PracticeUpdate: Dermatology - Vol 1 - No.1 - 2017

PAEDIATRIC DERMATOLOGY 21

Standard reporting and evaluation guidelines for Stevens-Johnson syndrome and toxic epidermal necrolysis Take-home message • The authors of this study developed a standardised format for the reporting of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) to facilitate the collection and comparison of patient information in future studies using the Delphi consensus method. In addition to providing a recommended case report format, the experts advised using the SCORTEN to predict outcomes; consulting urology, Ob/Gyn, ophthalmology, and pulmonology when appropriate; and screening patients of Chinese descent for high-risk human leukocyte antigens prior to starting allopurinol or carbamazepine. • This consensus statement could help standardize care for individuals with SJS/TEN. JAMA Dermatology (Chicago, Ill.)

COMMENT By Jonathan Cotliar MD T his consensus statement from an NIH working group that generated an SJS/ TEN case report form via a Delphi exer- cise, is a major step in the development of a standardized approach to diagnosis of these severe adverse cutaneous events. Discussions among this expert panel used to create this form included an emphasis on the need for all patients with SJS/TEN to have active mucous membrane lesions, such that patients affected could be differ- entiated from those patients with erythema multiforme (EM), who lack mucosal lesions. Historically, inclusion of patients with EM into case reports/series of SJS/TEN has undoubtedly led to confusion among read- ers, and, more importantly, skewed data that have been used to inform clinicians about optimal medical treatment of SJS/TEN. This panel also reinforced several key con- cepts about SJS/TEN: morphology of skin lesions, a positive Nikolsky sign, presence of constitutional symptoms, and calculation of a SCORTEN value to estimate patient mortality were all important components in accurately diagnosing SJS/TEN patients. In addition, transfer to a burn unit or intensive care unit, and consulting urology, gynecol- ogy, ophthalmology, and, in some cases, pulmonology experts, constitute important components of care for SJS/TEN patients. Finally, use of genetic testing for patients of Chinese descent, or those with HLA- B*58:01 and HLA-B*15:02 haplotypes, prior to initiation of allopurinol or carba- mazepine should be considered given the strong association of these genetic poly- morphisms and the incidence of SJS/TEN in individuals exposed to these medications. This new case report form is likely the first step in what will hopefully lead to a pro- spective, interventional therapeutic trial to determine whether agents such as sys- temic steroids, IVIG, cyclosporine, TNF inhibitors, and other immunosuppressive/ immunomodulating drugs have a role in the management of SJS/TEN.

Abstract IMPORTANCE Toxic epidermal necrolysis (TEN) and Stevens-Johnson Syndrome (SJS) are rare, acute, life-threatening dermatologic disorders involving the skin and mucous membranes. Research into these conditions is hampered by a lack of standardization of case reporting and data collection. OBJECTIVE To establish a standardized case report form to facilitate comparisons and main- tain data quality based on an international panel of SJS/TEN experts who performed a Delphi consensus-building exercise. EVIDENCE REVIEW The elements presented for committee scrutiny were adapted from pre- vious case report forms and from PubMed literature searches of highly cited manuscripts pertaining to SJS/TEN. The expert opinions and experience of the members of the con- sensus group were included in the discussion. FINDINGS Overall, 21 out of 29 experts who were invited to participate in the online Delphi exercise agreed to participate. Surveys at each

stage were administered via an online survery software tool. For the first 2 Delphi rounds, results were analyzed using the Interpercen- tile Range Adjusted for Symmetry method and statements that passed consensus formulated a new case report form. For the third Delphi round, the case report form was presented to the committee, who agreed that it was “appro- priate and useful” for documenting cases of SJS/TEN, making it more reliable and valuable for future research endeavors. CONCLUSIONS AND RELEVANCE With the consen- sus of international experts, a case report form for SJS/TEN has been created to help stand- ardize the collection of patient information in future studies and the documentation of indi- vidual cases. Stevens-Johnson syndrome and toxic epi- dermal necrolysis standard reporting and evaluation guidelines: results of a National Institutes of Health Working Group. JAMA Dermatol 2017 Mar 15;[EPub Ahead of Print], E Maverakis, EA Wang, K Shinkai, et al.

Dr Cotliar is a dual-trained and board-certified internist and dermatologist with a special interest in complex medical dermatology. He previously served as a full-time faculty physician at the David Geffen School of Medicine at UCLA,

and at Northwestern University Feinberg School of Medicine before joining City of Hope National Medical Center to lead a new dermatology program there.

VOL. 1 • NO. 1 • 2017