of erythema multiforme is still the subject of discussion. The physiopathologic events that lead to this rapidly extensive necrosis of the epidermis are not understood. Indirect evidence suggests a hypersensitivity reaction, but the search for potential immunologic mechanisms has resulted in little data to support this hypothesis. Accumulated clinical evidence points to drugs as the most important, if not the only, cause of toxic epidermal necrolysis. Sulfonamides, especially long-acting forms, anticonvulsants, nonsteroidal anti-inflammatory agents, and certain antibiotics are associated with most cases of toxic epidermal necrolysis. Many other drugs have been implicated in isolated case reports. All organs may be involved either by the same process of destruction of the epithelium as observed in the epidermis or by the same systemic consequences of "acute skin failure" as seen in patients with widespread burns. Sepsis is the most important complication and cause of death. Approximately 20% to 30% of all patients with toxic epidermal necrolysis die. Elderly patients and patients with extensive lesions have a higher mortality rate. Surviving patients completely heal in 3 to 4 weeks, but up to 50% will have residual, potentially disabling ocular lesions. The prognosis is improved by adequate therapy, as provided in burn units, that is, aggressive fluid replacement, nutritional support, and a coherent antibacterial policy. Corticosteroids, advocated by some in high doses to halt the "hypersensitivity" process, have been shown in several studies to be detrimental and should be avoided.
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Affiliation(s)
- J C Roujeau
- Department of Dermatology, Hôpital Henri Mondor, Université Paris XII
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34
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Mion G, Le Gulluche Y, Carsin H, Verdon R, Paulet R. [Fatal Lyell's syndrome caused by griseofulvin]. ANNALES FRANCAISES D'ANESTHESIE ET DE REANIMATION 1990; 9:305-8. [PMID: 2196842 DOI: 10.1016/s0750-7658(05)80192-3] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
A case of toxic epidermolysis (TE) with a fatal outcome is reported. It occurred after administration of 500 mg griseofulvin twice daily in a 19-year-old female patient. She developed the first skin lesions on the sixth day of treatment. All the body surface was involved, except for the scalp. Several complications arose in the course of the disease, thrombocytopaenia, lymphocytopaenia, rhabdomyolysis, and non cardiogenic pulmonary oedema. Death occurred as a result of multiple organ failure following septic shock associated with adult respiratory distress syndrome. The pathogenesis of these complications and the major therapeutic difficulties encountered are discussed. The involvement of griseofulvin in TE has only been reported once before. The arguments in favour of its involvement in the present case are discussed.
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Affiliation(s)
- G Mion
- Centre de Traitement des Brûlés, Hôpital d'Instruction des Armées Percy, Clamart
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