People with Stevens-Johnson syndrome require treatment in a hospital due to the life threatening complications of the condition. These include sepsis, scarring of mucosal surfaces (such as the eyes and genital region), multiple organ failure, and the risk of severe disturbances in body temperature, hydration status, and other bodily functions.
There is little consensus on the most effective treatment for Stevens-Johnson syndrome, but depending on the characteristics of the person, some doctors suggest that the off-label use of oral cyclosporine with or without eternacept is the superior treatment strategy.
Despite the many treatment options, people can die from Stevens-Johnson syndrome. Individuals with more severe forms of Stevens-Johnson syndrome have higher mortality rates. Older adults and people with other underlying medical conditions also have a higher risk of dying.
The goals of the treatment are: prevention of attacks recurrence, normalization of inflammatory markers, control of subclinical inflammation in attacks-free intervals and prevention of medium and long-term complications, as amyloidosis. Colchicine is the first step in the treatment; biological drugs are effective in non-responder patients.
Padcev (enfortumab vedotin-ejfv), an injection drug for intravenous use, was approved by the FDA in December 2019 for the treatment of certain adult patients with locally advanced or metastatic urothelial cancer (mUC). Urothelial carcinoma, also known as transitional cell carcinoma (TCC), is by far the most common type of bladder cancer. As such, if you have bladder cancer it is almost certain to be a urothelial carcinoma. It is important for bladder cancer patients and their families to know that the Padcev skin side effects Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious skin reactions that can be life-threatening and even fatal.
Severe cutaneous adverse reactions, including fatal cases of Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) occurred in patients treated with PADCEV. SJS and TEN occurred predominantly during the first cycle of treatment but may occur later.
The full-text (PDF) version of \"Toxic epidermal necrolysis and Stevens-Johnson syndrome\" is available for free online for those interested in learning more about TEN and SJS, two rare but potentially fatal drug adverse reactions.
Fatal and other serious complications can occur in patients who receive allogeneic hematopoietic stem cell transplantation (HSCT) before or after being treated with a PD-1/L-1 blocking antibody. Transplant-related complications include hyperacute graft-versus-host-disease (GVHD), acute GVHD, chronic GVHD, hepatic veno-occlusive disease (VOD) after reduced intensity conditioning, and steroid-requiring febrile syndrome (without an identified infectious cause). These complications may occur despite intervening therapy between PD-1/L-1 blockade and allogeneic HSCT. Follow patients closely for evidence of transplant-related complications and intervene promptly. Consider the benefit versus risks of treatment with a PD-1/L-1 blocking antibody prior to or after an allogeneic HSCT.
Stevens-Johnson syndrome and toxic epidermal necrolysis are acute, rare, and potentially fatal skin reactions involving loss of skin and, in some cases, mucosal membranes accompanied by systemic symptoms. Medications are causative in over 80 percent of cases. Stevens-Johnson syndrome and toxic epidermal necrolysis are distinguished based on the extent of the detached skin surface area. This activity illustrates the evaluation and treatment of Stevens-Johnson syndrome and of toxic epidermal necrolysis and reviews the role of the interprofessional team in managing those with these conditions.
Objectives: Describe the difference between Stevens-Johnson syndrome and toxic epidermal necrolysis. Summarize differential diagnoses of Stevens-Johnson syndrome and of toxic epidermal necrolysis. Review the treatment options for Stevens-Johnson syndrome and toxic epidermal necrolysis. Explain the role of interprofessional team members in optimizing collaboration and communication to ensure patients with Stevens-Johnson syndrome or toxic epidermal necrolysis receive high-quality care, which will lead to enhanced outcomes.
The management of SJS is interprofessional. A number of specialists are usually involved in the care of these patients, including a dermatologist, intensivist, ophthalmologist, pulmonologist, nephrologist, plastic surgeon, and gastroenterologist, functioning as an interprofessional team. The acute care of these patients is provided by wound care. The pharmacist must also closely assess the medications that the patient is receiving to prevent exacerbation of the disorder or determine if any of the patient's medications could be the trigger for the condition. Even after treatment, these patients may have severe cosmetic deficits and may require mental health counseling. If the lesions occur across joints, the patient may benefit from physical therapy to restore function and muscle strength. The patient has to be educated on the use of ocular lubricants because of the sicca-like syndrome. Many patients do lose weight after suffering a severe reaction and should be referred to a dietitian. Following discharge, the patients need long-term follow-up to ensure that there are no functional deficits, including vision loss. Once a patient has suffered an SJS, it is highly recommended that the patient wear a warning bracelet indicating the toxic agent or allergen. [Level 5]
Prompt and intensive treatment with daily follow-up by an ophthalmologist is essential to reduce the risk of acute and chronic complications, . Topical treatment involves intensive one- to two-hourly preservative-free lubricant eye drops (e.g. hyaluronate 0.2%) throughout the day and night, prophylactic topical antibiotic eye drops (e.g. chloramphenicol 0.5% or a quinolone such as levofloxacin 5mg/mL) four times daily, and steroid eye drops (e.g. dexamethasone 0.1%) every two hours. Topical medication must be preservative-free as preservatives can cause damage to the already fragile ocular surface, . Ocular ointments are generally preservative-free, provide good long-term lubrication and can provide sustained drug delivery , which is useful for reducing the frequency of administration, particularly during sleeping hours.
In cases of corneal ulceration, topical antibiotic treatment should be altered according to clinical findings and microbiological results, which may be intensified up to hourly throughout the day and night. A combination of preservative-free quinolone and second-generation cephalosporin (e.g. cefuroxime 5%) may be required and the frequency of topical steroids may need to be adjusted.
Severe Cutaneous Reactions: Severe cutaneous reactions including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug reaction with eosinophilia and systemic symptoms (DRESS) have been reported. These events can be fatal. Patients with a prior history of Grade 4 rash associated with thalidomide treatment should not receive REVLIMID. Consider REVLIMID interruption or discontinuation for Grade 2-3 skin rash. Permanently discontinue REVLIMID for Grade 4 rash, exfoliative or bullous rash, or for other severe cutaneous reactions such as SJS, TEN, or DRESS. 153554b96e