Source: This post on Toxic epidermal necrolysis (TEN) has been created based on the article “Australian-German research finds world first cure for deadly skin disease” published in Assam Tribune on 13th November 2024.
Why in news?
Researchers from Australia and Germany have achieved a groundbreaking medical milestone, developing the first cure for toxic epidermal necrolysis (TEN).
About the cure
1. Experts identified that the JAK-STAT signalling pathway is a cellular process integral to immune response, cell death, and tumour formation and is hyperactivated in TEN patients.
2. By employing JAK inhibitors, a drug class typically used to treat inflammatory diseases, researchers successfully treated seven patients with TEN.
About toxic epidermal necrolysis (TEN)
1. Toxic epidermal necrolysis (TEN) is a severe, life-threatening skin reaction characterized by widespread skin necrosis and detachment of the epidermis.
2. Cause: Most commonly caused by reactions to medications, including antibiotics (e.g., sulfonamides), anticonvulsants, nonsteroidal anti-inflammatory drugs (NSAIDs), and allopurinol. Less frequently, infections or other factors may trigger TEN.
3. Symptoms: High fever and flu-like symptoms often precede skin lesions, Rapid development of red, painful skin areas that evolve into large blisters and peeling, extensive skin detachment, often covering more than 30% of body surface area and mucous membrane involvement, such as in the mouth, eyes, and genitals.
4. Diagnosis: Based on clinical appearance and patient history of recent medication use. Skin biopsy can confirm the diagnosis, showing characteristic necrosis of the epidermis.
5. Complications:
i) Severe fluid loss and electrolyte imbalance due to extensive skin loss.
ii) Increased risk of infections (especially sepsis) due to loss of the skin barrier.
iii) Long-term complications can include scarring, pigmentation changes, and eye damage (such as blindness).
6. Treatment: Immediate hospitalization, often in an intensive care unit or burn unit, Discontinuation of the suspected drug(s) and supportive care for skin lesions, including fluid replacement, pain management, and wound care.
Immunosuppressive therapies (e.g., intravenous immunoglobulins or corticosteroids) may be considered, though their efficacy is debated.
7. Prognosis: The mortality rate for TEN can be high, especially if diagnosis and treatment are delayed. The SCORTEN score is often used to predict prognosis based on several clinical factors.
8. Prevention: Genetic testing (such as for HLA-B*1502 allele) may help prevent TEN in susceptible populations when prescribing certain drugs (e.g., carbamazepine in Asian populations).
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