Innovation in

Dermatomyositis
Kimberly B. Hashemi, MDa,b,c,1, Katharina S. Shaw, MDd,e,1, Rochelle Castillo, MD, MSa,b,c,
Ruth Ann Vleugels, MD, MPH, MBAa,b,c,*

KEYWORDS
 Dermatomyositis  Cutaneous dermatomyositis  Myositis-specific antibodies  Interferon
 Emerging treatments

KEY POINTS
 The discovery of myositis-specific autoantibodies has led to the identification of biologically distinct
subtypes of dermatomyositis (DM) with differential risk for associated malignancy and extracutane-
ous disease.
 Recently published international guidelines for idiopathic inflammatory myopathy–associated can-
cer screening provide the first evidence-based and consensus-supported recommendations for
malignancy screening in dermatomyositis patients.
 The identification of type I interferon dysregulation as a key player in DM disease pathogenesis has
led to the identification of novel therapeutics for recalcitrant skin disease in DM.

INTRODUCTION systemic disease and malignancy. This article fo-

cuses on the clinical presentation of distinct DM
Dermatomyositis (DM) is an idiopathic, multi- subtypes, recent updates in screening guidelines
system inflammatory disease that typically pre- for malignancy in patients with DM, new insights
sents with symmetric proximal muscle weakness into the immunologic underpinnings of DM, and
and hallmark cutaneous findings (Fig. 1).1,2 Clinical the management of cutaneous DM, with a partic-
manifestations of DM are protean, with some pa- ular emphasis on new and emerging therapeutics.
tients experiencing skin-limited disease, whereas
others developing serious extracutaneous mani-
BACKGROUND
festations including interstitial lung disease (ILD),
myopathy, esophageal dysfunction, and inflam- DM is defined by the presence of characteristic
matory arthritis, among others. In addition, adult- cutaneous findings with or without concomitant
onset DM is associated with an increased risk of myositis.6 Patients with both muscle and skin
malignancy, particularly in the 2 to 3 years before involvement are classified as having classic DM.
and after DM onset.3–5 Early recognition of cuta- When patients present without muscle disease or
neous signs of DM is key to promptly instituting with subclinical elevations in muscle enzymes for
appropriate therapy and screening protocols for 6 months or more, they are classified as amyopathic

a
Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, 221 Longwood
Avenue, Boston, MA 02115, USA; b Dermatology Program, Division of Immunology, Boston Children’s Hospital,
300 Longwood Avenue, Boston, MA 02115, USA; c Autoimmune Skin Disease Program, Department of Derma-
tology, Brigham and Women’s Hospital, Harvard Medical School, 221 Longwood Avenue, Boston, MA 02115,
USA; d Division of Dermatology, The Children’s Hospital of Philadelphia, 3615 Civic Center Boulevard, Philadel-
phia, PA 19104, USA; e Department of Dermatology, Perelman School of Medicine, University of Pennsylvania,
derm.theclinics.com

3700 Walnut Street Suite 1, Philadelphia, PA 19104, USA

1
Denotes co-first authors.
* Corresponding author. 221 Longwood Avenue, Boston, MA 02115.
E-mail address: rvleugels@bwh.harvard.edu

Dermatol Clin 43 (2025) 103–110

https://doi.org/10.1016/j.det.2024.08.008
0733-8635/25/Ó 2024 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training,
and similar technologies.
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104 Hashemi et al

Fig. 1. Characteristic cutaneous manifestations of DM. (A) Pink to violaceous erythema on the forehead, upper
eyelids (heliotrope sign), and midface with involvement of the nasolabial folds; (B) photodistributed poikilo-
derma and erythema on the upper chest (V-neck sign) and (C) upper back and shoulders (shawl sign); (D) viola-
ceous macules and papules overlying extensor surfaces (Gottron sign) with associated ulceration; (E)
erythematous papules studding the metacarpal and interphalangeal joints of the dorsal hand (Gottron papules);
(F) periungual erythema and edema, nailfold capillary dilation and drop out, and cuticular hypertrophy with he-
mosiderin deposition; (G) poikiloderma overlying the lateral thigh (holster sign); (H) scalp erythema, psoriasiform
scale, and nonscarring hair loss.

DM (ADM) and hypomyopathic DM (HDM), respec- extensor surface involvement (Gottron sign). Pink
tively—collectively, patients with ADM and HDM to violaceous papules overlying the metacarpal
are considered to have clinically amyopathic dis- and interphalangeal joints, also known as Gottron
ease (CADM) and, importantly, constitute at least papules, are also characteristic of DM. Other sug-
20% of patients with DM overall.7 The skin manifes- gestive cutaneous features include periungual ery-
tations of CADM are analogous to those seen in thema and edema, nailfold capillary dilation,
classic DM, and patients with CADM remain at tortuosity and drop out, and cuticular hypertrophy.
risk for systemic disease involvement. Thus, der- Lastly, patients frequently present with scalp
matologists play a critical role not only in identifying involvement, characterized by pruritus, nonscar-
patients with cutaneous findings that suggest DM ring alopecia, erythema, and psoriasiform scale.
but also in screening for underlying ILD, myositis, Although careful evaluation for underlying ILD,
and malignancy in a timely fashion. Early recogni- myositis, and/or malignancy is necessary in all pa-
tion of characteristic cutaneous stigmata is of tients presenting with DM, it is important to recog-
particular clinical relevance, as patients with DM nize that certain cutaneous findings may portend
are at highest risk for developing ILD and malig- differential risk for certain systemic sequelae. In
nancy in the first year after cutaneous disease recent years, our understanding of distinct clinical
onset.8 phenotypes of DM, including their associated
Classically, DM patients present with pink to cutaneous features, has continued to evolve.
lilac erythema on the upper eyelids, known as
the heliotrope sign, along with midfacial erythema CORRELATES IN DISEASE PHENOTYPE AND
involving the nasolabial folds (NLFs). It is important MYOSITIS-SPECIFIC AUTOANTIBODIES
to differentiate the midfacial erythema of DM from
the malar rash of lupus, as the latter classically The recent discovery of myositis-specific autoan-
spares the NLF. Other pathognomonic features tibodies (MSAs) has been an important step for-
of DM include a photosensitive eruption on the ward in understanding DM disease heterogeneity
chest (V sign) and upper back (shawl sign), poikilo- and paving the way for personalized medicine.9,10
derma on the lateral thighs (holster sign), and Broadly speaking, MSAs are associated with

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 Innovation in Dermatomyositis 105

distinct clinical phenotypes, and it is now possible particularly in those patients with signs and symp-
to use MSAs to stratify patients with DM for risk of toms of rapidly progressive ILD, severe myositis,
potential disease complications and associated or underlying malignancy. Thus, MSAs must be
outcomes (Table 1). considered as an adjunctive tool for clinicians at
Importantly, before reviewing specific phenotype- this time—MSAs can be used to assist in clarifying
MSA correlates, we must urge readers to exercise relative risk for systemic complications, anticipating
caution when interpreting MSAs in the context of disease course, and/or lending support to a unifying
the patient’s overall clinical picture. Notably, no diagnosis, but we must emphasize that the diag-
gold standard for MSA testing exists at this time. nosis of DM can and should be made without MSA
Not only are there several different methodologies testing. That said, international efforts to standardize
for detecting MSAs currently in practice (ranging MSA testing are ongoing, and as such, MSAs may
from immunoprecipitation to enzyme-linked immu- make their way into formal diagnostic schema for
nosorbent assay to line immunoblot assays), but DM going forward.
due to individual laboratories using their own MSAs that define distinct clinical phenotypes of
proprietary assays, MSA results can vary, even DM include anti-transcriptional intermediary factor
within the same patient.11 Moreover, turnaround 1g (TIF-1g), -Sp4, -CCAR1, -Mi-2, -NXP-2, -MDA-
times can exceed weeks to months. Delaying sys- 5, -SAE, -SRP, and -aminoacyl-tRNA synthetase
temic workup for MSA assessment is inappropriate, antibodies (see Table 1). These MSAs seem to

Table 1
Myositis-specific autoantibodies and associated clinical features

Antigen Cutaneous Manifestations Clinical Features

TIF-1g Red-on-white telangiectatic patches; Increased risk of malignancy,
ovoid palatal patch; psoriasiform low risk of ILD
plaques; severe, extensive and
recalcitrant skin disease
Sp4 (may coexist Same as TIF-1g Attenuates risk of malignancy
with TIF-1g) in TIF-1g-positive patients
CCAR1 (may coexist Same as TIF-1g Attenuates risk of malignancy
with TIF-1g) in TIF-1g-positive patients
Mi-2 Classic cutaneous features of DMa Milder muscle disease with overall
favorable prognosis and brisk
response to treatment
NXP-2 Calcinosis, peripheral edema, Increased risk of malignancy,
milder cutaneous disease myositis with risk for severe
dysphagia, intestinal vasculopathy
MDA-5 Cutaneous ulcerations (overlying Increased risk of ILD, inflammatory
Gottron papules and Gottron sign), arthritis, typically clinically
oral ulcerations, vasculopathic amyopathic, rarely malignancy
skin lesions (painful palmar associated
macules and papules), alopecia
SAE “Angel wing” poikilodermatous Clinically amyopathic at disease
eruption on back, severe and onset, variable development
recalcitrant skin disease of myositis, dysphagia
SRP Mild cutaneous disease Necrotizing myopathy (severe
weakness, high CK), often
refractory to treatment
Aminoacyl-tRNA Mechanics hands Fever, erosive polyarthritis, Raynaud
synthetasesb phenomenon, increased risk for ILD

Abbreviations: CCAR1, cell division cycle and apoptosis regulator protein 1; CK, creatine kinase; DM, dermatomyositis;
ILD, interstitial lung disease; MDA-5, melanoma differentiation associated gene 5; Mi-2, nucleosome remodeling deace-
tylase complex; NXP-2, nuclear matrix protein 2; SAE, small ubiquitin-like modifier activating enzyme; Sp4, transcription
factor Sp4; SRP, signal recognition particle: TIF-1g, transcription intermediary factor 1 gamma.
a
Classic cutaneous features of dermatomyositis include heliotrope sign, V-neck sign, shawl sign, Gottron papules, and
Gottron sign.
b
Anti-aminoacyl-tRNA synthetase antibodies include anti-Jo-1, -PL-7, -PL-12, -EJ, -OJ, and -KS.

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106 Hashemi et al

not only portend differential risk for systemic organ is important to recognize that the real-world perfor-
involvement, response to treatment, and overall mance of these cancer screening recommenda-
disease course but also reflect differing HLA sub- tions has yet to be empirically investigated.
types, transcriptomic profiles, and systemic inter- Although efforts to validate these guidelines both
feron (IFN) levels. Although we have traditionally prospectively and within existing DM cohorts are
thought of MSAs as being mutually exclusive, the ongoing, the authors encourage clinicians to refer-
recent discovery of Sp4 and CCAR1 has chal- ence these guidelines as a starting point when
lenged this notion. Interestingly, when these auto- approaching malignancy screening for their pa-
antibodies cooccur with TIF-1g (an MSA that, on tients with DM.
its own, portends a higher risk for malignancy
[see Table 1]), Sp4 and CCAR1 seem to have a NEW INSIGHTS INTO THE IMMUNOLOGIC
protective effect against malignancy.11–13 Finally, UNDERPINNINGS OF DERMATOMYOSITIS
whether MSAs themselves play a pathogenic role
in DM (or are just innocent bystanders) is unclear To date, the cause and pathogenesis of DM remain
and warrants further investigation. incompletely understood. Although the current
literature supports a role for genetic, environ-
UPDATES IN SCREENING GUIDELINES FOR mental, and autoimmune factors in DM, a complete
MALIGNANCY IN DERMATOMYOSITIS characterization of the immunologic march under-
lying DM disease pathogenesis remains elusive.
The International Myositis Assessment and Clin- In addition, heterogeneity in disease phenotype
ical Studies Group recently published the first ev- (eg, amyopathic vs classic vs paraneoplastic DM)
idence- and consensus-based cancer screening likely reflects distinct pathomechanisms of action
guidelines for patients with idiopathic inflamma- within DM itself.
tory myopathies (IIMs), including DM. Given the Type I IFNs are a cytokine class that includes
well-documented association between DM and IFN-a and IFN-b. Binding of type I IFNs to their re-
malignancy,14–16 these recommendations aim to ceptor on target cells stimulates the transcription
provide standardized guidance for cancer and translation of a specific subset of genes
screening by stratifying patients as “high,” “inter- (type I IFN–inducible genes) that play a role in anti-
mediate,” or “low” risk for malignancy based on a viral innate immunity. Although the relationship be-
constellation of factors including age of disease tween type I IFNs and DM has been reported in the
onset, autoantibody profiles, and clinical features. literature for over a decade, our understanding of
Based on the patient’s risk status, the guidelines the role type I IFNs play in the pathogenesis of
recommend either basic or enhanced screening DM has expanded in recent years.
panels: patients at low risk for malignancy are rec- Much of the initial work interrogating the patho-
ommended to have “basic” cancer screening, genesis of DM focused on gene expression
which includes routine blood and urine studies, profiling from muscle biopsies. These studies
plain chest films, and up-to-date age- and sex- identified a marked overproduction of type I IFN–
appropriate cancer screening. Patients deemed inducible transcripts and proteins in the muscle
to be at intermediate or high risk for malignancy of patients with DM.18 Later studies examining
are recommended to have “enhanced” cancer gene expression from the peripheral blood, lung,
screening, which includes computed tomography and skin of patients with DM similarly showed
(CT) scans of the neck, thorax, abdomen, and a marked upregulation of type I IFN–inducible
pelvis; pelvic/transvaginal ultrasonography; Pap gene products.18–20 Although these early studies
smear; mammography; prostate-specific antigen demonstrated a strong correlation between type
or cancer antigen (CA)-125 blood tests; and fecal I IFNs and DM, it was not until recently that type
occult blood test. In addition, the published rec- I IFNs were found to correlate with skin disease ac-
ommendations also provide guidance on the tivity directly: notably, transcriptomic analyses of
timing and frequency of subsequent cancer both blood and lesional skin from patients with
screenings.17 DM have demonstrated an association between
Fundamentally, the aim of these guidelines is to elevated type I IFN gene signatures and more se-
ensure that all patients with DM receive adequate vere skin involvement.21–23 Most recently, IFN-b
malignancy screening. They are accessible to clini- has specifically been identified as a likely patho-
cians worldwide, including those with limited expe- genic driver in DM skin disease.24
rience managing DM, with the hope of facilitating The identification of type I IFNs, specifically IFN-
early cancer detection and potentially improving b, as a primary catalyst in DM skin disease has
outcomes. Although these guidelines represent a facilitated both the repurposing of already avail-
major step forward for cancer screening in DM, it able medications targeting the type I IFN pathway

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 Innovation in Dermatomyositis 107

as well as novel drug development. To that end, FDA-approved for patients with DM with both skin
there are several ongoing clinical trials in this and muscle involvement. Given that up to 20% of
area, and we may soon see new Food and Drug patients with DM have skin predominant disease,7
Administration (FDA)-approved therapies for DM a crucial need to include patients with amyopathic
including a novel antagonist of the Janus kinase DM in future clinical trials persists.
1 and tyrosine kinase 2 (JAK1/Tyk2) receptors25 However, despite IVIg’s demonstrated efficacy
and a monoclonal antibody targeting IFN-b.26 and safety in DM, prescribing practices vary signif-
icantly among providers. This variance is likely
TREATMENT ADVANCES IN influenced, at least in part, by IVIg’s black box
DERMATOMYOSITIS warning for venous thromboembolism (VTE). The
risk of VTE associated with IVIg may be overstated,
Historically, the treatment of DM has relied on as it is confounded by the fact that DM itself is
therapeutics with broad immunomodulating prop- linked to an increased risk of VTE.30 Research has
erties. Systemic corticosteroids are frequently indicated that there is no discrepancy in the rate
administered as first-line therapy, followed by of VTE between patients with DM receiving IVIg
traditional nonsteroidal immunosuppressants, and those who do not.31 Although caution is war-
most commonly methotrexate or mycophenolate ranted in patients with additional underlying risk
mofetil. Although these agents are often effective factors such as malignancy, smoking, and coagul-
in treating the extracutaneous manifestations of opathy, these are not necessarily absolute contra-
DM, they can fall short in managing skin disease. indications for IVIg use. In fact, IVIg is often
The recently published ProDERM (Progress in considered the preferred option for patients with
DERMatomyositis) trial marked one of the first sig- paraneoplastic DM to avoid unnecessary immuno-
nificant milestones in DM treatment to date. This suppression. Nonetheless, the benefits and risks
trial was a double-blind, randomized, placebo- must be carefully evaluated in each patient, and
controlled phase 3 trial (RCT) designed to assess consultation with a hematologist may be necessary
the efficacy and safety of intravenous immuno- in certain cases.
globulin (IVIg) in DM. The primary endpoint was Although the incidence of VTE is fortunately
achieving a total improvement score (TIS) of at quite low, many patients experience headaches
least 20 (indicating at least minimal improvement) during IVIg treatment.27 Based on the investiga-
at week 16. The TIS is a weighted composite tors’ experience, the most effective interventions
score that reflects the change in six measures of to reduce this risk include extending the infusion
myositis activity over time, where higher scores duration to a minimum of 6 to 7 hours and admin-
indicate greater improvement. Seventy-nine istering concurrent IV fluids. In addition, clinicians
percent of patients in the treatment group versus may consider modifying the infusion schedule
44% in the placebo group (P<.001) achieved the from 2 gram (g)/kilogram (kg) over two consecutive
primary endpoint. The results from this RCT ulti- days every 4 weeks to 1 g/kg/day every 2 weeks. It
mately led to the first (and only) FDA-approved is worth noting that although this regimen has not
therapy for DM.27 Notably, this trial excluded pa- been studied in clinical trials, in the investigators’
tients with skin predominant disease. A post-hoc clinical practice, it has proved to be equally effec-
analysis of the ProDERM trial was performed to tive and has reduced the incidence and severity of
evaluate the efficacy of IVIg in cutaneous DM.28 headaches.
The investigators assessed skin disease severity Unfortunately, IVIg is not universally effective,
using the cutaneous dermatomyositis disease with some patients experiencing recalcitrant dis-
area and severity index activity (CDASI-A) score, ease. JAK inhibitors have increasingly been used
a validated DM skin outcome measure in which in this setting, as they have demonstrated remark-
a reduction of four to five points correlates able efficacy in patients with refractory cutaneous
with a clinically meaningful improvement in skin DM.32,33 The efficacy of JAK inhibitors stems from
disease severity.29 Results from the post-hoc their role in blocking the downstream signaling of
analysis demonstrated that those treated with type I IFNs. Unfortunately, JAK inhibitors are not
IVIg compared with placebo had a significant FDA-approved for DM, and thus their use is often
reduction in CDASI-A score (9.4 vs 1.2, respec- complicated by insurance challenges. That said,
tively), underscoring the efficacy of IVIg in the there is an ongoing phase 3 clinical trial for brepo-
management of cutaneous DM. Moreover, IVIg citinib, a small molecule inhibitor that blocks both
improved cutaneous disease regardless of JAK1 and Tyk2 receptors.25 It is worth noting
disease severity at baseline.28 Although IVIg rep- that type I IFNs primarily signal through JAK1/
resents an important step forward in the manage- Tyk2 receptors,34 and as such, an FDA-approved
ment of DM, it is important to note that IVIg is only JAK inhibitor for DM may be on the horizon.

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108 Hashemi et al

For those patients who fail or have contraindica- risk assessment. Furthermore, the publication of
tions to treatment with JAK inhibition, therapeutic the first evidence- and consensus-based cancer
options remain limited at this time. However, the screening guidelines represents an important
recent identification of IFN-b as central to DM step toward standardizing malignancy screening
skin disease pathogenesis has facilitated novel in patients with DM going forward. In addition,
drug development. A global phase 3 RCT is identifying key immunologic drivers of skin disease
currently underway for a monoclonal antibody tar- in DM has facilitated the development of more tar-
geting IFN-b.26 Notably, this drug was created geted therapies. In addition to the recent FDA
based on the results of the translational study approval of IVIg for DM, several phase II and III
identifying IFN-b as central to DM skin disease clinical trials of novel therapeutics are underway,
severity.24 Thus, this is the first drug developed offering hope for the availability of more FDA-
specifically for patients with DM from patients approved treatment options for patients with DM
with DM. Results from the phase 2 clinical trial in the near future.
were recently presented at the 2023 American
Academy of Dermatology Late Breaking Research
Symposium.35 In the phase 2 trial, 55 patients CLINICS CARE POINTS
were randomly assigned to receive either 150 mg
of anti-IFN-b, 600 mg of anti-IFN-b, or placebo.
The primary endpoint assessed at 12 weeks was  Dermatologists should recognize the classic
a greater than 5-point reduction in the CDASI-A cutaneous stigmata of DM so that expedi-
score. The response rate was 100%, 96%, and tious workup for occult malignancy, ILD,
35.7% for the 150 mg dose, 600 mg dose, and pla- myositis, and/or other extracutaneous pathol-
cebo, respectively. These results are noteworthy, ogy can be pursued.
given that nearly all patients achieved clinically  Although MSAs may portend differential risk
meaningful improvement in skin disease activity. for systemic sequelae in DM, limitations to
Treatment-related adverse events were mild, and their use and interpretation persist in clinical
there were no major safety concerns or signals practice.
for herpes zoster or VTE.35  The International Myositis Assessment and
Finally, the recent off-label use of anifrolumab in Clinical Studies Group recently published
recalcitrant cutaneous DM bears mentioning. Ani- consensus-based, standardized cancer
frolumab, a monoclonal antibody targeting type I screening guidelines for IIMs including DM;
IFN receptor, recently received FDA approval for efforts to validate these recommendations
the treatment of moderate-to-severe systemic are ongoing.
lupus erythematous.36 Notably, similar to cuta-  IVIg was recently FDA-approved for the treat-
neous DM, dysregulated type I IFN signaling is ment of DM. For patients with cutaneous dis-
central to cutaneous lupus disease pathogenesis ease refractory to IVIg and traditional
as well.37 A recent study from the authors’ group DMARDs, JAK inhibitors and other therapies
demonstrated the efficacy of anifrolumab in a pa- targeting the type I IFN pathway may be
considered. Several clinical trials evaluating
tient with juvenile DM refractory to nonsteroidal
novel therapeutics in DM are ongoing, with
immunosuppressants, IVIg, and JAK inhibition. the promise of new and approved therapies
Remarkably, anifrolumab led to near-complete for DM on the horizon.
resolution of skin disease activity after only
2 months.38 This study further emphasizes the util-
ity of targeting the type I IFN pathway in patients
with refractory cutaneous DM. Although larger DISCLOSURE
prospective studies are needed, anifrolumab may
be considered in patients with DM with severe The authors have no conflicts of interest or funding
skin disease in whom standard therapies have sources to disclose.
failed or are contraindicated.
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