Toxic epidermal necrolysis (TEN), also known as Lyell's syndrome, is a type of severe skin reaction.
[2] A few days later the skin begins to blister and peel forming painful raw areas.
Before these severe findings develop, people often have a flu-like prodrome, with a cough, runny nose, fever, decreased appetite and malaise.
A history of drug exposure exists on average 14 days (ranging from 1–4 weeks) prior to the onset of symptoms, but may result as early as 48 hours if it is a reexposure.
[8] Initial skin findings include red-purple, dusky, flat spots known as macules that start on the trunk and spread out from there.
[10] Those who survive the acute phase of TEN often develop long-term complications affecting the skin and eyes.
Skin manifestations can include scarring, eruptive melanocytic nevi, vulvovaginal stenosis, and dyspareunia.
These can include dry eyes, photophobia, symblepharon, corneal scarring or xerosis, subconjunctival fibrosis, trichiasis, decreased visual acuity, and blindness.
Contrast agents used in imaging studies as well as transplantation of bone marrow or organs have also been linked to TEN development.
Other agents, including tumor necrosis factor alpha and Fas ligand, also appear to be involved in TEN pathogenesis.
The presence of oral, ocular, and/or genital mucositis is helpful diagnostically, as these findings are present in nearly all patients with TEN.
In more advanced TEN, full thickness epidermal necrosis is visualized, with a subepidermal split, and scant inflammatory infiltrate in the papillary dermis.
[7] Larger, high quality trials are needed to assess the actual benefit of IVIG in TEN.
[11] Loss of the skin leaves patients vulnerable to infections from fungi and bacteria, and can result in sepsis, the leading cause of death in the disease.
[23] One point is given for each of the following factors:[12] Of note, this scoring system is most valuable when used on the first and third day of hospitalization, and it may underestimate mortality in patients with respiratory symptoms.