Severity-of-illness Score For Toxic Epidermal Necrolysis Research Articles

Toxic Epidermal Necrolysis

Toxic epidermal necrolysis (TEN) represents the most severe drug-related skin condition that is potentially life-threatening with no well-established treatments. The application of corticosteroid therapy is controversial, whereas recently intravenous immunoglobulin (IVIG) therapy is emerging as a promising new method. A severity-of-illness score for TEN (SCORTEN) has gained acceptance in some western countries. In this study, our objectives were to assess the applicability of SCORTEN in Chinese patients with TEN and to evaluate the efficacy of the combination therapy of IVIG and corticosteroid in these patients. We performed a retrospective review of data from 61 patients with TEN treated at our intensive care unit from 2000 to 2010 to assess the performance of SCORTEN. In particular, 55 patients between 2002 and 2010 were grouped as a series to compare the therapeutic effects of corticosteroid therapy and IVIG combined therapy contemporaneously. During this period, 16 patients were administered with corticosteroid therapy and 39 were treated with the combination therapy. An initial dose of 1.5 mg/kg/day of methylprednisolone was given to all TEN patients. The combination therapy was combined with a total dose of 2 g/kg IVIG within 5 days. Areas under receiver operating characteristic curves and Hosmer-Lemeshow statistic were analyzed to illustrate the performance of SCORTEN. The comparison of the efficacy of the two therapies was conducted on the basis of clinical outcomes, standardized mortality ratio (SMR), and survival analysis. The overall actual mortality of patients between 2000 and 2010 was 16% (10/61), statistically insignificantly lower than predicted (24%, SMR = 67.98). Excellent discriminatory power (the areas under the receiver operating characteristic curves: 88.9, 88.2, 90.6%) and good calibration (P = .637, .833, .530) were found in all the groups. In patients admitted between 2002 and 2010, IVIG combined therapy showed a trend toward reducing the mortality rate (13%, SMR = 52.35), whereas corticosteroid monotherapy suggested no such difference (31%, SMR = 123.92). Besides, the cumulative survival rates of the combination therapy were higher at almost all the levels of SCORTEN (P = .002), especially at the score of 5 (P = 3.10 × 10⁻⁷). Compared with corticosteroid alone, the combination therapy arrested progression earlier (P = .013), although it did not significantly lead to a tapering of corticosteroid or a reduction of the time of hospitalization. We concluded that SCORTEN was generally applicable to Chinese patients with TEN. The comparison of the effect indicated that the combination therapy might achieve a better therapeutic effect than the administration of corticosteroid alone, especially in severe TEN patients.

In a Patient With Toxic Epidermal Necrolysis, Does Intravenous Immunoglobulin Improve Survival Compared With Supportive Care?

Clinical Question: A 67-year-old Chinese man presented with pain and widespread skin erythema and detachment. A clinical diagnosis of toxic epidermal necrolysis (TEN) was made, which was supported by skin biopsy findings. The TEN was thought to be due to recent initiation of lamotrigine for mild epilepsy, and the drug was stopped immediately. The severity of TEN, measured by the Severityof-Illness Score for Toxic Epidermal Necrolysis (SCORTEN) scale, was 3 on admission owing to renal impairment, indicating a mortality risk of 35%. The patient was transferred to the burns unit for close monitoring of temperature, fluid balance, and appropriate dressings for his affected skin. A debate ensued among the dermatology, plastic surgery, and intensive care teams responsible for his care about whether intravenous immunoglobulin (IVIg) should be added to his treatment because improvement was slow with conservative treatment.

Toxic epidermal necrolysis following thalidomide and dexamethasone treatment for multiple myeloma: a case report

Dear Editor, Toxic epidermal necrolysis (TEN) is a life-threatening idiosyncratic adverse drug reaction characterized by fullthickness epidermal necrosis and eventual development of subepidermal bullae [1, 2]. Carbamazapine, phenytoin, and allopurinol are frequent causes of TEN in Asia [1]. Although thalidomide has been suggested as a cause of TEN, to the best of our knowledge, only two cases had been reported worldwide [3, 4]. We present the case of a 65-year-old male patient who was initially diagnosed with Immunoglobulin G-κ (IgG-κ) multiple myeloma stage IIIA, and had been treated with combination chemotherapies including a VAD (vincristine, doxorubicin, and dexamethasone) regimen as well as an MP (melphalan and prednisone) regimen. About 3 years after finishing MP treatment, the patient presented with bone pain and a serum monoclonal protein level of 4,170 mg/dl. We initiated treatment with 200 mg thalidomide per day for 12 days and 40 mg dexamethasone per day for 4 days, and the dose of thalidomide was then increased to 400 mg per day. Eleven days after the boost, the patient’s course was complicated by the development of sloughing from the periorbital and perioral regions. Three days after the onset of the initial event, the patient was admitted to the emergency department with fever, dysphagia, and itching. The same day, oral mucositis, angioedema of lips and eyebrow, and diffuse erythematous to purpuric patches and plaques appeared on the face, neck, trunk, and extremities. On the third day of hospitalization, a skin biopsy showed subepidermal bullous change with an infiltration of lymphocytes and polymorphonuclear cells in the epidermis which suggested TEN (Fig. 1). On the fifth day, the clinical course was complicated by the worsening rash over the entire trunk, and the patient was started on 400 mg/kg intravenous immunoglobulin (IV-Globulin S®, Green Cross Inc., Korea) per day for five consecutive days. In addition, the patient was intensively treated as a burn patient. On the sixth day, focal skin detachment was noticed and, after 2 days, was followed by generalized detachment from the face to the lower extremities. The patient’s severity-ofillness score for TEN (SCORTEN) was 4 with an expected W. K. Eo : S. H. Cheon : S. H. Lee : J. S. Jeong Integrative Cancer Center, Kyung Hee University, East-West Neo Medical Center, Seoul, South Korea