Stevens‐Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are closely related, severe, episodic acute mucocutaneous intolerance reactions that may be elicited by certain drugs, including trimethoprimsulfamethoxazole (marketed as Bactrim or Bactrim DS) and other sulfonamide antibiotics .
As a practical matter, SJS and TEN may be considered to be two ends of a spectrum of the same disease, differing only in the degree and severity of skin detachment (SJS affects < 10% body surface area (BSA), while TEN involves > 30% BSA). The onset of SJS/TEN is sudden and constitutes a potentially fatal medical emergency requiring hospitalization in an ICU/burn unit. Notably, SJS/TEN is marked by rapid and progressive desquamation and skin sloughing.
The onset of SJS/TEN is typically characterized by a prodromal malaise lasting a few days and which includes fever, headache, sore throat, etc. These symptoms are generally followed by the appearance of a rash that may at first be limited, but then spreads to the rest of the body. The rash is paralleled or preceded by mucous membrane involvement, most often initially evidenced in the oral cavity, eyes and lips, and heralded by burning sensations, swelling and blistering. SJS/TEN is invariably accompanied by telling vital signs and abnormal laboratory findings. As SJS/TEN progresses (maximal disease expression @4‐5 days), the individual lesions comprising the rash rapidly increase in numbers and size. The epidermis within such lesions become loose and easily detachable. Sheets of necrotic epidermis slide off, leaving red, oozing erosions. The key to the success of the treatment of SJS/TEN is the rapid detection of the disease and the immediate initiation of appropriate care. The offending drug must be withdrawn without delay. As with burn patients, the loss of the epidermis in SJS/TEN situations necessitates the meticulous adjustment of fluid and electrolyte balance, nutritional support, wound care/dressing, pain management, and early detection and treatment of infection. Moreover, short‐term, high‐dose intravenous corticosteroids may lessen the severity of symptoms in adults and shorten recovery time if initiated within 24‐48 hours of the first sign of symptoms. Intravenous immunoglobulin therapy (IVIG) has similarly been demonstrated to halt the progression of SJS/TEN and lead to rapid re‐epithelialization. In addition to being potentially fatal, a delay in the detection and treatment of SJS/TEN may lead to serious complications, such as permanent skin damage, damage to internal organs and vision problems. As the lesions progress, a lack of protection to the fragile skin, i.e., the use of appropriate dressings to maintain the epithelial layer and the use of protective surfaces to prevent further shearing of the skin, as well as the institution of appropriate sterile techniques to reduce the risk of iatrogenic infection, all substantially increase the risk of further complications. Moreover, by maintaining the integrity of the epidermal layer, an appropriate biological dressing serves to enhance the healing process subsequently, reduce pain and minimize scarring.
Stevens-Johnson syndrome (SJS) / toxic epidermal necrolysis (TENS )medical expert witness specialties include burn medicine/surgery, dermatology, and emergency medicine.
IF YOU NEED A Stevens-Johnson Syndrome MEDICAL EXPERT, CALL MEDILEX AT (212) 234-1999.
Image courtesy of Nephron, Wikimedia Commons. This file is licensed under the Creative Commons Attribution-Share Alike 3.0 Unported license.