SJS/TEN and Na

Q: Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) usually causes? - select one

A) hypernatremic dehydration

B) hyponatremic dehydration

Answer: B

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a serious and potentially fatal mucocutaneous drug reaction, characterized by extensive necrosis and detachment of the epidermis due to massive keratinocyte apoptosis. It usually starts as a prodrome of fever and influenza-like symptoms followed in one to three days by an eruption of ill-defined, coalescing, erythematous macules with atypical target lesions. As the disease progresses, vesicles and bullae form, and the skin begins to slough within days. Mucosal involvement occurs in over 90 percent of cases.

Morbidity (and mortality) occurs due to massive loss of fluids, electrolyte imbalance, hypovolemic shock with hyponatremic dehydration, sepsis, and multiple system organ failure (MSOF). These patients are usually managed in a burn unit because of extensive skin detachment. 

#dermatology

References:

1. Hung CC, Liu WC, Kuo MC, Lee CH, Hwang SJ, Chen HC. Acute renal failure and its risk factors in Stevens-Johnson syndrome and toxic epidermal necrolysis. Am J Nephrol. 2009;29(6):633-8. doi: 10.1159/000195632. Epub 2009 Jan 21. PMID: 19155617.

2. Huang SC, Tsai SJ. Hyponatremia and Stevens-Johnson syndrome in a patient receiving carbamazepine. Gen Hosp Psychiatry. 1995 Nov;17(6):458-60. doi: 10.1016/0163-8343(95)90049-7. PMID: 8714810.

3. Shah H, Parisi R, Mukherjee E, Phillips EJ, Dodiuk-Gad RP. Update on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Diagnosis and Management. Am J Clin Dermatol. 2024 Nov;25(6):891-908. doi: 10.1007/s40257-024-00889-6. Epub 2024 Sep 15. PMID: 39278968; PMCID: PMC11511757.