Dermatology - Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)
SJS and toxic TEN are both severe and potentially fatal mucocutaneous reactions marked by widespread necrosis and separation of the epidermis. SJS and TEN are different forms of the same disease, which might be caused by unknown factors or drugs. The main difference between them is the extent to which the body surface is affected. The interval between initial drug exposure and the appearance of symptoms typically ranges from 1 to 3 weeks, but this is more frequently observed with subsequent exposure. Prior to the eruption, there is commonly a prodrome characterized by fever, malaise, and arthralgia that typically lasts for 1-3 days. Malnutrition, sensitivity to light, uncomfortable urination, and anxiety may manifest. Abnormalities The individual experiences a mild to moderate sensation of tenderness in the skin, along with a burning or itchy feeling in the conjunctiva. This is followed by skin pain, a burning sensation, tenderness, and paresthesia. Oral ulcers cause significant discomfort and sensitivity. The initial rash is characterized by a morbilliform appearance and may exhibit target-like patterns, with or without the presence of purplish discoloration. The lesions quickly merge together; alternatively, there may be no distinct lesions, but instead a widespread redness and absence of a rash. As the rash advances, the outer layer of the skin undergoes necrosis, resulting in wrinkled patches that grow in size and merge together. Subsequently, there is a detachment of the outer layer of the skin, known as the epidermis, in a sheet-like manner. This is accompanied by the formation of soft and raised blisters that can spread when pressure is applied sideways (known as the Nikolsky sign) on reddened parts of the skin. Following trauma, the skin experiences a complete separation of the outermost layer, resulting in the exposure of the underlying dermis. This exposed dermis appears red and is accompanied by leaking, similar to a second-degree burn caused by heat. The lips, buccal mucosa, conjunctiva, and vaginal and anal skin are consistently affected. The eyes exhibit conjunctival diseases characterized by hyperemia, pseudomembrane development, keratitis, corneal erosions, and subsequent adhesions between the eyelids and bulbar conjunctiva. The differential diagnosis comprises drug eruptions, erythema multiforme, scarlet fever, phototoxic eruptions, toxic shock syndrome, graft-versus-host disease, thermal burns, staphylococcal scalded-skin syndrome (rare in adults but present in young children), fixed drug eruption, and exfoliative dermatitis. Prompt identification and cessation of potentially implicated medication(s) are of utmost significance. Patients receive optimal care in an intermediate or intensive care unit. Administer IV fluids and electrolytes in a manner consistent with patients suffering from a severe third-degree heat burn. However, a smaller amount of fluid is typically needed for a thermal burn of same size. Administering systemic glucocorticoids during the onset of the disease can be beneficial in minimizing the occurrence of illness or death, particularly when given in large quantities. In the advanced stages of the condition, they are not recommended. Administering a large amount of immunoglobulin through an intravenous route during the initial phases. It is not advisable to have surgical debridement. Early treatment of ocular lesions with erythromycin ointment is recommended.
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