This young man developed a diffuse bullous, erosive eruption days after using a new whitening tooth product. Unfortunately, the patient presented years ago before TNF agents were employed. He died.
This young man developed a diffuse bullous, erosive eruption days after using a new whitening tooth product. Unfortunately, the patient presented years ago before TNF agents were employed. He died.
Toxic Epidermal Necrolysis (TEN) is a potentially fatal skin disease in which much of the skin sloughs from the body.
The acute onset of malaise and fever followed by erythema and edema of the skin is characteristic. Some patients experience a sore throat, cough and burning eyes during the prodrome. Bulla then develop which enlarge until the epidermis sloughs off in large sheets. Mucous membrane crusting, pain and erosions occur. The patient becomes life-threateningly ill rapidly.
The four main clinical mimickers of SJS/TEN are DRESS, morbilliform drug eruption, erythema multiforme and AGEP. A positive Nikolsky's sign was present in 80% of SJS/TEN patients and none of DRESS or morbilliform drug eruptions, 7% of EM and 23% of AGEP in one large study. Typical target lesions are characteristic of EM whereas atypical target lesions are characteristic of SJS/TEN. A typical target lesion may be defined as less than 3 cm in diameter, round with well-defined borders and at least 3 distinct zones. An atypical target lesion may be defined as a 2-zoned, flat or elevated targetoid lesion with poorly defined borders with or without bulla/erosion.
A pencil eraser or finger may be used to test for Nikolsky sign. The skin is pulled to the side with a shearing pressure on the surface, or by rotating the eraser back and forth. If thin top layer of skin shears off, leaving the skin pink and moist, the test is positive.
An adult with diffuse desquamation of the skin is likely to have TEN, but the possibility of Staphylococcal scalded skin syndrome (SSSS) should not be ignored. A skin biopsy reliably distinguishes between the two and can be performed rapidly by frozen section. Histologic examination of the roof of a blister shows full thickness epidermis (in contrast to SSSS). Clinically, TEN often has mucosal involvement whereas SSSS does not; pustules are more characteristic of SSSS, and a positive Nikolsky's sign of uninvolved skin is more characteristic of SSSS. There is a TEN-like lupus syndrome so an ANA should be drawn. Of note, vancomycin-induced linear IgA bullous dermatosis can be of such acute onset and severe as to mimic toxic epidermal necrolysis.
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