| Toxic epidermal necrolysis | |
|---|---|
| Other names | Lyell syndrome, Lyell's syndrome[1] |
 | |
| Characteristic skin loss of toxic epidermal necrolysis | |
| Specialty | Dermatology |
| Symptoms | Fever, skin blisters, skin peeling, painful skin, red eyes[2] |
| Complications | Dehydration, sepsis, pneumonia, multiple organ failure.[2] |
| Usual onset | Age > 40[3] |
| Risk factors | HIV/AIDS, systemic lupus erythematosus, genetics[2] |
| Diagnostic method | > 30% of the skin involved, skin biopsy[3] |
| Differential diagnosis | Chickenpox, staphylococcal epidermolysis, staphylococcal scalded skin syndrome, autoimmune bullous disease[3] |
| Treatment | Hospitalization, stopping the cause, pain medication[3] |
| Prognosis | Mortality 20–50%[2][3] |
| Frequency | 1–2 per million per year (together with SJS)[2] |
Toxic epidermal necrolysis (TEN), also known as Lyell's syndrome, is a type of severe skin reaction.[2] Together with Stevens–Johnson syndrome (SJS) it forms a spectrum of disease, with TEN being more severe.[2] Early symptoms include fever and flu-like symptoms.[2] A few days later the skin begins to blister and peel forming painful raw areas.[2] Mucous membranes, such as the mouth, are also typically involved.[2] Complications include dehydration, sepsis, pneumonia, and multiple organ failure.[2]
The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics, and nevirapine.[2] Other causes can include infections such as Mycoplasma pneumoniae and cytomegalovirus or the cause may remain unknown.[3][4] Risk factors include HIV/AIDS and systemic lupus erythematosus.[2] Diagnosis is based on a skin biopsy and involvement of more than 30% of the skin.[3] TEN is a type of severe cutaneous adverse reactions (SCARs), together with SJS, a SJS/TEN, and drug reaction with eosinophilia and systemic symptoms.[5] It is called SJS when less than 10% of the skin is involved and an intermediate form with 10 to 30% involvement.[3] Erythema multiforme (EM) is generally considered a separate condition.[6]
Treatment typically takes place in hospital such as in a burn unit or intensive care unit.[3][7] Efforts include stopping the cause, pain medication, and antihistamines.[3][4] Antibiotics, intravenous immunoglobulins, and corticosteroids may also be used.[3][4] Treatments do not typically change the course of the underlying disease.[3] Together with SJS it affects 1 to 2 persons per million per year.[2] It is more common in females than males.[3] Typical onset is over the age of 40.[3] Skin usually regrows over two to three weeks; however, recovery can take months and most are left with chronic problems.[3][4]
- ^ Rapini, Ronald P., Bolognia, Jean L., Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
- ^ a b c d e f g h i j k l m n "Stevens-Johnson syndrome/toxic epidermal necrolysis". Genetics Home Reference. July 2015. Archived from the original on 27 April 2017. Retrieved 26 April 2017.
- ^ a b c d e f g h i j k l m n o "Orphanet: Toxic epidermal necrolysis". Orphanet. November 2008. Archived from the original on 27 April 2017. Retrieved 26 April 2017.
- ^ a b c d "Stevens-Johnson syndrome". GARD. Archived from the original on 28 August 2016. Retrieved 26 August 2016.
- ^ Adler NR, Aung AK, Ergen EN, Trubiano J, Goh MS, Phillips EJ (2017). "Recent advances in the understanding of severe cutaneous adverse reactions". The British Journal of Dermatology. 177 (5): 1234–1247. doi:10.1111/bjd.15423. PMC 5582023. PMID 28256714.
- ^ Schwartz RA, McDonough PH, Lee BW (August 2013). "Toxic epidermal necrolysis: Part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis". Journal of the American Academy of Dermatology. 69 (2): 173.e1–13, quiz 185–6. doi:10.1016/j.jaad.2013.05.003. PMID 23866878.
- ^ Schwartz RA, McDonough PH, Lee BW (August 2013). "Toxic epidermal necrolysis: Part II. Prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment". Journal of the American Academy of Dermatology. 69 (2): 187.e1–16, quiz 203–4. doi:10.1016/j.jaad.2013.05.002. PMID 23866879.