S3 guideline: Diagnosis and treatment of epidermal necrolysis (Stevens‐Johnson syndrome and toxic epidermal necrolysis) – Part 1: Diagnosis, initial management, and immunomodulating systemic therapy
Ruben Heuer, Maren Paulmann, Thorsten Annecke, Björn Behr, Katharina Boch, Anja M. Boos, Knut Brockow, Lars E. French, Jochen Gille, Verena Gundlach, Bernd Hartmann, P. Höger, Silke Hofmann, Tobias Klein, Marcus Lehnhardt, Yvonne Liß, Philip Maier, Philipp Mandel, Nicos Marathovouniotis, Finnja Marlok, Hans Mittelviefhaus, Uwe Pleyer, Marie Pradeau, Katharina Rall, Siegbert Rieg, Heike L. Rittner, Frank Sander, Stefan Schnitzler, Christina Schut, Annette Stolle, Artem Vorobyev, Bettina Wedi, Johannes M. Weiss, Maximilian Zepp, Mirjana Ziemer, Maja Mockenhaupt, Alexander Nast
Abstract
Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are rare, predominantly drug-induced, acute, life-threatening diseases of skin and mucosae. SJS and TEN are nowadays considered variants of one disease entity with varying degrees of severity called epidermal necrolysis (EN). EN is associated with high morbidity and mortality and constitutes a major disease burden for affected patients. The guideline "Diagnosis and treatment of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis)" was developed under systematic consideration of existing scientific literature and in a formal consensus process according to regulations issued by the Association of Scientific Medical Societies in Germany (AWMF) to establish an evidence-based framework to support clinical decision-making. The interdisciplinary guideline commission consisted of representatives from various specialist societies and patient representatives. The guideline is aimed at specialists in the fields of dermatology, ophthalmology, plastic surgery, intensive care, and pediatrics in hospitals and offices, as well as other medical speciallved in the diagnosis and treatment of EN. The guideline is also aimed at patients, their relatives, insurance funds, and policymakers. This first part focuses on the diagnostic aspects, the initial management as well as the immunomodulating systemic therapy.