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Standard reporting and
evaluation guidelines for
Stevens-Johnson syndrome
and toxic epidermal
necrolysis
JAMA Dermatology (Chicago, Ill.)
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.
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.
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.
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.
PAEDIATRIC DERMATOLOGY
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VOL. 1 • NO. 1 • 2017