Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Toxic epidermal necrolysis (TEN), also known as Lyell’s syndrome, is a life-threatening dermatological condition that is frequently induced by a reaction to medications.[1] It is characterized by the detachment of the top layer of skin (the epidermis) from the lower layers of the skin (the dermis) all over the body.
There is broad agreement in medical literature that TEN can be considered a more severe form of Stevens-Johnson syndrome, and debate whether it falls on a spectrum of disease that includes erythema multiforme.[2][3] Some authors consider that there is an overlap between the two syndromes (usually between 10% and 30% of skin detachment).
The incidence is between 0.4 and 1.2 cases per million each year.[1]
Microscopically, TEN causes cell death throughout the epidermis. Keratinocytes, which are the cells found lower in the epidermis, specializing in holding the skin cells together, undergo necrosis (cell death).
Toxic epidermal necrolysis is a rare and usually severe adverse reaction to certain drugs. History of medication use exists in over 95% of patients with TEN.[1] The drugs most often implicated in TEN are antibiotics such as sulfonamides; nonsteroidal anti-inflammatory drugs; allopurinol, antiretroviral drugs; and corticosteroids; and anticonvulsants such as phenobarbital, phenytoin, carbamazepine, and valproic acid.[1] If the condition results from a negative reaction to medication, the body forces that medication out of the system by way of depositing it within the outer layers of the skin. The condition might also result from immunizations, infection with agents such as Mycoplasma pneumoniae or the herpes virus; and transplants of bone marrow or organs.[1]
TEN affects many parts of the body, but it most severely affects the mucous membranes, such as the mouth, eyes, and vagina. The severe findings of TEN are often preceded by 1 to 2 weeks of fever. These symptoms may mimic those of a common upper respiratory tract infection. When the rash appears it may be over large and varied parts of the body, and it is usually warm and appears red. The dermal layer fills with fluid being deposited there by the body’s immune system, usually as a result of a negative reaction to an antibiotic. The skin then begins to sag from the body and can be peeled off in great swaths. The mouth becomes blistered and eroded, making eating difficult and sometimes necessitating feeding through a nasogastric tube through the nose or a gastric tube directly into the stomach. The eyes are affected, becoming swollen, crusted, and ulcerated and blindness may occur.
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