ABSTRACT
Toxic epidermal necrolysis and Stevens-Johnson Syndrome describe a spectrum of severe cutaneous skin reactions constituting a medical emergency, and no formal treatment guidelines exist to direct systemic immunosuppressive therapy although referral to a burns unit and wound management remains a mainstay of treatment. We performed a retrospective chart review on all patients at a single centre with TEN between 2017 to 2021 to compare clinical characteristics and outcomes of those treated with the tumour necrosis factor-alpha (TNF-α) inhibitor adalimumab against non-TNF-α immunosuppressants such as glucocorticoids, intravenous immunoglobulin and cyclosporine. All patients treated with adalimumab had successful resolution of their TEN, resulting in a mean duration of hospital admission of 22.5 days compared to 33 days for patients treated with non-TNF-α inhibitors. We highlight adalimumab as a promising systemic immunomodulator in the treatment of TEN with efficacy comparable to other immunosuppressive agents and associated with a shorter duration of hospital admission.
Subject(s)
Burns , Stevens-Johnson Syndrome , Humans , Stevens-Johnson Syndrome/drug therapy , Stevens-Johnson Syndrome/etiology , Adalimumab/therapeutic use , Retrospective Studies , Cyclosporine/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Immunosuppressive Agents/therapeutic use , Immunologic Factors/therapeutic useABSTRACT
Significance: Toxic epidermal necrolysis (TEN) and Steven-Johnson syndrome (SJS) are potentially fatal acute mucocutaneous vesiculobullous disorders. Evidence to date suggests that outcomes for patients with both TEN and SJS are largely dependent on stopping the causative agent, followed by supportive care and appropriate wound management in a specialized burns unit. These are life-threatening conditions characterized by widespread full-thickness cutaneous and mucosal necrosis. This article outlines the approach to holistic management of such patients, in a specialized unit, highlighting various practical aspects of wound care to prevent complications such as infection, mucosal and adhesions, and ocular scaring. Recent Advances: There is improved understanding of pain and morbidity with regard to the type and frequency of dressing changes. More modern dressings, such as nanocrystalline, are currently favored as they may be kept in situ for longer periods. The most recent evidence on systemic agents, such as corticosteroids and cyclosporine, and novel treatments, are also discussed. Critical Issues: Following cessation of the culprit trigger, management in a specialized burns unit is the most important management step. It is now understood that a multidisciplinary team is essential in the care of these patients. Following admission of such patients, dermatology, ear, nose, and throat surgery, ophthalmology, urology, colorectal surgery, and gynecology should all be consulted to prevent disease sequelae. Future Directions: Looking forward, research is aimed at achieving prospective data on the efficacy of systemic immunomodulating agents and dressing types. Tertiary centers with burns units should develop policies for such patients to ensure that the relevant teams are consulted promptly to avoid mucocutaneous complications.
Subject(s)
Holistic Health , Nutritional Support/methods , Palliative Care/methods , Skin Transplantation/methods , Stevens-Johnson Syndrome/therapy , Adrenal Cortex Hormones/pharmacology , Adrenal Cortex Hormones/therapeutic use , Animals , Bandages , Burn Units/organization & administration , Cyclosporine/pharmacology , Cyclosporine/therapeutic use , Hospitalization , Humans , Immunoglobulins, Intravenous/pharmacology , Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/pharmacology , Immunologic Factors/therapeutic use , Length of Stay , Patient Care Team/organization & administration , Skin/drug effects , Skin/immunology , Stevens-Johnson Syndrome/epidemiology , Stevens-Johnson Syndrome/etiology , Swine , Tertiary Care Centers/organization & administration , Transplantation, Heterologous/methods , Treatment Outcome , Wound Healing/drug effects , Wound Healing/immunologyABSTRACT
BACKGROUND/OBJECTIVES: Atopic dermatitis (AD) has significant negative impact on health-related quality of life, mood, sleep, work productivity and everyday activities. Research into the use of new drugs in the management of AD continues to develop, and international updates and recommendations have been published. However, questions remain in the Australian setting. This consensus aims to provide evidence-based insights and practical advice on the management of adult AD in Australia. METHODS: A panel (five dermatologists and one clinical immunologist) met to review the literature, critically examine clinical questions of relevance to Australian healthcare practitioners and develop a series of recommendation statements. A consensus panel, comprising the initial panel plus nine additional members, used a 2-round Delphi voting process to determine a set of final guidance statements. CONSENSUS: ≥75% agreement in the range 7-9. RESULTS: Round 1 voting comprised 66 guidance statements. Of these, consensus was reached on 26, which were retained, and five were removed. The remainder (35) were modified and one new guidance statement was added for inclusion in round 2 voting. After round 2, consensus was reached on 35, which were retained, and one was removed (considered redundant). The 61 guidance statements upon which consensus was reached were then used to support a series of core consensus recommendations and a management flow chart. CONCLUSIONS: Expert consensus recommendations providing practical guidance of clinical relevance to specialists and primary care physicians in Australia have been developed. Dissemination of this guidance and evaluation of its impact on patient outcomes remain to be undertaken.
