Drug-induced Stevens-Johnson syndrome/toxic epidermal necrolysis

被引:131
作者
Fritsch P.O. [1 ,2 ]
Sidoroff A. [1 ]
机构
[1] Department Dermatology, University of Innsbruck, Innsbruck
[2] Department of Dermatology, University of Innsbruck, A 6020 Anichstraße 35, Innsbruck
关键词
Body Surface Area; Toxic Epidermal Necrolysis; Erythema Multiforme; Causative Drug; Acute Graft Versus Host Disease;
D O I
10.2165/00128071-200001060-00003
中图分类号
学科分类号
摘要
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare (occurring in approximately 2 to 3 people/million population/year in Europe and the US), life-threatening, intolerance reaction of the skin. It is most often caused by drugs (most commonly sulfonamides, nonsteroidal anti-inflammatory drugs, antimalarials, anticonvulsants, and allopurinol). SJS/TEN is characterized by a macular exanthema ('atypical targets') which focusses on the face, neck, and the central trunk regions. Lesions show rapid confluence, a positive Nikolsky's sign, and quickly result in widespread detachment of the epidermis and erosions. Mucosal, conjunctival, and anogenital mucous membranes are prominently involved. Histopathology shows satellite cell necrosis in the early stages progressing to full thickness necrosis of the epidermis, contrasting with rather inconspicuous inflammatory infiltrates of the dermis. Damage to the skin is thought to be mediated by cytotoxic T lymphocytes and mononuclear cells which induce apoptosis in keratinocytes expressing drug-derived antigens at their surfaces. No guidelines for the treatment of SJS/TEN exist since no controlled clinical trials have ever been performed. The controversy over whether systemic corticosteroids should be used to curtail progression is still unresolved; while many authors agree that corticosteroids do in fact suppress progression, it is obvious that they also greatly enhance the risk of infection, the complication which most frequently leads to a fatal outcome. It appears reasonable to only administer corticosteroids in the phase of progression and to withdraw them as soon as possible, and to add antibacterials for prophylaxis. Recently, in a small series of patients, intravenous immunoglobulins were presumed to be effective by the blockade of lytic Fas ligand-mediated apoptosis in SJS/TEN. However, these results have to be confirmed by large clinical trials. Supportive treatment and monitoring of vital functions is of utmost importance in SJS/TEN, and out-patient treatment is unacceptable. Recovery is usually slow, depending on the extent and severity and the presence of complications, and may take 3 to 6 weeks. Skin lesions heal without scars as a rule, but scarring of mucosal sites is a frequent late complication, potentially leading to blindness, obliteration of the fornices and anogenital strictures. There is no reliable laboratory test to determine the offending drug; diagnosis rests on the patient's history and the empirical risk of drugs to elicit skin SJS/TEN. Provocation tests are not indicated since re-exposure is likely to elicit a new episode of SJS/TEN of increased severity.
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页码:349 / 360
页数:11
相关论文
共 73 条
[41]  
Kakourou, T., Klontza, D., Soteropoulou, F., Corticosteroid treatment of erythema multiforme major (Stevens-Johnson syndrome) in children (1997) Eur J Pediatr, 156 (2), pp. 90-93
[42]  
Stables, G.I., Lever, R.S., Toxic epidermal necrolysis and systemic corticosteroids (1993) Br J Dermatol, 128 (3), p. 357
[43]  
Parsons, J.M., Toxic epidermal necrolysis (1992) Int J Dermatol, 31 (11), pp. 749-768
[44]  
Revuz, J., Penso, D., Roujeau, J.C., Toxic epidermal necrolysis. Clinical findings and prognosis factors in 87 patients (1987) Arch Dermatol, 123 (9), pp. 1160-1165
[45]  
Roujeau, J.C., Stern, R.S., Severe adverse cutaneous reactions to drugs (1994) N Engl J Med, 331 (19), pp. 1272-1285
[46]  
Halebian, P.H., Corder, V.J., Madden, M.R., Improved burn center survival of patients with toxic epidermal necrolysis managed without corticosteroids (1986) Ann Surg, 204 (5), pp. 503-512
[47]  
Roujeau, J.C., Drug-induced toxic epidermal necrolysis. II. Current aspects (1993) Clin Dermatol, 11 (4), pp. 493-500
[48]  
Rzany, B., Schmitt, H., Schöpf, E., Toxic epidermal necrolysis in patients receiving glucocorticosteroids (1991) Acta Derm Venereol, 71 (2), pp. 171-172
[49]  
Guibal, F., Bastuji-Garin, S., Chosidow, O., Characteristics of toxic epidermal necrolysis in patients undergoing long-term glucocorticoid therapy (1995) Arch Dermatol, 131 (6), pp. 669-672
[50]  
Egan, C.A., Grant, W.J., Morris, S.E., Plasmapheresis as an adjunct treatment in toxic epidermal necrolysis (1999) J Am Acad Dermatol, 40 (3), pp. 458-461