By Gary M. White, MD
The skin comes off in sheets.
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.
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 [JAAD Dec 2013] so an ANA should be drawn. Of note, vancomycin-induced LIGABD can be of such acute onset and severe as to mimic toxic epidermal necrolysis.
Treatment is often carried out in a burn unit. The offending medication should be stopped immediately. It has been shown that early cessation of the causative drug improves survival. Meticulous skin and mucosal care, fluid balance and temperature maintenance should all be performed. Infection is common and may lead to sepsis and death. Specialists in ophthalmology, plastic surgery, nutrition and wound care are important. Various topical dressings may be used, the most common being petrolatum-impregnated gauze and non adherent dressings with nanocrystalline silver [JAAD 2016;74;379].
Clinicians have struggled to find a medication that effectively treats this life-threatening disease. For many years, oral steroids were given, but results were not impressive and some studies suggested steroids worsened the prognosis. Then, IVIG became the front runner. Subsequently, various studies have suggested cyclosporin is superior to IVIG [JAAD 2014;71:941–947]. Just recently, etanercept has been brought to the forefront as possibly the treatment of choice for TEN. In this author's opinion--based upon non-randomized studies and case reports--either cyclosporin or etanercept (and possibly infliximab) are the current first choice treatment options for TEN.
Several case reports and small studies have suggested that either oral or IV CSA at a dose of 3-5 mg/kg/day can cease blister formation within 24-48 hours. Various studies have suggested cyclosporin is superior to IVIG in terms of reducing mortality [JAAD 2014;71:941–947]. CSA at a dose of 3–5 mg/kg daily IV or oral for a duration of 8–24 days (till re-epithelialisation) followed by weaning off the dose in another 2 weeks is recommended [JAAD 2017;76;106].
Etanercept 50 mg subcutaneous injection within 6 hours of admission was used successfully in a study of 10 patients, none of whom died. All patients promptly responded to treatment, reaching complete reepithelialization without complications or side effects. The median time to healing was 8.5 days [J Am Acad Dermatol 2014 Aug;71:278-83].
See example orders for acute blistering.
TEN in a child from Sulfa.
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