Toxic epidermal necrolysis: a study of 22 cases

Abstract

Toxic epidermal necrolysis leads to extensive exfoliative epidermal slough, fever, systemic toxic reactions, conjunctivitis and severe mueous membrane involvement. As it evolves many other organs can be affected. Whether or not toxic epidermal necrolysis is the most severe form of erythema multiform is still a subject of discussion. The pathophysiological events involved are not well understood. Indirect evidence suggests a hyperscnsitivity reaction, but the search for potential immunoiogical mechanisms has resulted in little data to support this hypothesis. Drug reactions remain the most common associated factor. Twenty-two patients, 3-84 years old, have been included in this retrospective study. In 15 patients, clinical evidence points to drugs as the most important cause of toxic epidermal necrolysis. Recent infections were implicated in three patients and ulcerative colitis and lymphoma in one case each. Symptomatic therapy included fluid replacement, nutritional support and local treatment. Steroids were administered in 13 patients, followed by plasmapheresis in three. The mortality rate was approximately 27%. Elderly patients and patients with extensive lesions were at greater risk. Results of immunofluorescence and immunoblot analysis were of no significant interest.

A Study of the Efficacy of Plasmapheresis for the Treatment of Drug Induced Toxic Epidermal Necrolysis

“…Since 1984, when Gerard ( 5 ) reported the first successful treatment of a drug induced TEN patient with plasmapheresis, 22 drug induced TEN patients treated with plasmapheresis have been reported in the literature (2,3,(5)(6)(7)(8)(9)(10). The effectiveness of plasmapheresis for the treatment of drug induced TEN was apparent not only in our case but also in most of the reports.…”

“…The pathogenesis of TEN is still unknown, and objective laboratory criteria for diagnosis are lacking. Nevertheless, a large number of etiological factors such as drugs, viral and bacterial infections, neoplastic diseases, etc., have been proposed for the etiology of TEN (2). In this report, we describe a case of drug induced TEN successfully treated with double filtration plasmapheresis (DFPP) and also summarize it and 22 other drug induced TEN cases reported in the literature as having been treated with plasmapheresis, commenting on the efficacy of DFPP in the treatment of TEN.…”

“…Histologically, it is distinguished by necrosis of the entire thickness of the epidermis (1). TEN is often a life threatening disorder, and the mortality rate is high (2). The pathogenesis of TEN is still unknown, and objective laboratory criteria for diagnosis are lacking.…”

Status of Plasmapheresis for the Treatment of Toxic Epidermal Necrolysis in Japan

“…In the English‐based literature, mortality in a small series of TEN patients treated with PE is very low. The number of patients who did not survive was: 0 out of 5 patients reported by Kamanabroo (6); 1 out of 5 by Sakellario (7); 0 out of 7 by Chaidemos (8); 0 out of 6 by Egan (9); and 1 out of 8 by Furubacke (10); while the mortality rate in a slightly larger series of TEN patients treated with PE was 27.3% (6/22) reported by Ioannides (2) and 23.1% (3/13) by Bamichas (20). These values are almost the same as our results.…”

“…Histologically, it is distinguished by necrolysis of the entire thickness of the epidermis (1). TEN is often a life threatening disorder and the mortality rate is high (2). The pathogenesis of TEN is still unknown and the standard therapy for TEN has not yet been established.…”