Symptoms, what causes them?

syndrome of Lyell An acute, very rare and serious disease, which leaves reaction in the patient. “We estimate 2 to 6 cases per million inhabitants per year in the world. It also affects adult As children as well old. However, the average age of patients is Between 40 and 50 years“ Professor Saskia Oro, dermatologist and venereologist at the Henri-Mondor Hospital, explains the coordination site of the French reference center for toxic bullous dermatosis and severe toxic dermatosis.

What is Lyell Syndrome?

“Lyell’s syndrome is a disease of the skin and mucous membranes. It affects the uppermost layer of the skin as well as the oral, ocular or genital mucous membranes. The skin lesions are more or less widespread. Sometimes, they extend to the entire body”

Our interlocutor explains.

What is the difference with Stevens-Johnson syndrome?

Toxic epidermal necrolysis (NE) includes Lyell syndrome (toxic NE) and Stevens-Johnson syndrome. “It’s the same disease, with the same processes involved. However, we’re talking about it Stevens-Johnson syndrome when less than 10% of the body surface area is affected and syndrome Lyell or toxic epidermal necrolysis when the lesion is concerned More than 30% of body surface. Between the two, it’s an overlap syndrome”, The doctor answers.

What causes Lyell syndrome?

In 85% of cases, the cause of Lyell’s syndrome is allergy. MedicinalOften difficult to predict. “The patient’s body recognizes the drug abnormally, causing it Violent, sudden, severe and unexpected allergies.

In 85% of cases, the cause of Lyell’s syndrome is a drug allergy

Allergy activates cytotoxic T lymphocytes, leading to the production of protein, especially granulysin. The latter is Toxic to epidermisAnd it is he who is responsible for it Destruction of the superficial layer of the skin. In summary, we have an unusual recognition of a specific drug by cytotoxic T lymphocytes that leads to an acute inflammatory response.” According to dermatologists, certain populations are present Genetic susceptibility Especially with certain medications. This is the case for native people Asian

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The Three major families of drugs Those at high risk of Lyell syndrome are known to:

• Antiepileptics: carbamazepine, phenobarbital, phenytoin and lamotrigine
• Allopurinol: Treatment for gout
• Sulfonamide Antibiotics

Other drugs are at risk, such as nevirapine, non-steroidal anti-inflammatory drugs (NSAIDs), and proton pump inhibitors. “These are the key molecules that cause Lyell syndrome, even though in theory, All medicines can cause allergies” More rarely, a bacterium The Mycoplasma family is involved. “Lyle syndrome due to bacterial infection is very rare in children, and very rare in adults.” The Autoimmune diseases There is also a possible cause. Finally, “Sometimes, no cause is found, then we talk about idiopathic Lyell syndrome”.

What are the symptoms?

Symptoms of Lyell syndrome begin within a few days. No Small purple spots Purpuric (which does not disappear with pressure) appears on the skin. They are often very painful. “The patient often presents with fever As well as a change in its normal state. No Initial soreness of mucous membrane in mouth And then the eyes arise. This is the most common clinical picture. Professor Saskia Oro explains. Then, usually, the topmost layer of skin, the epidermis, Raises and forms blisters (bubbles).

Meanwhile Epidermal detachment is an opening of the skin. Symptoms are very painful for the patient, whether on the skin or mucous membranes, to the point that they have difficulty eating.

There is no cure for quick recovery

The ideal is to treat the patient at a regular center to increase his chances of recovery. “This disease is rare and serious; It is better to transfer the patient to a regular centre.” contains treatment to wait That wounds heal on their own, for that Spontaneous healing in 10 days Approximately “We don’t have any treatment to cure the disease quickly.” At the same time, the treatment includes Compensation for all body failures associated with the absence of epidermis Significantly:

• Dehydration: by hyperhydration;
• Calorie loss, especially protein and vitamin loss: tube feeding;
• Heat loss: the environment heats up;
• Tachycardia (heart beats too fast);
• fever

“In case of proven bacterial infection, the patient is treated with antibiotics. Ointments and creams are applied to the skin and eyes every two to four hours.” Some patients suffer from severe respiratory and ENT problems. Intensive care treatment is then required. “We put the patient under sedation and intubation to treat them better. Severe cases affect about a third of patients. Length of stay in intensive care varies from a few days to a few weeks.”

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Lyell syndrome is a serious illness that can be life-threatening: “In the first 6 weeks there is a risk of death in the acute phase Between 15 and 20%“

Lyell syndrome: what are the after effects?

Lyell syndrome is a disease that sometimes leaves serious aftereffects. The patient’s life is often turned upside down, especially in the event of ophthalmological sequelae. “The after effects meanwhileEye damage The acute phase is more or less severe, such as dry eyes, discomfort with light or driving at night. Some disorders are more disabling, potentially leading to blindness. Other after effects exist, viz Psychological after-effects, With the fear of taking the drug again, a worry Chronic, and sometimes, Depression. It is a disease of which Patients do not leave unscathed. In fact, at least two-thirds of patients have after-effects that can last for years.” Care is necessary during the acute phase, but support is essential during the chronic phase. “Associative support is possible, thanks to the AMALYSTE association or blogs of former patients. Professor Oro concludes.

Thanks to Professor Saskia Oro, Dermatologist and Venereologist at the Henri-Mondor Hospital, coordinating site of the French reference center for toxic bullous dermatosis and severe toxic skin toxicbull

• Management of Lyell syndrome or toxic epidermal necrolysis – NIH
• Toxibull