Guidelines for Care of Adults With Steven-Johnson Syndrome, Toxic Epidermal Necrolysis

Stevens-Johnson Syndrome
Stevens-Johnson Syndrome
Evidence-based guidelines address supportive care treatment options for adult patients with Stevens-Johnson syndrome and toxic epidermal necrolysis.

Dermatology experts outlined a series of supportive care guidelines for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), published in the Journal of the American Academy of Dermatology.

Members of the Society of Dermatology Hospitalists with experience treating SJS/TEN were invited to participate in a modified online Delphi process. Members were asked to evaluate the appropriateness of 135 statements regarding supportive care of adults with SJS/TEN. Participants rated each statement on a 1-to-9 Likert scale of appropriateness. Statements were also evaluated by level of disagreement among experts, expressed as disagreement index (DI). Any statement with an “appropriateness” score of 6.5 to 9.5 points and a DI ≤1 was considered an approved SJS/TEN guideline.

Guidelines were rated for the following care aspects: hospital setting and care team, wound care, ocular care, oral care, urogenital care, pain management, infection surveillance, fluid and electrolyte management, nutrition and stress ulcer prevention, airway management, and anticoagulation. Regarding care team, participants agreed that a dermatologist must directly participate in SJS/TEN care. Transfer to burn or intensive care units was generally recommended. Wound care should be approached conservatively, authors wrote; high-velocity debridement should be avoided. Panelists instead recommended anti-shear strategies. Experts also recommended early-stage evaluation of the eyes, oral cavity, and urogenital tract, with frequent follow-up evaluations to monitor involvement.

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Care guidelines varied depending on the level of ocular, oral, and urogenital involvement, but in all cases assistance from the relevant specialists was recommended. Pain management should be administered on a case-by-case basis; ketamine, synthetic opiates, gabapentin, and pregabalin were recommended, and nonsteroidal anti-inflammatory drugs were discouraged. Infections are highly prevalent in patients with SJS/TEN; experts recommended consistent skin examinations for signs of infection. Antibiotic therapy for infections should be undertaken with care, authors wrote, and tailored to skin culture results. Investigators also recommended consistent monitoring of electrolyte levels; fluid resuscitation guidelines for SJS/TEN were adapted from those of burn patients. Regarding nutrition, enteral nutrition was preferable for attaining caloric requirements and preventing stress ulcer formation. In the case of airway involvement, intubation and early tracheostomy may be necessary. Given the increased risk for thromboembolism in patients with SJS/TEN, experts also recommended early mobilization, compression stocking, and consistent monitoring for bleeding.

Guidelines were developed from the recommendations of an extensive panel of dermatology experts. Recommendations for special populations—including pregnant patients and children—remain underdeveloped and require future investigation. Revisions to SJS/TEN guidelines will continue in the coming years. Appropriate SJS/TEN care varies widely, authors wrote, “[and the] appropriateness of any specific therapy lies with the treating clinician.”

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Seminario-Vidal L, Kroshinsky D, Malachowski SJ, et al. Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults [published online March 6, 2020]. J Am Acad Dermatol. doi: 10.1016/j.jaad.2020.02.066