Description

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Context

Researchers from Australia and Germany have made a significant medical breakthrough by developing the first-ever cure for Toxic Epidermal Necrolysis (TEN), a rare and potentially fatal skin disease.

This international study involved collaboration between the Walter and Eliza Hall Institute of Medical Research (WEHI) in Melbourne and the Max Planck Institute of Biochemistry in Germany.

About the Study

The researchers discovered that hyperactivation of the JAK-STAT signaling pathway is a major driver of TEN.

The JAK-STAT signaling pathway is a chain of protein interactions that transmits extracellular signals to the nucleus of a cell, causing changes in DNA transcription.

It plays a central role in cell function and regulates many cellular processes, including immunity, cell division, cell death, tumor formation, proliferation, migration, and differentiatio

The team used JAK inhibitors, a class of drugs already in use for inflammatory diseases, to treat patients with TEN.

What is Toxic Epidermal Necrolysis (TEN)?

Toxic Epidermal Necrolysis (TEN) is a very rare, but serious skin condition where the top layer of the skin, called the epidermis, detaches from the layers below.

TEN, also known as Lyell's Syndrome, is characterized by:

Widespread blistering and skin detachment, resembling large burns.

It often leads to dehydration, infection (sepsis), pneumonia, and organ failure.

Triggered by adverse reactions to common medications.

Has a high mortality rate of around 30%.

Causes of Toxic Epidermal Necrolysis

TEN is mainly caused by an allergic reaction to certain medications, which can include:

Antibiotics (e.g., sulfonamides and penicillins)

Anticonvulsants (used to treat seizures)

Pain relievers (e.g., ibuprofen, acetaminophen)

Treatment

Since TEN affects large parts of the skin, treatment often takes place in a hospital’s burn unit or intensive care unit (ICU). Key treatments include:

Stopping the medication causing the reaction.

Fluids and electrolytes to prevent dehydration.

Pain management to relieve discomfort.

Special wound care to prevent infections and help the skin heal.

In some cases, patients may receive immunosuppressive drugs to control the immune system’s reaction.

What is the Epidermis?

The epidermis is the outermost layer of the skin, acting as the body’s first line of defense. This layer protects the body from germs, injuries, and harmful substances. The epidermis is very thin — often about as thin as a sheet of paper — yet it plays a crucial role in keeping us safe and healthy.

The epidermis regenerates every 27–30 days, which is why minor cuts and scrapes heal so quickly.

Cells in the Epidermis

The epidermis contains different types of cells, including:

Keratinocytes are the main cells, making up about 90% of the epidermis. They produce keratin, a protein that strengthens the skin.

Melanocytes cells create melanin, which gives skin its color and helps protect it from the sun. Melanin levels determine skin color: more melanin means darker skin, which provides better protection against UV rays.

Langerhans Cells are part of the immune system and help defend against germs.

Merkel Cells are involved in the sense of touch.

Sources:

AssamTribune

PRACTICE QUESTION

Q.Consider the following types of skin cells and their primary functions:

Keratinocytes:: Produce a protective barrier.
Melanocytes:: Generate pigments to protect against UV radiation.
Langerhans cells:: Provide structural support to the skin.
Merkel cells:: Involved in the sensory perception of touch.

Which of the above cell types is/are correctly matched with their function?

A. 1 and 2 only
B. 1, 2, and 4 only
C. 1, 2, and 3 only
D. All of the above

Answer: B

Explanation:

Statement 1 is correct. Keratinocytes are the most common cell type in the epidermis. They produce keratin, a protein that provides strength to the skin, hair, and nails, and helps form a protective barrier against environmental damage.

Statement 2 is correct. Melanocytes are cells located in the basal layer of the epidermis that produce melanin, which provides pigment to the skin and helps protect against UV radiation.

Statement 3 is incorrect. Langerhans cells are not responsible for structural support. They are part of the immune system, detecting pathogens and presenting antigens to other immune cells to mount an immune response.

Statement 4 is correct. Merkel cells are involved in the sensation of touch, particularly in areas like the fingertips. They work as mechanoreceptors, responding to light touch.