J AM ACAD DERMATOL
VOLUME 87, NUMBER 2
based on family history was not considered in our
analysis, and data on disease severity were not
collected. Furthermore, the nature of our tertiary
care center resulted in many patients being lost to
follow-up, limiting our long-term data on
comorbidities.
The established association between atopy, auto-
immune diseases, mental health disorders, and AA
remains consistent in patients with a childhood
diagnosis of AA. Our study brings light to new
considerations by suggesting a protective cardiovas-
cular effect. It reiterates the importance of physician-
recommended screening for atopy, autoimmune
conditions, and mental health disorders when a
diagnosis of AA in childhood is made.
Nora S. Ali, MD,a Megha M. Tollefson, MD,a,b
Christine M. Lohse, MS,c and Rochelle R.
Torgerson, MD, PhDa,d
From the Department of Dermatology,a Depart-
ment of Pediatrics and Adolescent Medicine,b
Department of Quantitative Health Sciences,c
and Department of Obstetrics and Gynecology,
Mayo Clinic, Rochester, Minnesota.d
Funding sources: None.
IRB approval status: This study was approved by the
Mayo Clinic Institutional Review Board, IRB
#15-000989.
Reprints available from Rochelle R. Torgerson, MD,
PhD.
Key words: alopecia areata; atopy; autoimmune
disease; childhood alopecia; epidemiology; pedi-
atric alopecia areata; pediatric hair loss.
Correspondence and reprint requests to: Rochelle R.
Torgerson, MD, PhD, Department of Dermatology,
Mayo Clinic, 200 First Street SW, Rochester, MN
55905
E-mail: torgerson.rochelle@mayo.edu
Conflicts of interest
None disclosed.
REFERENCES
1. Mirzoyev SA, Schrum AG, Davis MD, Torgerson RR. Lifetime
incidence risk of alopecia areata estimated at 2.1% by
Rochester Epidemiology Project, 1990-2009. J Invest Dermatol.
2014;134(4):1141-1142.
2. Safavi KH, Muller SA, Suman VJ, Moshell AN, Melton LJ III.
Incidence of alopecia areata in Olmsted County, Minnesota,
1975 through 1989. Mayo Clin Proc. 1995;70(7):628-633.
3. Huang KP, Joyce CJ, Topaz M, Guo Y, Mostaghimi A. Cardio-
vascular risk in patients with alopecia areata (AA): a
propensity-matched retrospective analysis. J Am Acad Derma-
tol. 2016;75(1):151-154.
Research Letters 429
4. Conic RR, Chu S, Tamashunas NL, Damiani G, Bergfeld W.
Prevalence of cardiac and metabolic diseases among patients
with alopecia areata. J Eur Acad Dermatol Venereol. 2021;35(2):
e128-e129.
5. Kang JH, Lin HC, Kao S, Tsai MC, Chung SD. Alopecia areata
increases the risk of stroke: a 3-year follow-up study. Sci Rep.
2015;5(1):1-6.
https://doi.org/10.1016/j.jaad.2021.08.050
Medical management of Stevens-
Johnson syndrome/toxic epidermal
necrolysis among North American
dermatologists
To the Editor: Stevens-Johnson syndrome (SJS) and
toxic epidermal necrolysis (TEN) are rare
cutaneous reactions associated with significant
morbidity and mortality.1,2 Although recommen-
dations about the supportive management of
SJS/TEN are available,3 no guidelines exist for
their medical management. Furthermore, current
therapies are limited by the lack of standardization
of patients and treatment, causing discrepancies in
their reported efficacy.2,4 Our study aimed to
assess variation in the medical management of
SJS/TEN among dermatology hospitalists in the
United States.
We conducted a cross-sectional survey between
June 10, 2020 and June 30, 2020 among members of
the Society of Dermatology Hospitalists who had
participated in a previous Delphi process on sup-
portive care for patients with SJS/TEN.3 The survey
was distributed using Qualtrics XM (Qualtrics). The
Mass General Brigham institutional review board
approved this study.
The survey was developed to determine varia-
tions in treatment across a series of clinical scenarios
describing various degrees of involved body surface
area, ocular involvement, renal disease (ie, chronic
kidney disease and end-stage renal disease), and
bacteremia (Supplemental 1 available via Mendeley
at https://doi.org/10.17632/zp2bs5hjbh.)
Descriptive statistics were used to summarize
responses. For provided clinical scenarios, the most
preferred treatment for each scenario was organized
into a Sankey plot.
Twenty-four of 32 dermatologists contacted
completed our survey (75% response rate).
Intravenous immunoglobulin and supportive care
alone were the most common treatments (both
n ¼ 20, 83%) over the previous 5 years (Table I).
However, there was no consensus regarding the
drug and the dose and/or duration of the treatments
used (Supplemental Table 2 available via Mendeley
at https://doi.org/10.17632/zp2bs5hjbh.)
430 Research Letters J AM ACAD DERMATOL
AUGUST 2022

Table I. Summary of respondent characteristics Most consulted the urology and, sometimes,
and treatment plans used otolaryngology departments (46% and 54%,
Respondent characteristics n (%)
respectively), and 100% of the participants consulted
the ophthalmology department (88% always and
Respondents, n (response rate) 24 (75%)
12% most of the time) (Supplemental Table 3
Years since completion of residency, 8 (IQR: 6, 10.5)
median (SD) available via Mendeley at https://doi.org/10.17632/
Geographic region of practice n (%) zp2bs5hjbh.)
New England 3 (13%) The results of our study demonstrated substantial
Mid-Atlantic 5 (21%) variation in the management of SJS/TEN among
Midwest 5 (21%) experienced dermatology hospitalists in all regions
Southeast 5 (21%) of the United States. Even among physicians using
Southwest 2 (8%) the same treatment, specific treatment plans varied
West Coast 4 (17%) (ie, route of administration, dosage, and duration of
SJS/TEN patients treated in the past treatment). Treatment choices were also dependent
year on the degree of involved body surface area and
None 2 (8%) comorbid conditions. Furthermore, consultative
1-5 5 (21%)
practices differed, although current guidelines
6-10 8 (33%)
recommend routine consultation with the ophthal-
11-15 7 (29%)
[16 2 (8%)
mology, gynecology, and urology departments
Percentage of inpatient clinical care, 38 (23) [0%-90%] while managing SJS/TEN.3
mean (SD) [range] The heterogeneity of clinical decision making
among a select group of physicians who specialize
Treatments n (%) in the care of this rare condition reflects the
Treatments used within the past limitations of the current literature with inconsistent
5 years for patients with methodologies and small sample sizes, making it
suspected or confirmed SJS/TEN difficult to draw conclusions. The variation in active
Corticosteroids 15 (63%) treatment is also congruent with guidelines from
Cyclophosphamide 0 other countries, which are inconclusive regarding
Cyclosporine 19 (79%) the most effective medical management or even the
Granulocyte colony-stimulating 4 (17%) indication to use medical management over sup-
factor portive care alone. A potential solution is to develop
Hemoperfusion 0 consensus-derived treatment plans that can be
IVIg 20 (83%) broadly implemented to unify patient assessment
Plasmapheresis 1 (4%)
and treatment protocols and can account for patient
Supportive care alone 20 (83%)
Thalidomide 0 variation while ensuring consistency in treatment,
TNF inhibitors 14 (58%) which will enable head-to-head comparisons of
How long after SJS/TEN symptom protocols.
onset do you no longer consider The findings of our study are limited by its study
treatment with active design. We used clinical vignettes to determine
pharmacology? management for certain conditions, which might
0d 1 (4%) not have provided all details that might have been
3d 1 (4%)
needed while making treatment decisions.
5d 1 (4%)
7d 6 (25%)
Always consider active 15 (63%) Jane J. Han, BS,a,b Andrew Creadore, BS,a,c Lucia
pharmacology Seminario-Vidal, MD,d Robert Micheletti, MD,e,f
Megan H. Noe, MD, MPH, MSCE,a and Arash
IVIg, Intravenous immunoglobulin; SJS/TEN, Stevens-Johnson Mostaghimi, MD, MPA, MPHa
syndrome/toxic epidermal necrolysis; TNF, tumor necrosis factor.
From the Department of Dermatology, Brigham
and Women’s Hospital, Boston, Massachusettsa;
Loyola Stritch School of Medicine, Maywood,
Illinoisb; School of Medicine, Boston University,
The participants demonstrated variation in the
Massachusettsc; Department of Dermatology and
medical management of SJS/TEN across all clinical
Cutaneous Surgery, University of South Florida,
vignettes (Fig 1).
Morsani College of Medicine, Tampa, Floridad;
J AM ACAD DERMATOL Research Letters 431
VOLUME 87, NUMBER 2

Fig 1. Most preferred treatments in various clinical scenarios. Participants frequently chose
supportive care alone or cyclosporine with a baseline clinical vignette of 5% body surface area
(BSA) involvement. Participants rarely used tumor necrosis factor inhibitors for patients with
5% BSA involvement. With the addition of ocular symptoms, participants deviated from the use
of supportive care alone and used 5 unique medical therapeutics. When patients presented
with chronic kidney disease, cyclosporine was frequently discontinued, and the patients were
instead treated with supportive care alone, corticosteroids, or tumor necrosis factor inhibitors.
Escalation of chronic kidney disease to end-stage renal disease similarly resulted in a lower
number of participants using cyclosporine. Participants preferred active medical management
over supportive care alone when BSA increased from 5% to 30%. The addition of bacteremia in
a patient with 30% BSA involvement resulted in most participants opting for the use of
intravenous immunoglobulin. BSA, Body surface area; CKD, chronic kidney disease; ESRD,
end-stage renal disease; IVIg, intravenous immunoglobulin; TNF, tumor necrosis factor.

and Department of Dermatology,e and Depart-
ment of Medicine, Perelman School of Medicine,
University of Pennsylvania, Philadelphia,
Pennsylvania.f
Funding sources: None.
IRB approval status: This study (Protocol #
2019P002350) was approved by the Mass Gen-
eral Brigham institutional review board.
Key words: dermatology hospitalists; Stevens-John-
son syndrome; toxic epidermal necrolysis.
Reprints not available from the authors.
Correspondence to: Arash Mostaghimi, MD, MPA,
MPH, Department of Dermatology, Brigham and
Women’s Hospital, 221 Longwood Avenue, Boston,
MA 02115
E-mail: amostaghimi@bwh.harvard.edu
Conflicts of interest
Dr Mostaghimi has received personal fees from Pfizer,
hims, and 3Derm and holds equity in hims and Lucid. Drs
Seminario-Vidal, Micheletti, and Noe and Authors Han and
Creadore have no conflicts of interest to declare.
REFERENCES
1. Micheletti RG, Chiesa-Fuxench Z, Noe MH, et al. Steven-
s-Johnson syndrome/toxic epidermal necrolysis: a multicenter
retrospective study of 377 adult patients from the United
States. J Invest Dermatol. 2018;138(11):2315-2321. https:
//doi.org/10.1016/j.jid.2018.04.027
2. Sekula P, Dunant A, Mockenhaupt M, et al. Comprehensive
survival analysis of a cohort of patients with Stevens-Johnson
syndrome and toxic epidermal necrolysis. J Invest Dermatol.
2013;133(5):1197-1204. https://doi.org/10.1038/jid.2012.510
3. Seminario-Vidal L, Kroshinsky D, Malachowski SJ, et al. Society
of Dermatology Hospitalists supportive care guidelines for the
management of Stevens-Johnson syndrome/toxic epidermal
necrolysis in adults. J Am Acad Dermatol. 2020;82(6):
1553-1567. https://doi.org/10.1016/j.jaad.2020.02.066
4. Zimmermann S, Sekula P, Venhoff M, et al. Systemic Immu-
nomodulating therapies for Stevens-Johnson syndrome and
toxic epidermal necrolysis: a systematic review and meta-
analysis. JAMA Dermatol. 2017;153(6):514-522. https://doi.org/
10.1001/jamadermatol.2016.5668
https://doi.org/10.1016/j.jaad.2021.08.051