Toxic epidermal necrolysis in Stevens--Johnson syndrome.

Three cases are described in which Stevens-Johnson syndrome progressed in the course of a few days to toxic epidermal necrolysis. Trimethoprim-sulfamethoxazole, allopurinol in combination with hydrochlorothiazide, phenytoin and possibly ampicillin were implicated in the causation of the disease.     

替雷利珠单抗致中毒性表皮坏死松解症1例报告

目的 了解程序性细胞死亡受体-1（programmed cell death-1,PD-1）/程序性细胞死亡受体-配体1（programmed cell death-ligand 1,PD-L1）抑制剂治疗肿瘤导致Stevens-Johnson综合征（Stevens-Johnson syndrome,SJS）/中毒性表皮坏死松解症（toxic epidermal necrolysis,TEN）的临床特点。方法 回顾性分析海军军医大学第三附属医院皮肤科诊治的1例及既往文献报道的31例PD-1/PD-L1抑制剂治疗肿瘤导致SJS/TEN的临床资料。结果 共收集32例,其中SJS 15例,潜伏期平均8.4周,SJS-TEN 3例,潜伏期平均2.5周,TEN 14例,潜伏期平均3.8周。好转21例,转为慢性扁平苔鲜1例,加重1例,死亡8例,未报道1例。结论PD-1/PD-L1抑制剂治疗肿瘤导致SJS/TEN少见但危重,需引起临床医师的高度重视。     

Toxic epidermal necrolysis: a burn-centre challenge.

OBJECTIVE: To describe the clinical features and outcome of patients with toxic epidermal necrolysis. DESIGN: Case series. SETTING: Ross Tilley Burn Centre, Wellesley Hospital, Toronto. PATIENTS: Ten patients (seven women) with toxic epidermal necrolysis referred to the centre from 1984 to 1990. INTERVENTION: Biobrane (Winthrop Pharmaceuticals, New York) was applied to all raw surfaces after the epidermis had been completely separated. It was not removed until the epidermis had regenerated. MAIN RESULTS: The age of the patients varied from 27 to 90 (mean 45) years. The proportion of body surface area involved was 20% to 95% (mean 58.5%). Nine of the patients had received steroids and antibiotics prophylactically before being referred to the burn centre; the steroids were immediately tapered and the antibiotics withdrawn unless there was a specific infection. Urinary tract infection developed in seven patients, respiratory failure in six and systemic sepsis in six. All of the patients ultimately required antibiotics for treatment of systemic infections. Two patients died; both had severe illness before the onset of the toxic epidermal necrolysis. Biobrane provided a marked reduction in pain, eliminated the need for further dressings and allowed early, aggressive physiotherapy. The wounds healed completely within 14 to 21 days, with no significant scarring or need for skin grafting. CONCLUSIONS: The use of a temporary skin substitute is recommended for the treatment of toxic epidermal necrolysis. There is no indication for prophylactic steroid or antibiotic therapy; indeed, such therapy is probably contraindicated.     

Toxic epidermal necrolysis. A step forward in treatment

Toxic epidermal necrolysis is an uncommon but severe form of epidermal sloughing with associated mucositis. Treated in a general hospital, it carries a high mortality (25% to 70%) and substantial long-term morbidity. If the patient is referred early to a burn center, where it can be treated with biologic dressings and intensive support care, the mortality can be reduced below 20% and there may be negligible long-term morbidity. We describe 19 patients so treated, with three deaths and no long-term complications.     

Toxic epidermal necrolysis in a patient receiving concurrent phenytoin and whole brain and thoracic radiotherapy

Toxic epidermal necrolysis (TEN) is a severe drug induced type IV hypersensitivity syndrome that can be caused by anticonvulsant drugs, especially the aromatic anticonvulsants such as phenytoin. Most patients with brain metastasis receive whole brain radiotherapy along with anti-edema measures and anticonvulsants either as prophylactic or for symptom control; phenytoin being the most commonly used drug. In a subset of patients, cranial irradiation may act as a precipitating factor along with anticonvulsants for the development of TEN. We report a 54-year-old patient with metastatic non-small cell lung cancer treated with palliative whole brain and mediastinal radiotherapy with concurrent phenytoin-developing TEN, which started within the radiation portals with subsequent generalization. Though a rare, but serious complication, avoidance of the use of phenytoin concurrent with radiotherapy, replacing phenytoin with newer anticonvulsants, early recognition, aggressive management and awareness of this possible complication has been implied upon in this report.Steven Johnson Syndrome (SJS), Steven Johnson Syndrome-toxic epidermal necrolysis (SJS-TEN), and toxic epidermal necrolysis (TEN) are a spectrum of type IV hypersensitivity drug-induced disorders, which are characterized by blisters and epidermal detachment resulting from epidermal necrosis in the absence of substantial dermal inflammation often associated with anticonvulsant medications.1,2 Patients with brain metastasis, irrespective of the primary tumor location are often symptomatic. They require active treatment in the form of palliative radiotherapy to the whole brain and symptomatic management, which includes anti-edema measures with systemic steroids and use of anticonvulsants either for seizure control or prophylactic use. Phenytoin is the most common drug used for seizure control. A subgroup of patients receiving this combination of whole brain radiotherapy and phenytoin develop severe cutaneous hypersensitive drug reaction that may manifest as SJS-TEN spectrum of systemic syndrome.3,4 In this report, we describe one case of TEN developing in a patient with metastatic lung cancer receiving palliative whole brain and thoracic radiotherapy (RT) with concurrent phenytoin, emphasizing the need for anticipation, early recognition, and aggressive management of this potentially fatal medical emergency.