Toxic epidermal necrolysis (TEN) is a severe drug-induced bullous eruption that has significant mortality and morbidity.1 Induction of Fas ligand, a cytokine that induces apoptosis, may mediate cell death in TEN in part because the soluble Fas ligand (sFasL) level is elevated in the blood of patients with TEN.2 Pooled human immunoglobulin blocks the action of Fas and may be beneficial in TEN.2 The kinetics and specificity of sFasL induction in TEN and other bullous dermatoses are unknown. We present preliminary results of serial sFasL measurement in a patient with TEN and 10 patients with 5 types of chronic bullous diseases.
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Clinical and histologic findings of a patient with toxic epidermal necrolysis. A, Erosion on the lips and hard palate and scattered bullae with erythematous macules were noted on the first day of the skin eruption. B and C, The erythematous macules on the trunk and soles progressed to tense bullae by day 2. By day 7 oral lesions had healed (D) and most of the bullae were covered by hemorrhagic crust and had begun reepithelialization (E). F, Skin with subepidermal split (arrow) and an overlying area of confluent epidermal necrosis (arrowhead) (hematoxylin-eosin, original magnification ×100). The dermis shows only a sparse lymphohistiocytic infiltrate.
Serologic studies of soluble Fas ligand (sFasL). A, Kinetics of sFasL in toxic epidermal necrolysis. Concentration of sFasL is shown on the left axis and the extent of total body surface area (TBSA) involved by toxic epidermal necrolysis is estimated on the right axis. sFasL was measured in triplicate, and the mean values are shown. The SD ranges from 15.2% to 20% of the mean value. Arrowheads signify the times when immunoglobulin (IVIG) was infused. The upper limit of normal level of sFasL (0.1 ng/mL) is shown by the dashed line. B, Specificity of sFasL in autoimmune bullous diseases. Shown are serum levels of sFasL in a normal donor; patients with bullous pemphigoid (BP); subepidermal, lamina lucida blistering disease (Chan disease)4; cicatricial pemphigoid (CP); epidermolysis bullosa acquisita (EBA); linear IgA bullous dermatosis (LABD); and pemphigus vulgaris (PV). Dashed line denotes upper limit of normal level of sFasL.
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