ABSTRACT
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, life-threatening diseases that cause sloughing of the skin and mucous membranes. Despite improved survival rates, few studies focus on long-term outcomes. We conducted a single-center review of all patients with SJS/TEN admitted from January 2008 to 2014. SJS/TEN survivors were invited to participate in the validated Veterans RAND 12-Item Health Survey (VR-12) to assess health-related quality of life using a mental health composite score and physical health component score (PCS). The sample was compared to U.S. norms using one-sample two-tailed t tests. A second questionnaire addressed potential long-term medical complications related to SJS/TEN. Of 81 treated subjects, 24 (30%) long-term survivors responded. Participants identified cutaneous sequelae most frequently (79%), followed by nail problems (70%), oral (62%), and ocular (58%) sequalae. Thirty-eight percent rated their quality of life to be "unchanged" to "much better" since their episode of SJS/TEN. The average PCS was lower than U.S. population norms (mean: 36 vs 50, P = .006), indicating persistent physical sequelae from SJS/TEN. These results suggest that SJS/TEN survivors continue to suffer from long-term complications that impair their quality of life and warrant ongoing follow-up by a multidisciplinary care team.
Subject(s)
Burns/psychology , Health Status , Quality of Life/psychology , Severity of Illness Index , Stevens-Johnson Syndrome/psychology , Survivors/psychology , Adult , Attitude to Health , Burns/rehabilitation , Female , Humans , Male , Middle Aged , Physical Examination/methods , Retrospective Studies , Stevens-Johnson Syndrome/rehabilitationABSTRACT
Toxic epidermal necrolysis (TEN) is an unpredictable, life-threatening drug reaction associated with a 30 po cento mortality. Massive keratinocyte apoptosis is the hallmark of TEN. Cytotoxic T lymphocytes appear to be the main effector cells and there is experimental evidence for involvement of both the Fas-Fas ligand and perforin/granzyme pathways. Optimal treatment for these patients remains to be clarified. Discontinuation of the offending drug and prompt referral to a burn unit are generally agreed upon steps. Beyond that, however, considerable controversy exists. Evidence both pro and con exists for the use of IVIG, systemic corticosteroid, and other measures. There is also evidence suggesting that combination therapies may be of value. All the clinical data, however, is anecdotal or based on observational or retrospective studies. Definitive answers are not yet available. Given the rarity of TEN and the large number of patients required for a study to be statistically meaningful, placebo controlled trials are logistically difficult to accomplish. The absence of an animal model further hampers research into this condition. This article reviews recent data concerning clinical presentation, pathogenesis and treatment of TEN. LEARNING OBJECTIVES: At the conclusion of this learning activity, participants should have acquired a more comprehensive knowledge of our current understanding of the classification, clinical presentation, etiology, pathophysiology, prognosis, and treatment of TEN.
Subject(s)
Humans , Stevens-Johnson Syndrome/complications , Stevens-Johnson Syndrome/diagnosis , Stevens-Johnson Syndrome/physiopathology , Stevens-Johnson Syndrome/genetics , Stevens-Johnson Syndrome/rehabilitation , Stevens-Johnson Syndrome/therapyABSTRACT
Stevens-Johnson syndrome and toxic epidermal necrolysis are dermatologic disorders that demonstrate pathophysiologic similarities to partial-thickness burn injury and benefit from treatment in a multidisciplinary burn center. Although burn therapists frequently participate in the care of these patients, guidelines for rehabilitative intervention have not been previously identified. A case report highlights the potential rehabilitative complications and treatment interventions indicated for patients with severe forms of these conditions.
