LUPUS
Ultraviolet B generates Type 1 Interferon and induces autoantibody-mediated disease
in a mouse model of cutaneous lupus.
Sontheimer , Clayton, Elkon , Keith.
Background/Purpose: Photosensitivity is a common symptom in patients with systemic
lupus erythematosus (SLE) and lupus skin lesions often contain plasmacytoid dendritic cells
(pDC). The mechanisms linking ultraviolet (UV) light to inflammation and cutaneous flares is
not well understood. While in vitro experiments have suggested that UV-induced apoptosis
exposes lupus-specific nuclear antigens and immune complex mediated inflammation, this
has not been shown in vivo. Here, we asked whether, and under what conditions, UVBinduced inflammation could induce Type I interferon (IFN-I) and the roles of pDCs and also
autoantibodies in cutaneous lupus.
Methods: Shaved C57BL/6 (B6), IFNAR KO, BDCA2 DTR, and huFcgR2a transgenic mice
were irradiated with narrowband UVB at 100 mJ/cm2/day for 5 consecutive days. To induce
interface dermatitis, shaved and depilated mice were subject to 15 strokes of tape stripping
using medical tape (3M). Serial punch biopsies (6 mm) were obtained at 3, 24, and 72 hrs
following UVB exposure or tape stripping. PDCs were detected in enzyme digested skin
samples by flow cytometry (CD45+, Ly6C+, PDCA1+, CD11c+, Siglec H+). Skin samples were
examined for mRNA expression by QPCR of pro-inflammatory cytokines and Interferon
Stimulated Genes (ISG). mRNA fold change was calculated by comparison with nonirradiated control mice. In experiments with huFcgR2a Tg mice, mice were irradiated as
above but injected i.p. at the time of the final UVB exposure with purified immunoglobulin
pooled from human lupus patients and the skin was examined by immunofluorescence for
the presence of human IgG.
Results: Whereas tape stripping induced a robust ISG response associated with the presence
of pDC in the skin, repeated UVB exposure induced a more modest IFN-I skin response with
bimodal peaks at 3 and 72 hrs when compared to control mice (p<0.05, n=20). UVBirradiated IFNAR KO mice had increased levels of pro-inflammatory cytokines TNFα and IL-6
at (p<0.01 at 3 and 24 hr time points, n=12-13) and had increased levels of inflammation by
visual scoring suggesting a protective role for IFN-I. Interestingly, pDCs did not appear to be
the source of IFN following UVB as pDC-depleted BDCA2 DTR mice maintained moderate
expression of ISGs. Immunoglobulin from human lupus patients, but not IVIG, localized to the
skin at the dermal/epidermal junction following UVB of FcgR2A transgenic and wild-type
mice, but FcγR2a signaling was required for cellular uptake and enhanced Type 1 IFN
signaling (p<0.05, n=5-9).
Conclusion: In the normal host, repeated doses of UVB induce a protective, pDCindependent Type1 IFN response in the skin that attenuates pro-inflammatory signals and
limits tissue damage. In contrast, in situations associated with the presence of autoantibodies
as occurs in lupus, the antibodies bind to UVB-exposed antigen, deposit in the skin and
require Fcgr2a-mediated uptake to produce enhanced expression of IFN-I. This novel FcgR2a
mouse model of cutaneous lupus establishes the role of UVB in exposing otherwise
sequestered nuclear antigens and in facilitating immune-complex mediated skin disease in
lupus.
Unmet need for mental health care in skin-predominant lupus erythematosus and
dermatomyositis
Jordan C. Achtman and Victoria P. Werth
The Perelman School of Medicine at the University of Pennsylvania
Background: Patients with CLE and DM suffer from decreased quality of life compared to
the healthy population and patients with other chronic conditions. While psychiatric
comorbidities such as depression and anxiety have been thoroughly documented in other
chronic dermatological and rheumatological conditions, these mental health issues remain
less well described in skin-predominant lupus erythematosus and dermatomyositis. To date,
the literature does suggest increased rates of mood and anxiety disorders in these diseases in
the context of decreased quality of life; however, little is known about access and barriers to
and utilization of mental health care in this population. The aim of this study is to
characterize these aspects of mental health care in skin-predominant LE and DM and to
determine the unmet need for mental health care.
Methods: Patients recruited for this study were administered three questionnaires: PHQ-9,
GAD-7, and MHCAQ. The PHQ-9 and GAD-7 are questionnaires validated for the screening,
diagnosis, and severity determination of depression and anxiety, respectively. Based on
validation studies, a PHQ-9 score ≥10 and a GAD-7 score ≥8 were used to determine the need
for further evaluation and/or treatment. The MHCAQ is a novel questionnaire developed for
this study that determines use of and barriers to mental health care. Rates for depression or
anxiety with need for mental health care were calculated by the addition of all subjects who
met PHQ-9/GAD-7 thresholds and all subjects below threshold who were being treated for
depression or anxiety. A total of 44 skin-predominant LE and 31 dermatomyositis patients
were enrolled.
Results: For skin-predominant LE, 14 out of 44 (31.8%) subjects met criteria for depression,
with a need for mental health care. 5 out of the 14 (35.7%) subjects were receiving no
treatment giving a rate of 11.4% (5/44) untreated depression for all subjects. 14 out of 44
(31.8%) subjects met criteria for anxiety with need. 6 out of the 14 (42.9%) subjects were
receiving no treatment giving a rate of 13.6% (6/44) untreated anxiety for all subjects.
Overall, 7 out of 44 (15.9) subjects met criteria for depression and/or anxiety with need but
without treatment. For dermatomyositis, 8 out of 31 (25.8%) subjects met criteria for
depression with need. 5 out the 8 (62.5%) subjects were receiving no treatment giving a rate
of 16.1% (5/31) untreated depression for all subjects. 12 out of 31 (38.7%) subjects met
criteria for anxiety with need. 4 out of the 12 (33.3%) subjects were receiving no treatment
giving a rate of 12.9% (4/31) untreated anxiety for all subjects. Overall, 5 out of 31 (16.1%)
subjects met criteria for depression and/or anxiety with need but without treatment.
Conclusion: Given the significant unmet need for mental health care in these populations
and the lack of guidelines for dermatologists treating these autoimmune skin conditions,
these findings suggest a need for improvement in awareness, screening, and referral of
patients to appropriate psychiatric support services.
Quality of life in discoid lupus patients
Noelle M. Teske, BA, MSc1, Zachary Cardon, BS1, Xilong Li, PhD, MBA2, Benjamin F. Chong, MD,
MSCS1
Department of Dermatology1, Department of Clinical Sciences2
University of Texas Southwestern Medical Center, Dallas, TX
Background: Previous studies have demonstrated impairment of quality of life (QoL) in
cutaneous lupus (CLE) patients. However, no studies have examined QoL in discoid lupus
(DLE), whose unique clinical features may impact QoL distinctly.
Objective: To identify characteristics that correlate with worse QoL in DLE patients.
Methods: A cross-sectional study of DLE patients seen at University of Texas Southwestern
Medical Center and Parkland Memorial Hospital between April 2010 and April 2014 was
performed. 117 DLE patients completed demographic, medical history, and QoL
questionnaires, including SKINDEX-29, and underwent clinical assessments (e.g. Cutaneous
Lupus Erythematosus Disease Severity and Area Index (CLASI)). Univariate analyses were
performed to assess relationships between predictor variables and primary outcome
measures of SKINDEX-29 scores in emotions, functioning, and symptoms subdomains.
Predictor variables with p<0.25 in the univariate analyses were included in multivariate
logistic regression models.
Results: DLE patients had significantly higher and worse SKINDEX-29 symptoms scores
(40.58±21.19) than other CLE patients (33.21±22.13, n=47) (p=.0451). Univariate analyses
showed that higher SKINDEX-29 emotions, functioning, and symptoms scores correlated
with higher CLASI activity (p=0.0456 (emotions), p=0.0015 (functioning), p<0.0001
(symptoms)) and damage (p=0.0378 (emotions), p=0.0319 (functioning), p=0.0163
(symptoms)) scores, current smoking status (p=0.0002 (emotions), p=0.0011 (functioning),
p=0.0129 (symptoms)), and income less than $10K/year (p=0.013 (emotions), p<0.0001
(functioning), and p=0.003). Multivariate analyses highlighted female gender as a significant
factor associated with higher SKINDEX-29 emotions (p=0.0189) and symptoms scores
(p=0.0077), and correlated current smoking status with poorer QoL in the SKINDEX-29
emotion (p=0.0013) and functioning subdomains (p=0.0173).
Conclusions: DLE patients have greater symptom-related QoL impairment than other
subtypes of CLE. Subsets of DLE patients, such as females and current smokers, may be
particularly vulnerable to poorer QoL, and providers may consider these demographic
factors in their overall management of these patients.
Malar and discoid rash at diagnosis of SLE does not impact SLICC damage scores at 5and 10-year follow-up
Aaron M Drucker MD1, Farheen Mussani MD1, Jiandong Su BSc2, Dominique Ibanez MSc2,
Sanjay K Siddha1 MD, Dafna D Gladman MD2 and Murray B Urowitz MD2
From the Division of Dermatology (1) and Centre for Prognostic Studies in the Rheumatic
Diseases (2), University Health Network, Toronto, Ontario, Canada
Objective: Previous studies have suggested that systemic lupus erythematosus (SLE)
patients with cutaneous lupus erythematosus (CLE), in particular discoid lupus
erythematosus (DLE), may have less severe systemic disease than SLE patients without CLE.
We aimed to compare SLE patients without skin manifestations to SLE patients with DLE or
malar rash at diagnosis with regards to eventual organ damage.
Methods: The Toronto Lupus Clinic database has prospectively collected data on patients
with SLE from 1970 to the present. Data collected includes clinical phenotype and organ
damage as measured by the System Lupus International Collaborating Clinics/ACR Damage
Index (SLICC). The SLICC evaluates irreversible damage in 12 organs, including the skin, with
a maximum score of 46. We limited this study to an “inception cohort” of patients first
assessed in the Lupus Clinic within one year of diagnosis of SLE. We compared SLICC scores
and SLICC scores minus skin damage at 5 and 10 years post-inception between SLE patients
who never developed CLE and patients who had DLE or the malar rash at their inception
visit. Statistical significance was defined as a p-value ≤0.05 calculated using Wilcoxon or ChiSquare tests.
Results: In a cohort of 764 SLE inception patients, 284 (37.1%) never developed CLE, 65
(8.5%) had DLE at inception and 175 (22.9%) had a malar rash at inception. At 5 years of
follow-up, mean total SLICC scores were similar in the group that never developed CLE (0.75)
compared to the DLE (0.73, p=0.57) and malar rash (0.67, p=0.84) groups. Additionally, there
was no statistically significant difference between the percentage of patients with SLICC
scores above 0 in the group without skin manifestations (35.9%), the DLE group (41.5%,
p=0.4) and the malar rash group (36.0%, p=0.99). At 10 years, results were similar with no
statistically significant differences seen between the groups with regards to mean total SLICC
scores and percentage of patients with scores above 0. Additionally, there was no statistically
significant difference in mean SLICC scores and the percentage of patients with scores above
0 between the groups at 5 and 10 years of follow-up when skin damage was subtracted from
the total score.
Conclusions: Malar and discoid rashes around the time of diagnosis of SLE do not have
appear to have a significant prognostic impact on overall SLE damage.
Supported by a grant from the Canadian Dermatology Foundation
Sunscreen use in cutaneous lupus erythematosus patients
Elizabeth N. Le, MD*, Danielle Q. Lin, BA*, Ira Bernstein, PhD**, Steven Q. Wang, MD***, and
Benjamin F. Chong, MD, MSCS*
*University of Texas Southwestern Medical Center, Department of Dermatology, Dallas, TX
**University of Texas Southwestern Medical Center, Department of Clinical Sciences, Dallas,
TX
***Memorial Sloan Kettering Cancer Center, Division of Dermatology, New York, NY
Background: Sunscreen use among cutaneous lupus erythematosus (CLE) patients has been
suboptimal. Characteristics of non-sunscreen CLE users and reasons for CLE patients not
wearing sunscreens are unknown.
Objective: To determine the frequency and patterns of sunscreen use and identify barriers to
sunscreen use in patients with CLE.
Methods: A cross-sectional survey on sunscreen use was administered to patients enrolled
in the University of Texas Southwestern CLE Registry. Demographics and patient history
were obtained from the registry database and medical charts. Additional information
including disease severity and quality of life scores were collected from patients seen in
person.
Results: 100 CLE patients completed the survey. 32 patients reported daily sunscreen use,
and 40 patients noted that they did not use sunscreen. 50% and 63% of all CLE patients reapply sunscreen daily or while outside for a prolonged time, respectively. Univariate analyses
revealed that non-married status (p=0.007) and low income (p=0.01) were associated with
non-sunscreen use. Multivariate analyses identified non-married status (p=0.04) as
significant factors seen in non-sunscreen users. When comparing daily and non-sunscreen
users, significant barriers to regular sunscreen use included forgetfulness (p=0.0002),
inconvenience (p=0.006) and perception that sunscreen did not prevent lupus flares
(p=0.03).
Conclusions: A large proportion of CLE patients do not wear sunscreen. Non-sunscreen
users, who tend to be not married, would benefit from education on the virtues of sunscreen
application. Providers are encouraged to counsel CLE patients on appropriate timing and
frequency of sunscreen use throughout the day and protection from future lupus flares.
Furthermore, educational programs geared towards improving sunscreen use in this
population will be developed and implemented in future pilot studies.
The incidence of zoster in patients with cutaneous lupus erythematosus and
dermatomyositis is increased compared to the average U.S. population
ES Robinson,1,2 J Okawa,1,2 R Feng,3 AS Payne,2 VP Werth1,2
Veteran Affairs Medical Center, Philadelphia, PA
Department of Dermatology, University of Pennsylvania, Philadelphia, PA
3 Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, PA
1
2
Background: Herpes zoster is a common condition that causes significant pain and, often,
post-herpetic neuralgia. In the United States the incidence of zoster per 1,000 person-years is
6 for people 60 years old and increases with age to nearly 11 for people above 80 years old.
The incidence of zoster may be increased in autoimmune diseases, but few studies have
looked specifically at cutaneous autoimmune diseases. Prior studies have found that the
incidence of zoster in systemic lupus erythematosus is up to 32.5 per 1,000 person-years
(n=303).
Methods: This retrospective chart review examined the incidence of zoster in patients with
cutaneous lupus erythematosus (CLE) (n=105), dermatomyositis (DM) (n=66) and
pemphigus vulgaris (PV) (n=55) seen in the practices of two dermatologists between April 1,
2013 and September 31, 2013. An incidence of zoster was determined according to a
matched text search for “zoster” or “shingles” in all available electronic medical records. The
date of the zoster episode, if known, and the medications that the patient was taking at the
time of the episode were recorded. The date of each patient's earliest visit with his
dermatologist recorded in the electronic medical record until the most recent visit through
September 31, 2013 or an episode of zoster, whichever was earlier, was used to estimate the
time that the person had been at risk. The total number of incidences of zoster divided by the
total number of person-years at risk was used to determine the incidence rate. Patients with
a known history of zoster prior to the start of their electronic medical records and patients
whose date of zoster was unknown were excluded from the incidence rate calculations.
Results: The incidence rate of zoster per 1,000 person-years was 23 for CLE, 54 for DM, and
7 for PV. The incidence rates were based on 6 episodes of zoster per 257.9 person-years for
CLE, 8 episodes of zoster per 149.5 person-years for DM and 1 episode of zoster per 145.4
person-years for PV. Six CLE, 7 DM and 3 PV subjects had an unknown date of zoster. One
CLE, 5 DM and 2 PV patients had zoster prior to the start of their electronic medical records.
The mean (SD) duration of follow-up was 2.7 (1.7) years for CLE, 2.8 (1.7) years for DM and
3.0 (1.8) years for PV. The mean age (standard deviation) of each group was: 46.2 (14.1) for
CLE, 55.9 (14.2) for DM and 56.1 (13.8) for PV. Fourteen of the 16 patients who had zoster
were on immunosuppressive medications at the time of the zoster episode. Some patients
were on more than one immunosuppressive therapy at the time of their zoster episode. The
immunosuppressive medications were: mycophenolate mofetil (n=7), prednisone (n=6),
methotrexate (n=1) and azathioprine (n=1). The majority of patients in each disease group
were Caucasian women.
Conclusions: The incidence rate of zoster in CLE and DM is higher than in the average U.S.
population at or above 80 years old. The incidence of zoster in PV is close to that of the
average U.S. population of a similar age. The use of immunosuppressive therapies may play a
role in the increased incidence of zoster in CLE and DM.
Treatment of Scarring Alopecia in Chronic Cutaneous Lupus Erythematosus with
Tacrolimus 0.3% Solution
Emily Milam1, BA; Sarika Ramachandran1, MD; Jerry Shapiro1, MD, Andrew G. Franks, Jr.1, MD
1The
Ronald O. Perelman Department of Dermatology, New York University School of
Medicine, New York, NY
Abstract: Topical and intralesional steroids and antimalarials have been the mainstay of
treatment for chronic cutaneous lupus, e.g. discoid lupus erythematosus (DLE). For
recalcitrant cases, systemic immunosuppressants are sometimes used. Calcineurin inhibitors
have been used to treat psoriasis and eczema and have also shown some success in the
treatment of DLE lesions, namely in the form of tacrolimus ointment.1,2 We used a custom
formulated tacrolimus 0.3% (not 0.03%) in an alcohol base as adjunct therapy in three
patients with recalcitrant alopecia secondary to discoid lupus. All three patients had
significant improvement in disease activity consistent with CLASI criteria, including a
reduction in erythema, scale, and surface area of the lesions, and increased hair growth after
using the solution for many months. The results of these cases support the use of topical
tacrolimus 0.3% in an alcohol solution as an adjunct therapeutic option in resistant cases of
DLE-associated alopecia.
References
1. Sugano M. Shintani Y. Kobayashi K. et al. Successful treatment with topical tacrolimus
in four cases of discoid lupus erythematosus. J Dermatol. 2006 Dec; 33(12): 887-91.
2. Heffernan MP. Nelson MM. Smith DI et al. 0.1% Tacrolimus Ointment in the Treatment
of Discoid Lupus Erythematosus. Arch Dermatol. 2005;141(9):1170-1171.
3. Lampropoulos CE, Sangle S, Harrison P. et al. Topical tacrolimus therapy of resistant
cutaneous lesions in lupus erythematosus: a possible alternative.Rheumatology.2004
Nov;43(11):1383-5.
DERMATOMYOSITIS
Adequacy of Skin Variables in the New Classification Criteria for Adult and Juvenile
IdiopathicInflammatory Myopathies for the Diagnosis of Amyopathic Dermatomyositis
Neelam Khan, MS1, 2, Victoria P. Werth, MD1
1 Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania,
Philadelphia, PA, 2 Georgetown University School of Medicine, Washington, DC
Background: There have been numerous classification systems set forth for the diagnosis of
adult dermatomyositis (DM) that have varied in their inclusion of clinical criteria, diagnostic
technologies, and DM subtypes. As an international, multidisciplinary collaboration, the
International Myositis Classification Criteria Project (IMCCP) has sought to develop and
validate new classification criteria for adult and juvenile idiopathic inflammatory
myopathies (IIM). The purpose of this study is to evaluate the adequacy of the skin variables
included within the IMCCP classification criteria for the diagnosis of amyopathic
dermatomyositis (ADM). Those with ADM have a higher likelihood of misdiagnosis and are
particularly at risk for rapidly progressive interstitial lung disease. It is essential that any
new classification scheme for DM include criteria to appropriately classify those with ADM
as well as those with other subtypes of DM.
Methods: This retrospective study was conducted at the University of Pennsylvania Health
System. Patients included were 18 years or older with a clinical and/or histological
diagnosis of dermatomyositis and enrolled in a prospective database study for
dermatomyositis between July 2008-­­2014. A total of 135 patients were evaluated for their
skin findings at time of enrollment using the Cutaneous Dermatomyositis Disease Area and
Severity Index (CDASI), a validated outcome measure quantifying disease activity in fifteen
anatomic locations.
Results: The 135 patients evaluated included 51.85% with classic DM (CDM) and 48.15%
with clinically amyopathic DM (CADM). Of those with CADM, 41.48% had ADM and 6.67%
had hypomyopathic DM. There was no significant difference in the cutaneous presentation
of CDM vs. CADM. In patients with ADM, 91.07% had at least one of the three skin variables
included in the IMCCP criteria (heliotrope rash, Gottron’s sign, Gottron’s papules), with
26.79% presenting only with one skin variable, 41.07% with two skin variables, and 23.21%
with all three. Five patients with ADM presented with none of the three skin variables at
time of enrollment.
Conclusions: Most patients with ADM had at least one of the three skin variables included in
the new IMCCP classification criteria for diagnosis of adult dermatomyositis. Because the
IMCCP criteria uses a minimum probability cutoff and assigns a distinct score for each skin
variable, a patient with ADM must have at least two out of the three skin criteria for
diagnosis. Of the
ADM patients analyzed in this study, approximately ¼ would not meet such criteria. As
these patients with ADM all presented with cutaneous manifestations of disease, it is
important to consider the possibility of additional skin variables within the new
classification criteria or in a separate criteria for clinically amyopathic DM to allow for the
inclusion of such patients. Furthermore, as five patients with ADM did not present with any
of the three skin variables at enrollment, it is important for practitioners to consider the full
history of a patient’s cutaneous manifestations of DM and the impact medication use may
have on skin findings.
Disease Progression and Flare in Cutaneous Dermatomyositis: A Longitudinal Study
Using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI)
Jeannette M. Olazagasti, Rui Feng, Victoria P. Werth
Background/Purpose: To characterize disease course in cutaneous dermatomyositis (DM)
by conducting a longitudinal analysis using the Cutaneous Dermatomyositis Disease Area and
Severity Index (CDASI) activity score.
Methods: Patients 18 years or older with clinical or histologic evidence of DM who had the
CDASI activity scores recorded for at least 2 years from time of initial visit were included. For
each patient, time from initial visit (in years) was plotted on the x-axis and CDASI activity
score for each visit was plotted on the y-axis. The plots were then used to determine 3
features of disease course over time: average disease activity, overall progression, and
variability. The average disease activity over time was estimated by calculating the Area
under the Curve per number of years of follow-up. The overall progression of disease activity
over time was assessed by conducting a linear regression analysis and calculating the slope
to determine average change in CDASI per year. Disease progression in each patient was
classified as “improved,” “worsened,” or “stable” over time by the slopes of m < -4, m > +4,
and -4 < m < +4, respectively. The variability in disease activity over time was evaluated by
calculating the number of flares; with “flare” defined as a 4-point increase in the CDASI,
divided by the number of years of follow-up. Study patients were divided into two groups
based on disease severity at baseline (mild versus moderate-severe disease activity). The
above-mentioned parameters were analyzed in both groups and statistical significance was
evaluated using Mann-Whitney tests. Graphpad 5.0 software was used for all descriptive
statistics, AUC calculations, slope estimates, and group comparisons.
Results: A total of 40 DM patients fulfilled inclusion criteria. The majority of the patients
were female (90%) and Caucasian (95%), with a mean age of 52.9 years at the time of initial
visit. Disease subtype was classified as classic in 52.5% of patients and skin predominant in
47.5%. The mean follow-up time was 3.50 years. Patients with moderate-severe disease
activity at baseline made up a majority of the patients (N=23, 58%). The average disease
activity over time, calculated by AUC/yr, was statistically significantly different for mild and
moderate-severe disease patients (9.39 versus 15.08; P<0.002). Patients with mild disease
activity at baseline had stable disease activity over time (m = +0.36) while those with
moderate-severe disease activity tended to improve (m = -3.36; P<0.004).Variability in
disease activity over time was similar for the mild and moderate-severe patients (0.32 versus
0.33 flares/yr; P=0.86).
Conclusion: Cutaneous DM disease course over time can be characterized by 3 features:
average disease activity, overall progression, and variability. The definition of cutaneous DM
“flare” can be used to describe variability in disease activity. This longitudinal study provides
a framework for using CDASI to monitor disease course over time.
Anti-MDA5 Dermatomyositis: A Longitudinal Analysis
Matt Lewis, Shufeng Li, Lorinda Chung, David Fiorentino
Stanford University, Department of Dermatology
Background: Dermatomyositis patients with anti-melanoma differentiation-associated gene
5 (MDA5) antibodies are at increased risk of developing interstitial lung disease (ILD). The
natural history of ILD among this subset of dermatomyositis patients is poorly understood.
Objective: We sought to characterize the temporal trends in ILD severity, cutaneous
ulceration and calcinosis in MDA5 dermatomyositis patients.
Methods: We retrospectively reviewed the cohort of 23 MDA5 dermatomyositis patients
seen at Stanford University Dermatology in California between July 2004 and July 2014.
Results: Of the 23 MDA5 dermatomyositis patients seen, 14 patients (60%) had at least 2,
serial pulmonary function tests available for analysis. Among the 14 patients followed, we
found a statistically significant increase in diffusion capacity (DLCO) over time using
repeated measures model. The regression trend line estimated an average increase of 0.36%
in DLCO per month, (95% CI .05-0.66%, p=.02). Of the 12 MDA5 dermatomyositis patients
with abnormal lung function tests, 8 of those patients (67%) showed a greater than 15%
absolute increase in DLCO (average=24%). Among the 6 patients in whom both serial
pulmonary function tests and cutaneous dermatomyositis disease area and severity index
(CDASI) scores were available, 5 of the patients (83%) had nadirs in DLCO at their timematched peak CDASI scores, although this relationship did not reach statistical significance.
The median times to onset of cutaneous ulceration, ulcer healing and calcinosis were 6, 22
and 28 months, respectively. 4 patients (17%) died during follow-up: 1 patient died from
rapidly progressive ILD at 2 weeks, 1 patient died following a bilateral lung transplant, and 2
patients died at 7 and 18 months from progressive ILD.
Limitations: The trends and associations identified are limited by the small sample size,
location at a tertiary referral center, varied treatments and retrospective nature of the
investigation. We also may not capture dermatomyositis patients with rapidly progressive
ILD who present in critical condition.
Conclusion: There is a large subset of MDA5 dermatomyositis patients who have interstitial
lung disease that improves over time with treatment. Among MDA5 dermatomyositis
patients with ILD, their skin disease activity may be an indicator of their ILD severity. The
onset of calcinosis in MDA5 dermatomyositis patients temporally occurs around healing of
cutaneous ulcerations.
Histopathologic findings in dermatomyositis of the scalp
Santos, Leopoldo; Martinka, Magdalena; Shapiro, Jerry; Dutz, Jan
University of British Columbia, 835 West 10th avenue, Vancouver, BC, V5Z4E8, Canada
Background: Cutaneous features of dermatomyositis (DM) often begin and continue to
involve the scalp. While there are clinical descriptions of scalp dermatomyositis, there are no
published descriptions of the histopathology of this disorder affecting the scalp.
Objective: To describe the histopathologic features of dermatomyositis of the scalp.
Methodology: Scalp biopsies of two patients with typical cutaneous features of
dermatomyositis were examined and compared to scalp biopsies of patients with cutaneous
forms of lupus erythematosus (LE). Horizontal and transverse sections stained with
hematoxylin and eosin and transverse sections stained with PAS and mucin stains were
examined.
Results: DM histopathology showed the following features within the epidermis; follicular
plugging and mild vacuolar interface changes. Within the dermis, superficial and deep
perivascular and peri-adnexial lymphocytic infiltrates were noted that extended to the
subcutis in one case. Both cases showed basement membrane thickening on PAS stain and
increased mucin. The control case (LE) showed within epidermis; mild atrophy, subtle
vacuolar interface change and follicular plugging. Within the dermis, dense superficial and
deep perivascular, perifollicular and perieccrine lymphocytic infiltrates which extended to
the superficial subcutis. However, basement membrane thickening and increased mucin were
absent features.
Conclusion: The dermatopathological features of scalp dermatomyositis include superficial
and deep perivascular lymphocytic infiltrates, the presence of lichenoid interface changes,
colloid bodies, a thickened basement membrane and mucin deposition. Clinicians and
dermatopathologists should be aware that scalp dermatomyositis may mimic cutaneous
lupus erythematosus of the scalp and that a pathological distinction between these two
entities affecting the scalp may not be possible.
Double Trouble: Psoriasis-like Eruptions in Dermatomyositis Patients
Allison Truong BS, David Fiorentino MD, PhD
Department of Dermatology, Stanford University School of Medicine, Redwood City, CA
Background: Dermatomyositis (DM) and psoriasis are inflammatory skin disorders that
have heterogeneous clinical presentations, co-morbidities, and disease courses. Skin lesions
in DM patients manifest as atrophic scaly violaceous eruption on the eyelids, knuckles,
shoulders, and hips. Classic psoriatic lesions demonstrate well-demarcated, erythematous
plaques with underlying silvery, micaceous scales. Histopathologically, DM demonstrates
interface dermatitis with superficial perivascular infiltrate and dermal mucin deposition
whereas psoriasis displays epidermal acanthosis, hypogranulosis, confluent parakeratosis,
increased vascularity, subcorneal neutrophils, and thinning of the suprapapillary plate.
However, when DM patients present with erythematous scaly plaques in the scalp and
extremities, this poses a diagnostic dilemma for clinicians.
Objective: We aimed to describe three cases of psoriasis-like eruptions in DM patients and
discuss clinical and histopathological findings to identify whether these patients have two
distinct clinical phenomenon (DM and psoriasis) or rather atypical DM with psoriasis-like
eruption.
Methods: Our first case is a 32-year old female who presented with violaceous rash on the
eyelids, discolored fingernail beds, and scalp erythema. Our second case is a 38-year-old
female who presented with well-demarcated violaceous erythematous, scaly plaques on her
elbows, extensor forearms, dorsal hands, lateral thighs, knees, and lower legs along with
diffuse scalp and periungual erythema. Our third case is a 67-year-old female who presented
with well-demarcated erythematous scaly slightly atrophic plaques with surrounding illdefined erythema on scalp, face, trunk, and bilateral upper and lower extremities along with
scalp alopecia.
Results: All three patients initially presented with skin manifestations prior to meeting
clinical criteria for DM. Skin biopsies were performed on psoriatic-appearing plaques at time
of diagnosis or during acute flares. Our first patient’s skin biopsy revealed epidermal
acanthosis with mounded parakeratosis favoring psoriasis. Patient’s skin lesions remain
stable on Stelara, prednisone taper, IVIG, and MTX. Our second patient had epidermal
acanthosis, parakeratosis, mild superficial perivascular infiltrate, and dilated papillary
dermal vessels, without interface dermatitis consistent with psoriasis. However, a colloidal
iron stain revealed abundant mucin throughout the dermis, which is unusual in psoriasis and
more consistent with DM. Her skin lesions progressed to neck and chest on prednisone taper
and azathioprine. Our third patient’s biopsy revealed vacuolar interface dermatitis and
vascular ectasia without eosinophils, favoring DM. Her skin disease remains stable with MTX
and topical steroids.
Conclusion: Although rare, patients with DM and psoriasis-like skin eruptions represent
therapeutic challenges to clinicians. Histopathologically, our first patient appeared to have
both DM and psoriasis whereas our third patient appeared to have atypical DM with
psoriasis-like eruption. Our second patient initially appeared to have psoriasis but additional
stains clenched the diagnosis of DM. It remains unclear if there are patients with both distinct
clinical entities DM and psoriasis, or if DM patients develop psoriasis-like eruption.
Interestingly, there are common pathways to inducing both inflammatory skin disorders as
they share similar interferon-induced responses and dysregulation of cytokines. Further
research into ways to distinguish these two clinical entities where histopathologic criteria
may be inconclusive would facilitate therapeutic challenges seen in caring for these patients
with two concurrent autoimmune or inflammatory skin diseases. Currently, therapies for one
may exacerbate symptoms of the other requiring multi-tier therapeutic approach to avoid
flaring of coexisting conditions. In cases where diagnosis is unclear, we recommend
requesting additional stains to evaluate for atypical DM.
Venous Thromoboembolism and Cutaneous Autoimmune Disease:
Case Report of Amyopathic Dermatomyositis related Thrombophilia and Review of
Literature
Mark G Kirchhof1,2 and Jan P Dutz1,3
1Department of Dermatology and Skin Science, University of British Columbia, Vancouver, BC,
Canada; 2Division of Dermatology, Department of Medicine, Queen’s University, Kingston,
Ontario, Canada; 3Child and Family Research Institute, University of British Columbia,
Vancouver, British Columbia, Canada.
Abstract:
Autoimmune diseases, and in particular cutaneous autoimmune disease, increase the risk of
venous thromboembolism (VTE). Biochemical markers of hypercoagulability, such as Ddimer levels, correlate with disease activity and may provide a method of screening patients
for thromboembolic events.
We report a case of amyopathic dermatomyositis in which D-dimer levels and disease activity
were followed. The patient developed an elevated D-dimer level that correlated with active
cutaneous disease. One month after an elevated D-dimer level value was noted, the patient
suffered deep vein thrombosis (DVT) and bilateral pulmonary emboli (PE). Anticoagulant
therapy and changes to treatment of the dermatomyositis resulted in clinical improvement
with no recurrence of these thromboembolic events.
Dermatologists should be aware of the increased risk of VTE in patients with DM and that
this increased risk may include patients with clinically amyopathic DM. The clinical value of
screening DM patients and other autoimmune patients for thromboembolism in order to
prevent potentially fatal PEs and DVTs should be explored.
Contact information for MGK:
835 West 10th Avenue, Vancouver, British Columbia, Canada, V5Z 4E8
Phone: (604) 875-4747
Fax: (604) 873-9919
Email: kirchhof.mark@gmail.com
Conflicts of Interest: None to declare.
A Predictive Model of Disease Outcome in Rituximab-treated Myositis Patients Using
Clinical Features, Autoantibodies, and Serum Biomarkers
Jeannette M. Olazagasti, Cynthia S. Crowson, Molly S. Hein, Consuelo M. Lopez De Padilla,
Rohit Aggarwal, Chester V. Oddis, Ann M. Reed
Background/Purpose: Develop predictive models of early (8 week) and late (24 week)
disease outcomes using clinical features, autoantibodies, and serum biomarkers in patients
with refractory myositis treated with rituximab.
Methods: In the Rituximab in Myositis (RIM) trial, all subjects (76 with adult
dermatomyositis, 76 with adult polymyositis and 48 with juvenile dermatomyositis) received
rituximab (2 doses on consecutive weeks) with half the patients receiving drug at baseline
and half receiving drug 8 weeks later. Using start of treatment as baseline, serum samples
(n=177) were analyzed at baseline and after 8 and 24 weeks after rituximab. Potential
predictors included the following baseline factors: clinical features, serum muscle enzymes,
interferon gene score, autoantibodies (anti-synthetase n=28, TIF1- n=19, Mi-2 n=25, SRP
n=21, NXP2 n=18, non-myositis associated n=24, undefined autoantibody n=9), and
cytokines/chemokines measured by multiplexed sandwich immunoassays (Meso Scale
Discovery) (type-1 IFN-inducible [IP-10, I-TAC, MCP1], Th1 [IFNγ, TNFα, IL2], Th2 [IL4, IL5,
IL10, IL12, IL13], Th17 [IL6, IL17, IL1β] and regulatory cytokines [IL10, TNF, MIP-1α, MIP1β]). Our primary definition of response to treatment was based on absolute change from
baseline to 8 weeks and 24 weeks in physician global visual analog scale (VAS), muscle VAS,
and extramuscular VAS. Multivariable linear regression models were developed using
stepwise variable selection methods.
Results: Preliminary models were built with good predictive ability both for change in
physician global assessment and muscle disease activity at 24 weeks (R-square=0.41 and
0.40, respectively). The model for change in physician global assessment included the
following baseline clinical and lab features: muscle disease activity, physician global
assessment, and I-TAC (Table). The model for change in muscle disease activity included
baseline physician global assessment, skeletal disease activity, I-TAC and IFNγ. Similarly, a
predictive model was built with excellent predictive ability (R-square=0.67) for change in
extramuscular disease activity at 24 weeks. This model included the following baseline
clinical and lab features: constitutional, skeletal and extramuscular disease activity by VAS,
and MIP-1β and Mi-2. We also built models from baseline to 8 weeks but their predictive
ability was inferior compared to those for 24 weeks (R-square<0.3).
Conclusion: Changes in disease activity over time following treatment with rituximab in
patients with refractory myositis can be predicted. These models could be clinically useful to
optimize treatment selection in these patients.
Outcomes ->
Predictors
(baseline)
Physician
Global
Assessment
Muscle
Disease
Activity
Extramuscular
Global
Assessment
Skeletal
Disease
Activity
Constitutional
Disease
Activity
Mi-2
I-TAC
IFNγ
MIP-1β
Change in Physician
Global VAS
Coefficient
P-value
Change in Muscle VAS
Coefficient
P-value
Change in
Extramuscular VAS
Coefficient
P-value
-0.80
0.0007
-0.33
0.002
--
--
0.51
0.0008
--
--
--
--
--
--
--
--
0.60
0.0002
--
--
0.38
0.02
0.26
0.04
--
--
--
--
0.23
0.02
--0.01
---
-0.025
---
--0.02
-0.35
--
-0.01
0.01
--
-15.47
--0.008
0.002
--<0.0001
SYSTEMIC SCLEROSIS
Calcinosis is associated with digital ulcers and osteoporosis in patients with Systemic
Sclerosis: A Scleroderma Clinical Trials Consortium Study
Antonia Valenzuela1, Murray Baron2, Ariane Herrick3, Susanna Proudman4, Wendy Stevens5,
Tatiana S. Rodriguez-Reyna6, Alessandra Vacca7, Thomas A. Medsger Jr.8, David Fiorentino9,
Lorinda Chung1.
1Department
of Immunology and Rheumatology, Stanford University School of Medicine,
of Rheumatology, Jewish General Hospital McGill University, 3Department of
Rheumatology, University of Manchester, 4Rheumatology Unit, Royal Adelaide Hospital North
Terrace, 5Department of Rheumatology, St. Vincent’s Hospital Melbourne, 6Department of
Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador
Zubirán, 7II Chair of Rheumatology, University of Cagliari-Policlinico Universitario,
2Department
8Department
9Department
of Medicine/Rheumatology, University of Pittsburg School of Medicine,
of Dermatology, Stanford University School of Medicine
Background: Calcinosis is a debilitating cutaneous complication of systemic sclerosis (SSc)
with no effective treatments. We sought to determine the clinical factors associated with
calcinosis in an international multi-center collaborative effort with the Scleroderma Clinical
Trials Consortium (SCTC).
Methods: This is a retrospective cohort study of 5162 patients with SSc from 7 centers
within the US, Australia, Canada, United Kingdom, Italy, and Mexico. Calcinosis was defined as
the presence of calcium deposition in the skin and/or subcutaneous tissues as determined by
physical examination and/or radiography. Logistic regression was used to obtain odds ratios
(OR) relating calcinosis to various clinical features in multivariate analyses.
Results: The cohort was 84.8% female, 81.6% Caucasian, mean age at first non-RP symptom
was 45±14.4 years, and median follow-up was 2 years (range 0 -19). 38.3% had diffuse
cutaneous SSc, 60.6% had limited cutaneous SSc, and 0.8% had SSc sine sclerosis. A total of
1225 patients (24%) had calcinosis. Patients with calcinosis were older than patients without
calcinosis (59.5 ± 12.7 vs. 56.8±13.4), more likely to be female (88.9% vs. 83.6%), had higher
modified Rodnan skin score at baseline (10.9 ± 9.9 vs. 10± 10.6), and had longer disease
duration from first non-Raynaud’s phenomenon symptom to baseline (13± 10.4 vs. 8.3±9.2)
(p-value <.0001). They were more likely to have digital ulcers (65.8% vs. 34.4%) and
telangiectasias (89.9% vs. 64.9%). Regarding SSc-associated internal organ involvement,
patients with calcinosis were more likely to have cardiac disease (17.5% vs. 12.5%),
pulmonary hypertension (16% vs. 13.7%), gastrointestinal involvement (73.7% vs. 63%),
and arthritis (30% vs. 26.7%), but less likely to have myositis (8.2% vs. 12.3%). Osteoporosis
was much more common in patients who had calcinosis (11.8% vs. 2.7%). Autoantibodies
independently associated with the presence of calcinosis included anticentromere (ACA),
PM-1, and anticardiolipin antibodies. In multivariate analysis also inclusive of female gender,
ACA, arthritis, cardiac and gastrointestinal disease, the strongest associations with calcinosis
were digital ulcers (OR 3.4, 95%CI 2.4-4.9, p=<.0001), and osteoporosis (OR 4.4, 95%CI 2.38.5, p=<.0001) (Table 1).
Conclusion: Almost one quarter of patients with SSc have calcinosis. Our data support a
strong association of calcinosis with digital ulcers as well as osteoporosis, which may shed
light on the pathogenesis of calcinosis and guide the development of future therapies.
MORPHEA
Classification of morphea severity
Tina Michelle S. Vinoya, MD and Heidi T. Jacobe, MD, MSCS
University of Texas Southwestern Medical Center Department of Dermatology, Dallas, Texas
Background: Morphea, or localized scleroderma, is an inflammatory condition that affects
the dermis and extends into the subcutaneous fat and fascia. This produces thickening and
hardening of the skin. Recently-validated skin scoring tools in morphea are the Localized
Skin Severity Index (LoSSI), which describes activity and the Localized Scleroderma Disease
Severity (LoSSDI), which describes damage and extent of cutaneous lesions. To date, the
LoSSI and the LoSSDI have not been examined with regards to the clinical significance of
individual scores. This makes it difficult to put any given score in context clinically in terms of
what constitutes mild, moderate and severe disease.
Objective: We aim to develop an index of severity of disease for morphea.
Design: The Morphea Adult and Children (MAC) Cohort cross sectional study
Setting: The morphea clinic at the University of Texas Southwestern Medical Center at Dallas
Interventions: None
Main Outcome Measures: The LoSSI, LoSSDI, and the Physicians’ Subjective Assessment of
Activity and Damage were completed at every visit.
Results: Disease severity was assessed in one clinic visit of 51 patients enrolled in the MAC
Cohort from June 2007 to September 2014. Mean and standard deviation of the LoSSI
showed no significant difference in classifying disease activity into mild and moderate.
Analysis of the LoSDI showed that disease damage could be categorized into mild, moderate
and severe.
Limitations: Due to the rarity of the disease, a small sample size was used.
Conclusion: The LoSSI, LoSSDI and Physician’s Subjective Assessment of Severity can be
used as indices of morphea severity. For future studies, we recommend that more patients be
included and a wider range of assessment of activity and damage scores be used.
Key words:
Morphea, Localized Scleroderma, LoSSI, LoSSDI, Physician’s Subjective Assessment of
Activity and Damage
A Longitudinal Study of the Impact of Morphea on Quality of Life Over Time
Noelle Teske, BA, MSc, Simer Grewal, BA, Heidi T. Jacobe, MD, MSCS
Department of Dermatology
University of Texas Southwestern Medical Center, Dallas, TX
Background: Cross-sectional studies have examined the effect of morphea on health-related
quality of life (HRQOL). However, little is known about the effect of disease duration on
HRQOL in morphea.
Objective: Describe longitudinal changes in HRQOL in morphea over time and baseline
demographic and clinical features that are associated with impairment of HRQOL in morphea
Methods: Participants were selected from the prospective Morphea in Adults and Children
(MAC) cohort. Inclusion criteria were: age ≥18 years and ≥ 2 visits with a recorded HRQOL
measure: Dermatology Life Quality Indexes (DLQI), Skindex-29+3 (with Morphea-specific
subscale), and Short Form-36 (SF-36). Demographic features including age at first visit, sex,
and race, as well as clinical features of morphea subtype, disease activity and damage
(measured by Physician Global Assessment of Disease (PGA) activity and damage scores,
Localized Scleroderma Skin Severity Index (LOSSI) and damage correlate LOSDI scores) were
assessed as predictors.
Results: 142 patients met inclusion criteria and had a range of 2-4 visits annually. The
median DLQI score for participants was 4 at the initial visit (n=142), 2 at the second visit
(n=142), 2 at the third visit (n=68), and 1 at the fourth visit (n=36), reflecting a significant
improvement in HRQOL over time (p<.0001). At initial visit, DLQI scores did not differ
significantly by any demographic features, (age, sex, or race). Clinical features of morphea
subtype and disease damage (PGA Damage, LOSDI) did not show significant associations with
HRQOL. However, skin disease activity was correlated with poorer HRQOL at initial visit as
measured by the LOSSI (r=.2198, p=.0445). Skin disease activity decreased significantly over
time as measured by PGA Activity (p<.0001) and LOSSI scores (p<.0001). Skin damage (PGADamage and LOSDI) remained stable over the study duration. The QoL for patients with
morphea was shown to be worse than the general population, with median SF-36 PCS and
MCS scores below 50 at initial visit (n=38), and median Skindex scores were comparable to
those shown in other skin diseases such as alopecia and eczema.
Limitations: Participants were enrolled at a tertiary referral center and small sample sizes
for some measures, visits, and population subsets may limit analysis.
Conclusions: HRQOL was initially impaired in morphea patients as measured by the DLQI,
but improved over time as disease activity subsided. Impairment in HRQOL was similar
across age, gender, race, and morphea subtypes, but patients with increased disease activity
had greater impairment. In contrast to studies in other diseases (acne, vitiligo), damage was
not associated with greater impairment. This implies a potential role for adjustment to
chronic disease over time.
VASCULITIS
Three cases of macular arteritis: Separate entity or on the clinical spectrum of
cutaneous polyarteritis nodosa
Cecilia Larocca MD, Deon Wolpowitz MD PhD, Christina Lam MD
Department of Dermatology, Boston University School of Medicine, Boston, MA
Background: Macular arteritis, also known as macular lymphocytic arteritis or lymphocytic
thrombophilic arteritis, is a recently described clinical entity. It is characterized by
hyperpigmented macules of the lower extremities and histologically by a lymphocytic
arteritis. Given the histopathological overlap of this entity with the late stage findings of
cutaneous polyarteritis nodosa (PAN), controversy exists as to whether this represents a
latent or indolent form of cutaneous PAN or a separate entity.
Methods: We conducted a retrospective chart review of three cases of macular arteritis seen
at our institution in which we describe the demographic, clinical, histologic and laboratory
findings of these cases. We compare these cases with the existing 15 cases described in the
literature. We also compare and contrast macular arteritis with cutaneous polyarteritis
nodosa and other lymphocytic vasculitides.
Results: The median age of diagnosis is 37.5 years with a range of 6-73 years of age. The
majority of patients are female (83%) and Black (50%). The median duration of the skin
lesions is 12 months prior to presentation. Clinically, asymptomatic hyperpigmented macules
on the lower extremities are noted. Other common features include erythematous macules
and a livedoid pattern. The majority of lesions are asymptomatic but 22% of patients report a
mild pruritus. There are no defining serologies. To date there are no reports of systemic
involvement. Histopathologically all lesions have a lymphocytic arteritis. Commonly
considered differential diagnoses include polyarteritis nodosa, Sneddon’s syndrome, or a
thrombotic vasculopathy.
Conclusion: Here we present three new cases of macular arteritis to help expand the clinical
spectrum of this novel entity. Comparison of these patients with the known clinical history,
histological pattern, and evolution of skin lesions in PAN provides support that macular
arteritis is a distinct entity.
PSORIASIS
TNF-α Inhibitor-Induced psoriasis: A decade of experience at the Cleveland Clinic
Sean Mazloom, Stephanie Saed, Anthony P. Fernandez
Cleveland Clinic, Department of Dermatology
Background: Development of psoriasiform dermatitis precipitated by TNF-α inhibitor
therapy is a poorly understood phenomenon.
Objective: The goal of this study was to gain insight into this reaction by characterizing
affected patients seen at our institution over the past ten years. Methods: A systematic
retrospective chart review of all patients with TNF-α Inhibitor-induced psoriasis from 2003
to 2013 was performed. Results: 102 patients who developed TNF-α Inhibitor-induced
psoriasis were identified. 73.5% of affected patients were females. Age at onset ranged from
8-80 years, with a mean of 40 years. Crohn’s disease (46%) and Rheumatoid arthritis
(24.5%) were the most common primary conditions for which TNF-α Inhibitors were
prescribed. Among inciting TNF-α Inhibitors, infliximab was most common (51%). The most
common subtypes of psoriasis developing were plaque-type (49.5%), scalp (47.5%), and
palmoplantar pustulosis (41%). Patients who developed psoriasiform dermatitis while
taking infliximab had a higher percentage of scalp (58%) and inverse psoriasis (40%)
compared with those taking other TNF-inhibitors. Fifty biopsy specimens from 41 patients
were reviewed, and psoriasiform or spongiotic reaction patterns characterized the vast
majority (>85%). Plasma cells (62%) and eosinophils (81%) were found in the majority of
specimens. No trend was observed between histologic characteristics and medication type.
Characteristics did not significantly differ depending on time period between rash onset and
biopsy date. A variety of treatment interventions were attempted. Discontinuation of inciting
TNF-α Inhibitor agent with or without other interventions improved or resolved the psoriatic
lesions in 70% of cases. Methotrexate at doses >15mg/wk and cyclosporine also effectively
controlled the reaction in the majority of patients treated with these.
Conclusion: This study represents the largest single-institution cohort of patients with TNFα Inhibitor-induced psoriasis to date. Our cohort displayed many features described in other
published cohorts. The variability in onset, clinical presentation, and disease course suggests
that an additional insult, either endogenous or exogenous, is required to precipitate this
reaction. The presence of plasma cells and eosinophils suggests this reaction is distinct from
psoriasis vulgaris. Future studies focusing on potential inciting factors may add further
insight into the pathogenesis of this paradoxical reaction.