Acknowledgement of Lyell Syndrome has not been added yet.

Lyell Syndrome has an estimated incidence of 2/1,000,000 per year.

Name	Abbreviation
Toxic Epidermal Necrolysis	TEN
Steven Johnson Syndrome	SJS
Erythema Multiforme Major	EMM

The exact mechanism is not fully understood, but the immune system is known to be involved. It is thought to be a T cell mediated immune reaction altering skin proteins to be seen as foreign by the immune system. Skin cells are then attacked leading to widespread cell death. Once enough cell death occurs, the outer layer of the skin (epidermis) separates from the underlying dermis, resulting in the characteristic blistering and sloughing of the skin seen in Lyell Syndrome. In addition to the skin, the mucous membranes (mouth, genitals, eyes) are often affected due to similar immune-mediated destruction of epithelial cells (cells lining the surface). This can lead to difficulty swallowing, breathing issues, and severe pain. In severe cases, this immune-mediated destruction extends to internal organs, leading to multi-organ failure. The damage described is similar to severe burns, where the skin loses its protective function, increasing the risk of dehydration, infection, and systemic complications. The primary cause of Lyell Syndrome is a severe adverse reaction to medication. The drugs most commonly associated with triggering this reaction include: 

• Antibiotics (e.g., sulfonamides, penicillins)

• Antiepileptics (e.g., phenytoin, carbamazepine, lamotrigine)

• Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) (e.g., ibuprofen, naproxen)

• Allopurinol (used for gout)

• Antiretrovirals (used for HIV treatment)

Infections like Mycoplasma pneumoniae or vaccinations can trigger Lyell Syndrome as well, however drug reactions are the main cause. The following are risk factors for the SJS/TEN spectrum disorders:

• HIV infection- Among people with HIV, the incidence of SJS/TEN is about 100 times greater than that among the general population.

• A weakened immune system- The immune system can be affected by an organ transplant, HIV/AIDS and autoimmune diseases.

• Cancer- People with cancer, especially blood cancers (hematologic malignancies), are at increased risk of SJS/TEN.

• A history of SJS/TEN- If you've had a medication-related form of this condition, you are at risk of a recurrence if you use that drug again.

• A family history of SJS/TEN- If a first-degree relative, such as a parent or sibling, has had SJS/TEN, you may be more susceptible to developing it too.

• Genetic factors- Having certain genetic variations (HLA-B) puts you at increased risk of SJS/TEN, especially if you're also taking drugs for seizures, gout or mental illness.

• Begins with flu-like symptoms (fever, malaise)

• Progresses to widespread skin redness, peeling, and pain

• Large areas of skin peeling and detachment (>30% of body surface area)

• Blisters and sores around mucous membranes (eyes, mouth, genitals)

• Painful swallowing and urination

• Clinical evaluation:
  - examine skin and mucous membranes
  - level of pain
  - TBSA affected

• Lab testing: skin biopsy

• Immediate discontinuation of suspected causative agent

• Supportive care in specialized burn units or intensive care

• Fluid and electrolyte management

• Pain management

• Wound care and infection prevention

• Possible use of immunomodulatory therapies (e.g., IVIG, corticosteroids)

The prognosis for Lyell Syndrome (Toxic Epidermal Necrolysis, TEN) depends on several factors, including the extent of skin involvement, patient age, and overall health. It is a serious and life-threatening condition, but outcomes can improve with prompt diagnosis and treatment. The mortality rate ranges from 20% to 40%, depending on the severity of the disease and the care received. The risk is higher in older patients, those with extensive skin involvement (higher TBSA), and those with underlying health conditions. Septicemia (infection spread throughout the bloodstream) and multisystem organ failure are the primary causes of death. The re-epithelialization (outer skin layer regeneration) period lasts between one and three weeks, depending on the extent and severity of the clinical picture. High levels of care are needed during this period. Common complications include infection and multi-organ failure. Long-term complications include damage to the eyes, chronic skin/nail issues, difficulty swallowing, eating, and sexual dysfunction. Overall, many patients can survive with early and aggressive treatment, though the risk of complications remains significant. Survivors may experience a range of long-term sequelae, particularly affecting the skin, eyes, and mucous membranes. These chronic effects usually require ongoing symptomatic management.

Tips or Suggestions of Lyell Syndrome has not been added yet.

Augusto, J., De, R., Carlos Cunha Oliveira, & De, R. (2020). Lyell syndrome in 72% of the body surface. Revista Brasileira de Cirurgia Plástica, 35(2), 240–242. https://doi.org/10.5935/2177-1235.2020rbcp0039

Baroni, A., & Ruocco, E. (2005). Lyell’s Syndrome. SKINmed: Dermatology for the Clinician, 4(4), 221–225. https://doi.org/10.1111/j.1540-9740.2005.03593.x

Nunes, J., Santareno, S., Guerreiro, L., & Margalho, A. (2017). Lyell’s syndrome and antimalarials: A case report and clinical review. Journal of Global Infectious Diseases, 9(1), 23. https://doi.org/10.4103/0974-777x.199994

Toxic Epidermal Necrolysis (TEN): Causes, Symptoms & Treatment. (n.d.). Cleveland Clinic. https://my.clevelandclinic.org/health/diseases/21616-toxic-epidermal-necrolysis-ten