Background. High-dose intravenous immunoglobulin (IVIG)is emerging as a promising new therapy for treating the rare but potentially fatal drug reaction toxic epidermal necrolysis (TEN). Experimental in vitro studies s...Background. High-dose intravenous immunoglobulin (IVIG)is emerging as a promising new therapy for treating the rare but potentially fatal drug reaction toxic epidermal necrolysis (TEN). Experimental in vitro studies support that IVIG can block the Fas-FasL-mediated apoptosis in TEN. Methods. Twelve consecutive patients (7M, 5F) with TEN admitted over a 5-year period from January 1998 to December 2002 were treated with a dose of 0.5-1.0 g/kg/d of IVIG for 45 days along with standard care protocol. Clinical outcome in terms of average duration to arrest the progression, complete healing, hospital stay, side-effects and complications were determined to find the efficacy of IVIG treatment. Results. Average age was 27.16 years (7-50 years). There were four children (2M, 2F) aged 7-12 years. One patient had an underlying malignancy. No patient had HIV infection. The average total body surface area involvement was 57.5%(30-90%). An IVIG infusion was started, on average, 1.58 days (1-3 days) after admission. All patients responded well to the treatment. There was no mortality. The disease progression was arrested in a mean of 2.83 days (1-5 days). Time taken for complete healing (re-epithelialization)was 7.33 days (5-13 days). The average duration of hospital stay was 12.5 days (7-21 days). No side-effects of the IVIG treatment were observed in these patients. The drugs triggering TEN in these patients were phenytoin (four patients), followed by penicillin (three), cotrimoxazole (two), phenobarbital and furosemide (one patient each), respectively. In one patient, the offending drug could not be ascertained. Conclusion. Our experience of treating 12 patients with TEN using IVIG, in Kuwait, confirms that it is a safe and effective treatment for these patients.展开更多
Olmsted syndrome is a rare disorder characterized by the combination of periorificial keratotic plaques and bilateral palmoplantar keratoderma. New associated features continue to be reported. Olmsted syndrome in a fe...Olmsted syndrome is a rare disorder characterized by the combination of periorificial keratotic plaques and bilateral palmoplantar keratoderma. New associated features continue to be reported. Olmsted syndrome in a female patient is particularly rare, and we report such a case having a haemangioma in addition to other features of the syndrome.展开更多
文摘Background. High-dose intravenous immunoglobulin (IVIG)is emerging as a promising new therapy for treating the rare but potentially fatal drug reaction toxic epidermal necrolysis (TEN). Experimental in vitro studies support that IVIG can block the Fas-FasL-mediated apoptosis in TEN. Methods. Twelve consecutive patients (7M, 5F) with TEN admitted over a 5-year period from January 1998 to December 2002 were treated with a dose of 0.5-1.0 g/kg/d of IVIG for 45 days along with standard care protocol. Clinical outcome in terms of average duration to arrest the progression, complete healing, hospital stay, side-effects and complications were determined to find the efficacy of IVIG treatment. Results. Average age was 27.16 years (7-50 years). There were four children (2M, 2F) aged 7-12 years. One patient had an underlying malignancy. No patient had HIV infection. The average total body surface area involvement was 57.5%(30-90%). An IVIG infusion was started, on average, 1.58 days (1-3 days) after admission. All patients responded well to the treatment. There was no mortality. The disease progression was arrested in a mean of 2.83 days (1-5 days). Time taken for complete healing (re-epithelialization)was 7.33 days (5-13 days). The average duration of hospital stay was 12.5 days (7-21 days). No side-effects of the IVIG treatment were observed in these patients. The drugs triggering TEN in these patients were phenytoin (four patients), followed by penicillin (three), cotrimoxazole (two), phenobarbital and furosemide (one patient each), respectively. In one patient, the offending drug could not be ascertained. Conclusion. Our experience of treating 12 patients with TEN using IVIG, in Kuwait, confirms that it is a safe and effective treatment for these patients.
文摘Olmsted syndrome is a rare disorder characterized by the combination of periorificial keratotic plaques and bilateral palmoplantar keratoderma. New associated features continue to be reported. Olmsted syndrome in a female patient is particularly rare, and we report such a case having a haemangioma in addition to other features of the syndrome.
