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Recently, more emphasis has been placed on patient-reported outcomes in clinical trials, which has inadvertently led to an increase in respondent burden. In this investigation, we found that the Skindex-16 form captures patient-reported quality of life in dermatomyositis as well as the Skindex-29 form, but with fewer questions. Additional questions regarding photosensitivity, body image, and cosmetic use did not correlate with skin activity in dermatomyositis.

BP is a rare autoimmune skin-blistering disorder with a profound negative impact on quality of life, associated with burdensome morbidity and possible mortality. There is a need for effective targeted therapies with a demonstrated safety profile for BP. Dupilumab is a fully human monoclonal antibody that blocks the shared receptor component for interleukin (IL)-4 and IL-13, key and central drivers of type 2 inflammation that may play a role in BP. Published case series describes the use of dupilumab in patients with BP, but it has not been formally assessed in a clinical trial for BP. We describe the design of the LIBERTY-BP ADEPT trial (NCT04206553) that aims to investigate the efficacy and safety of dupilumab in achieving sustained remission off oral corticosteroids (OCS) in patients with moderate-to-severe BP. LIBERTY-BP ADEPT is a global, randomized, double-blind, placebo-controlled, parallel-group study consisting of a 35-day screening period, a 52-week double-blind treatment period and a 12-week follow-up period. Inclusion criteria include age 18–90 years and clinical features of BP with a confirmed diagnosis based on histopathology, immunopathology, and serology. Exclusion criteria include previous treatment with IL-4 or IL-13 antagonists or systemic or medium-to-high potency topical corticosteroids within 7 days of the baseline visit. All patients receive standard OCS to control disease activity at the start of the treatment period. Post randomization, after 2 weeks of sustained remission, OCS is to be gradually tapered and discontinued as long as disease control is maintained. The primary endpoint is the proportion of patients achieving sustained remission at week 36. Key secondary endpoints include total cumulative OCS dose, percent change in weekly average daily Peak Pruritus Numerical Rating Scale (NRS) score, and proportion of patients with improved daily Peak Pruritus NRS score ≥4 from baseline to week 36. LIBERTY-BP ADEPT aims to enroll 98 patients; enrollment is ongoing and will include more than 17 sites in Europe. BP shares pathophysiological pathways with type 2 inflammatory diseases mediated by IL-4 and IL-13. The ongoing LIBERTY-BP ADEPT study is the first randomized, controlled trial designed to evaluate the efficacy and safety of dupilumab in patients with moderate-to-severe BP.

There have been a number of advances in the clinical and translational understanding of cutaneous lupus and dermatomyositis, which both disproportionately affect women. These advances have involved ongoing collaborations between dermatology and rheumatology that highlight the importance of the skin in these disorders, with improvement in the education of trainees and clinical management of these complex multisystem diseases. In addition, a new disease classification has allowed inclusion of patients with skin-predominant dermatomyositis, frequently associated with systemic findings, in the spectrum of idiopathic inflammatory myopathies. Validated outcome measures allow translational research and facilitate progress toward better and more targeted therapeutics. Clinical trials using disease severity tools, such as the Cutaneous Lupus Erythematosus Area and Severity Index and the Cutaneous Dermatomyositis Disease Area and Severity Index, allow measurement of improvement in the skin. Recent results of phase 2 and 3 trials clearly show that patients will benefit from collaborative interactions and studies between dermatology and rheumatology.

For patients with lupus erythematosus (LE) or dermatomyositis (DM), there is an urgent need to address a heightened risk of clinical events, chiefly heart attacks and strokes, caused by atherosclerotic cardiovascular disease (ASCVD). Patients with LE or DM frequently exhibit high levels of conventional risk factors for ASCVD events, particularly dyslipoproteinemia and hypertension; an amplified burden of atherosclerotic plaques; and increased age- and sex-adjusted rates of ASCVD events compared with the general population. The rate of ASCVD events exceeds what would be expected from conventional risk factors, suggesting that disease-specific autoimmune processes exacerbate specific, known pathogenic steps in atherosclerosis. Importantly, despite their heightened risk, patients with LE or DM are often undertreated for known causative agents and exacerbators of ASCVD. Herein, we propose an approach to assess and manage the heightened risk of ASCVD events in patients with LE or DM. Our approach is modeled in large part on established approaches to patients with diabetes mellitus or stage 3 or 4 chronic kidney disease, which are well-studied conditions that also show heightened risk for ASCVD events and have been explicitly incorporated into standard clinical guidelines for ASCVD. Based on the available evidence, we conclude that patients with LE or DM require earlier and more aggressive screening and management of ASCVD. We suggest that physicians consider implementing multipliers of conventional risk calculators to trigger earlier initiation of lifestyle modifications and medical therapies in primary prevention of ASCVD events, employ vascular imaging to quantify the burden of subclinical plaques, and treat to lower lipid targets using statins and newer therapies, such as PCSK9 inhibitors, that decrease ASCVD events in nonautoimmune cohorts. More clinical vigilance is needed regarding surveillance, prevention, risk modification, and treatment of dyslipidemias, hypertension, and smoking in patients with LE or DM. All of these goals are achievable.

Aggarwal et al. recently reported results from the first successful phase III trial studying intravenous immunoglobulin (IVIG) in dermatomyositis (DM). The study showed that improvement in disease activity was clinically and statistically significantly greater in those who received IVIG than in those who received placebo, allowing US Food and Drug Administration approval for use of IVIG in DM. Despite its success, this study also highlights several concerns that must be addressed to inform future trials in the field, particularly the inclusion of patients with skin-predominant/amyopathic and post-myopathic DM.

Dermatomyositis is a multisystem autoimmune connective tissue disease that can manifest with proximal muscle weakness, interstitial lung disease, and characteristic skin findings such as erythema and papules over the dorsal joints of the hand, periungual changes, heliotrope rash, and pruritic erythematous inflammation of the chest, back, arms, legs, and scalp. Erythromelalgia is a clinical diagnosis characterized by swelling, erythema, and burning pain, typically of the extremities and often triggered by heat or exertion. It can be classified as primary, due to genetic mutations, or secondary, associated with myeloproliferative, vascular, musculoskeletal, or neurologic disease.1 There have been reports of biopsies of erythromelalgia having some features of connective tissue disease.2,3 There are also reports of erythromelalgia associated with systemic lupus erythematosus and rheumatoid arthritis, two autoimmune connective tissue diseases.4 Here, we present two cases of erythromelalgia associated with dermatomyositis, which, to our knowledge, has not been reported in the literature to date. Case 1 An 81-year-old woman with a history of treated breast cancer presented to the dermatology clinic for evaluation of swelling and discoloration of her bilateral hands and feet of approximately 10 years' duration associated with heat intolerance. She had been seen by numerous vascular and lymphedema specialists over the years with the ultimate conclusion that her swelling was not associated with a vascular etiology. The patient noticed improvement of her symptoms when she was exposed to air conditioning or cold water. At presentation, she also reported shortness of breath, a burning sensation on her feet and scalp, subjective proximal muscle weakness, dysphagia, and weight gain. Her physical examination was notable for periungual telangiectasias, scale, and erythema on the V of her neck area, her extensor arms, and around her eyes (Fig 1). She believed that the erythema on the V of her neck area started 30 years ago, indicating the possibility that she had had potentially undiagnosed amyopathic dermatomyositis (DM) for years before her diagnosis of erythromelalgia. The patient's basic laboratory workup and immunologic studies were unrevealing, and a myositis panel was negative. Muscle magnetic resonance imaging and electromyography were not performed because of the absence of muscle pain or objective weakness on physical examination. Two skin biopsy specimens from the upper portion of the arm and the dorsal surface of the hand showed interface dermatitis (Fig 2), consistent with a diagnosis of DM based on clinicopathologic correlation with typical features of DM (as opposed to lupus, drug reaction, viral exanthem, lichen planus, or graft versus host disease). Pulmonary function testing showed an obstructive pattern with decreased carbon monoxide diffusion in the lung (62%), and chest computed tomography (CT) showed stable interstitial lung changes. The malignancy workup (including age-appropriate screening and CT of the chest, abdomen, and pelvis) was negative. The patient has had some relief from the burning sensation on her feet from topical 5% lidocaine and petroleum jelly. Because of shared decision-making and risk-benefit analysis, the patient has not started immunosuppressive therapy for her DM, but she is being followed closely. Open in a separate window Fig 1 Patient 1. Swelling and erythema on bilateral hands and feet.

Dermatomyositis (DM) is a rare, systemic autoimmune disease that most frequently affects the skin, muscles, and lungs. The inflammatory infiltrate in the skin has not been fully characterized, and, in this study, we took a single-cell, unbiased approach using imaging mass cytometry. Substantial monocyte‒macrophage diversity was observed, with the CD14+ population correlating positively with Cutaneous Dermatomyositis Disease Area and Severity Index scores (P = 0.031). The T-cell compartment revealed CD4+ T, CD8+ T, and FOXP3+ T cells. Activated (CD69+) circulating memory T cells correlated positively with Cutaneous Dermatomyositis Disease Area and Severity Index scores (P = 0.0268). IFN-β protein was highly upregulated in the T-cell, macrophage, dendritic cell, and endothelial cell populations of DM skin. Myeloid dendritic cells expressed phosphorylated peroxisome proliferator‒activated receptor γ, phosphorylated IRF3, IL-4, and IL-31, and their quantity correlated with itch as measured in Skindex-29. Plasmacytoid dendritic cells colocalized with IFN-γ in addition to the known colocalization with IFN-β. Nuclear phosphorylated peroxisome proliferator‒activated receptor γ expression was found in the DM endothelium. Imaging mass cytometry allows us to characterize single cells in the immune cell population and identify upregulated cytokines and inflammatory pathways in DM. These findings have important implications for the development of future targeted therapies for DM.

Dermatomyositis (DM) is a rare inflammatory disorder commonly manifesting with proximal muscle weakness and characteristic skin findings. Classic findings include erythematous papules or macules over the small joints of the hand, periungual changes, pruritic/erythematous skin on areas including the scalp, chest, upper back, and other surfaces, as well as interstitial lung disease. Anasarca1 and chronic hyponatremia2 have rarely been reported in cases of DM, and have only been reported separately. Here, we present a case of dermatomyositis associated with both anasarca and hyponatremia.

BACKGROUND: Bruton tyrosine kinase (BTK) inhibition targets B-cell and other non-T-cell immune cells implicated in the pathophysiology of pemphigus, an autoimmune disease driven by anti-desmoglein autoantibodies. Rilzabrutinib is a new reversible, covalent BTK inhibitor demonstrating preclinical efficacy as monotherapy in canine pemphigus foliaceus. OBJECTIVES: To evaluate the efficacy and safety of oral rilzabrutinib in patients with pemphigus vulgaris in a multicentre, proof-of-concept, phase II trial. METHODS: Patients with Pemphigus Disease Area Index severity scores 8-45 received 12 weeks of oral rilzabrutinib 400-600 mg twice daily and 12 weeks of follow-up. Patients initially received between 0 and ≤ 0·5 mg kg-1 prednisone-equivalent corticosteroid (CS; i.e. 'low dose'), tapered after control of disease activity (CDA; no new lesions, existing lesions healing). The primary endpoints were CDA within 4 weeks on zero-to-low-dose CS and safety. RESULTS: In total, 27 patients with pemphigus vulgaris were included: nine newly diagnosed (33%) and 18 relapsing (67%); 11 had moderate disease (41%) and 16 moderate to severe (59%). The primary endpoint, CDA, was achieved in 14 patients (52%, 95% confidence interval 32-71): 11 using low-dose CS and three using no CS. Over 12 weeks of treatment, mean CS doses reduced from 20·0 to 11·8 mg per day for newly diagnosed patients and from 10·3 to 7·8 mg per day for relapsing patients. Six patients (22%) achieved complete response by week 24, including four (15%) by week 12. Treatment-related adverse events were mostly mild (grade 1 or 2); one patient experienced grade 3 cellulitis. CONCLUSIONS: Rilzabrutinib alone, or with much lower CS doses than usual, was safe, with rapid clinical activity in pemphigus vulgaris. These data suggest that BTK inhibition may be a promising treatment strategy and support further investigation of rilzabrutinib for the treatment of pemphigus.

Background Outcome measures of clinical trials in cutaneous lupus erythematosus (CLE) should reflect clinically meaningful improvement in disease activity, as measured by the Cutaneous Lupus Disease Area and Severity Index activity score (CLASI-A). Objective We aimed to define the degree of improvement in disease activity meaningful to a patient's quality of life. Methods The change in the CLASI-A in 126 patients needed to predict meaningful change in QoL, as defined by the Emotions and Symptoms subscales of the Skindex-29, was evaluated by linear regression models. Results In patients with an initial CLASI-A of ≥8, a 42.1% or ≥7-point and a 31.0% or ≥5-point decrease in CLASI-A predicts meaningful improvement in the Emotions and the Symptoms subscales, respectively. Limitations This is a retrospective study of prospectively collected data at a single site. Conclusions A CLASI-A score of ≥8 for trial entry allows for inclusion of patients with milder disease where CLASI-A improvement by ≥50% is clinically significant and meaningful.

Drug-induced subacute cutaneous lupus erythematosus (DI-SCLE) is subacute cutaneous lupus erythematosus (SCLE) in which the disease onset is associated with the ingestion of an offending medication. It is estimated that approximately one-third of all SCLE cases are drug-induced.1 Idiopathic SCLE and DI-SCLE are serologically, histopathologically, and clinically identical, with both characterized by widespread erythematous annular-polycyclic or papulosquamous psoriasiform lesions.2 DI-SCLE symptoms can arise weeks to years after exposure to a number of medications, including proton-pump inhibitors (PPIs), antihypertensives, antibiotics, and anticonvulsants. A lesser-known phenomenon is a lupus-associated flare or change in lupus phenotype associated with medication initiation in a person who has already been diagnosed with systemic lupus erythematosus (SLE). Cases of patients with previous diagnoses of idiopathic SCLE in remission being exacerbated by PPIs and patients with autoimmune connective tissue diseases developing DI-SCLE after PPI use have been reported.3,4 Here, we present the cases of 3 patients with previous diagnoses of SLE without SCLE skin manifestations developing DI-SCLE after using PPIs or antihypertensives. This pattern has rarely been described in the literature to date and suggests a phenomenon of which clinicians should be aware.

Four major medical societies involved with hydroxychloroquine (HCQ) therapy concur on the need for common principles and cooperation to minimize the risk of ocular toxicity. At a daily dosage of ≤5 mg/kg/day actual body weight, the risk of retinal toxicity from HCQ is

The use of herbal supplements that promise to improve immune health has gained popularity among dermatology patients. However, there is little to no evidence that herbal supplements improve dermatological conditions. Several in vitro and in vivo studes have shown that Spirulina platensis, Aphanizomenon flos-aqua, Chlorella, Echinacea, and alfalfa activate immune cells, via certain cytokines and chemokines. Case reports suggest the association of ingesting immunostimulatory herbs and the clinical onset or flares of diseases characterized by an exaggerated immune response such as lupus erythematosus, dermatomyositis and autoimmune blistering disorders. Therefore, it is imperative to investigate the prevalence of herbal supplement use in this patient population. In addition, in vitro studies should examine the underlying mechanisms by which herbs stimulate immune pathways that are already overactive in autoimmune patients. CAPSULE SUMMARY: How does this article integrate into what was already known? Spirulina, Aphanizomenon flos-aqua, Chlorella, Echinacea and alfalfa have been shown to stimulate the immune system. The use of these herbs may be associated with the precipitation or flare of autoimmune skin diseases. How does it change practice? Patients with autoimmune diseases should be screened for supplement use and should be advised to avoid these immunostimulatory herbs.

ImportanceMorphea is an insidious inflammatory disorder of the skin and deeper tissues. Determining disease activity is challenging yet important to medical decision-making and patient outcomes.ObjectiveTo develop and validate a scoring tool, the Morphea Activity Measure (MAM), to evaluate morphea disease activity of any type or severity that is easy to use in clinical and research settings.Design, Setting, and ParticipantsThis pilot diagnostic study was conducted from September 9, 2019, to March 6, 2020, in 2 phases: development and validation. During the development phase, 14 morphea experts (dermatologists and pediatric dermatologists) used a Delphi consensus method to determine items that would be included in the MAM. The validation phase included 8 investigators who evaluated the tool in collaboration with 14 patients with pediatric morphea (recruited from a referral center [Medical College of Wisconsin]) during a 1-day in-person meeting on March 6, 2020.Main Outcomes and MeasuresDuring the development phase, online survey items were evaluated by experts in morphea using a Likert scale (score range, 0-10, with 0 indicating not important and 10 indicating very important); agreement was defined as a median score of 7.0 or higher, disagreement as a median score of 3.9 or lower, and no consensus as a median score of 4.0 to 6.9. During the validation phase, reliability (interrater and intrarater agreement using intraclass correlation coefficients), validity (using the content validity index and κ statistics as well as correlations with the modified Localized Scleroderma Severity Index and the Physician Global Assessment of Activity using Spearman ρ coefficients), and viability (using qualitative interviews of investigators who used the MAM tool) were evaluated. Descriptive statistics were used for quantitative variables. Data on race and ethnicity categories were collected but not analyzed because skin color was more relevant for the purposes of this study.ResultsAmong 14 survey respondents during the development phase, 9 (64.3%) were pediatric dermatologists and 5 (35.7%) were dermatologists. After 2 rounds, a final tool was developed comprising 10 items that experts agreed were indicative of morphea activity (new lesion in the past 3 months, enlarging lesion in the past 3 months, linear lesion developing progressive atrophy in the past 3 months, erythema, violaceous rim or color, warmth to the touch, induration, white-yellow or waxy appearance, shiny white wrinkling, and body surface area). The validation phase was conducted with 14 patients (median age, 14.5 years [range, 8.0-18.0 years]; 8 [57.1%] female), 2 dermatologists, and 6 pediatric dermatologists. Interrater and intrarater agreement for MAM total scores was good, with intraclass correlation coefficients of 0.844 (95% CI, 0.681-0.942) for interrater agreement and 0.856 (95% CI, 0.791-0.901) for intrarater agreement. Correlations between the MAM and the modified Localized Scleroderma Severity Index (Spearman ρ = 0.747; P P Conclusions and RelevanceIn this study, the MAM was found to be a reliable, valid, and viable tool to measure pediatric morphea activity. Further testing to assess validity in adults and responsiveness to change is needed.

In this study, we evaluated Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) scores calculated from photos taken by trained clinic staff. We found excellent reliability between photographs and an in-person scoring of the CDASI activity portion in patients with dermatomyositis although the CDASI may be underestimated from photographs. More research is needed to determine if photographs taken outside the office setting may be used to assess clinical trials outcomes.

Background Dermatomyositis (DM) is conventionally characterized by interface dermatitis (ID) on skin histopathology. A subset of DM patients have skin biopsies showing spongiotic dermatitis (SD), a histopathology more commonly seen in eczema. In this study we aimed to 1) identify the percentage of clinically diagnosed DM patients with SD skin biopsies, 2) identify cytokine and cell markers that can help determine if a SD skin biopsy is consistent with DM. Methods In this case-control study, biopsies from ten DM patients with SD (DM-SD) were compared to biopsies from ten healthy controls, ten patients with eczema, and 12 patients with DM with ID (DM-ID). Biopsies were stained by immunohistochemistry for MxA, IFN-β, CD11c, and BDCA2. One-way ANOVA with Bonferroni's multiple comparison test was used to compare protein expression between groups. Results 11 of 164 (6.7%) patients with a clinical diagnosis of DM at our tertiary care center were identified as having SD. MxA, IFN-β, CD11c, and BDCA2 protein expression is significantly higher in DM-SD compared to eczema and healthy controls. Expressions of MxA, IFN-β and BDCA2 were not significantly different between DM-SD and DM-ID. Conclusion Increased MxA, IFN-β, CD11c, and BDCA2 protein expression may aid in distinguishing between DM-SD and eczema and warrants further investigation. This article is protected by copyright. All rights reserved.

Photosensitivity is a common symptom in the autoimmune diseases lupus erythematosus (LE) and dermatomyositis (DM). This review provides a framework for dermatologists, rheumatologists, and general practitioners to aid in recognizing and understanding photosensitivity reactions within the scope of these conditions. Photosensitivity reactions encompass not only cutaneous symptoms but also include systemic manifestations. Prevalence of photosensitivity is high in both LE and DM and tends to vary based on race and geographical location. Pathophysiology implicates the various effects of ultraviolet (UV) light on immunoreactants in the skin. Photoprotection remains the leading method of prevention of photosensitive response. Photosensitivity heavily impacts quality of life (QoL) in patients with LE and DM. By accurately recognizing these reactions, physicians can better treat these autoimmune conditions.

ObjectiveThere is a need to identify concerns unique to patients with cutaneous lupus erythematosus (CLE), which may not be captured by current common-practice dermatological quality-of-life tools. This study formally characterises what bothers patients with CLE about their disease by conducting semistructured, qualitative interviews.MethodsSixteen patients with CLE were interviewed about how their cutaneous findings impact their daily life. Each interview was transcribed, coded and categorised for recurrent themes. Current CLE activity and damage were also assessed by the Cutaneous Lupus Activity and Severity Index tool.ResultsResponses were categorised into six themes, including Fear of Disease Progression, Unwanted Attention, Self-Consciousness, Physical Signs/Symptoms, Emotional Symptoms and Functional Decline. The most commonly reported themes were Self-Consciousness, mentioned by 13 of 16 (81.3%) patients, Physical Symptoms, mentioned by 12 of 16 (75%), and then Fear of Disease Progression, by 11 of 16 (68.8%). Frequently mentioned physical signs/symptoms included erythema, itch, dyspigmentation, scar and alopecia. The physical signs/symptoms were categorised as activity signs/symptoms, damage signs and other. For activity signs, erythema was mentioned most frequently (5 of 16), then scale (2 of 16). For activity symptoms, itch was mentioned most frequently (6 of 16), then pain (5 of 16). For damage signs, dyspigmentation was mentioned most frequently (4 of 16), followed by scarring (3 of 16). Patients less than 60 years old were more likely to report emotional symptoms than older patients (pConclusionsThese patient experiences and resultant themes elucidated by this study are worth noting in future standardised estimations of the quality of life of patients with CLE. Additionally, the concerns shown by these interviews are important topics for providers to discuss when evaluating patient disease progression.

Treatment options are limited for skin disease in dermatomyositis. Lenabasum is a cannabinoid receptor type 2 agonist that triggers the resolution of inflammation.The objective of this study was to evaluate the safety and efficacy of lenabasum in patients with refractory cutaneous dermatomyositis.This study was a single-center, double-blind, randomized, placebo-controlled phase 2 study conducted from July 2015 to August 2017.The population included subjects aged ≥18 years with at least moderately active dermatomyositis skin activity by Cutaneous Dermatomyositis Disease Area and Severity Index activity ≥ 14 and failure or intolerance to hydroxychloroquine.Participants received 20 mg lenabasum daily for 28 days and then 20 mg twice per day for 56 days or placebo.The primary outcome was a change in Cutaneous Dermatomyositis Disease Area and Severity Index activity. Safety and other secondary efficacy assessments were performed till day 113.A total of 22 subjects were randomized to lenabasum (n = 11) or placebo (n = 11). No serious or severe adverse events were related to lenabasum, and no participants discontinued the study. The adjusted least-squares mean for Cutaneous Dermatomyositis Disease Area and Severity Index activity decreased more for lenabasum, and the difference was significant on day 113 (least-squares mean [standard error] difference = ‒6.5 [3.1], P = 0.038). Numerically greater improvements were seen in multiple secondary efficacy outcomes and biomarkers with lenabasum.Lenabasum treatment was well tolerated and was associated with greater improvement in Cutaneous Dermatomyositis Disease Area and Severity Index activity and multiple efficacy outcomes.This study was registered at ClinicalTrials.gov, NCT02466243.

Clinical question What are the roles of antimalarial therapy prescribers and eye care specialists in regard to antimalarial dosing, screening for retinal toxic effects, and antimalarial treatment cessation? Bottom line Antimalarial prescribers should prescribe antimalarial dosages at 5 mg/kg/d or less of actual body weight. A baseline retinal examination with optical coherence tomography should be performed within 6 months of starting antimalarial therapy to rule out confounding disease. Patients at low risk for retinopathy do not require annual screening until 5 years of antimalarial use. Eye care clinicians should not stop treatment with antimalarials because of equivocal findings, as retinopathy occurs slowly, and therefore there is time for repeated testing. Antimalarial treatment cessation should be a collaborative decision that involves the patient, the prescriber, and the eye care clinician and that focuses on patient values, the severity of the underlying disease, and the estimated risk of visual loss if treatment with the drug is continued.

It is common for multiple autoimmune diseases to occur in the same patient. However, autoimmune blistering diseases (AIBD) do not commonly associate with dermatomyositis (DM). We performed a literature review and found 12 previous reports that may be attributed to misdiagnosis, underreporting, or true rarity of association. Herein, we present a case of pemphigus vulgaris and a case of mucous membrane pemphigoid associated with DM and review the related literature. AIBD-associated interstitial lung disease, genetic predisposition, potential environmental triggers of both AIBD and DM, drug-related triggers, and paraneoplastic processes are discussed. Dermatologists must be vigilant for a second autoimmune disease in patients with AIBD that may have therapeutic implications. Keywords: Dermatomyositis, interstitial lung disease, pemphigus, pemphigoid, autoimmune, blistering

Background: Lack of established criteria for sunscreen product recommendations and potentially allergenic ingredients in sunscreens pose an issue for physicians and patients with autoimmune skin conditions. Objective: We reviewed popular sunscreens for effectiveness and potential allergenicity for recommendation and use in the autoimmune skin condition population. Methods: In this cohort study, we selected sunscreens from the bestseller lists of Amazon, Target, and CVS. Of those, sunscreens with sun protection factor of 50 to 99 and 100 that met our effectiveness criteria (52 sunscreens) were analyzed for allergenic ingredients. An allergen list was developed from the North American Contact Dermatitis Group core data and stratified into low-prevalence and high-prevalence allergens. Results: The allergenicity of popular sunscreens that met our effectiveness criteria are organized in a table by number of tiered potential allergens. Although no sunscreen was allergen-free, several products contained a minimal number of low-prevalence allergens. The most common low-prevalence allergens were chemical sunscreen ingredients avobenzone, octocrylene, and oxybenzone, and the most common high-prevalence allergen was fragrance. A limitation is that not all U.S. sunscreens were analyzed. Conclusion: With the wide range of sunscreens available, physicians and patients should be aware of the effectiveness and potential allergenicity of sunscreens and make recommendations and consumption choices accordingly. Keywords: dermatology, sunscreen, photosensitivity, allergens, contact dermatitis, autoimmune skin

Objective Pruritus is an important symptom frequently accompanying various inflammatory skin conditions and some recent data indicated that it may be associated with autoimmune connective tissue diseases. The aim of this study was to assess the frequency and clinical presentation of itch in CLE. Methods A multinational, prospective, cross-sectional study was performed to assess the prevalence, intensity and clinical characteristic of pruritus in various subtypes of CLE. A total of 153 patients with active CLE lesions were included. Their age ranged between 17 and 82 years (mean 49.8 ± 15.4 years), and 115 patients (75.2%) were women. The disease activity and damage were assessed according to the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Pruritus severity was assessed with Numeric Rating Scale (NRS) and the 12-Item Pruritus Severity Scale. Dermatology Life Quality Index and EQ-5D questionnaire were used to measure quality of life. Results Pruritus was present in 116 (76.8%) of patients of whom half had NRS scoring equal or above 4 points indicating moderate or severe pruritus. Most commonly itch was localized on the scalp, face (excluding ears and nose) and arms (40.5%, 36.2%, 31.9%, respectively). Sensations connected with pruritus were most frequently described as burning, tingling and like ants crawling feeling, but 31.9% patients described it as “pure itch”. More than half of patients reported that pruritus was present every day, and it was most frequent during the evenings. The pruritus scoring and the CLASI activity score were significantly correlated (r = 0.42, p = 0.0001), while no correlation was found with the CLASI damage score (p = 0.16). Both the maximum and average itch intensity were correlated with systemic lupus erythematosus (SLE) activity measured with the Systemic Lupus Erythematosus Disease Activity Index. Conclusions Pruritus is a common, but frequently overlooked symptom of CLE. Its intensity correlates with the activity of CLE, but not with the skin damage. In more than a half of patients it occurs on a daily basis. The correlation between the intensity of pruritus and the activity of the skin lesions and the systemic involvement indicate that pruritus could be an individual indicator of both SLE and CLE activity.

Dermatomyositis pathogenesis remains incompletely understood; however, recent work suggests a predominant IFN-1 response. We explored dermatomyositis pathogenesis by quantifying the inflammatory cells in the skin, comparing myeloid with plasmacytoid dendritic cell release of IFN-β, and assessing myeloid dendritic cell (mDC) contribution to hydroxychloroquine refractoriness. Immunohistochemistry was performed to assess cell-type expression in lesional skin biopsies from 12 patients with moderate-to-severe cutaneous dermatomyositis. Immunofluorescence, laser-capture microdissection, and flow cytometry were used to assess mDC release of IFN-β in lesional skin biopsies and blood of patients with dermatomyositis. Immunohistochemistry was utilized to determine whether myeloid or plasmacytoid dendritic cells were increased in hydroxychloroquine nonresponders. CD4+, CD11c+, and CD69+ cells were more populous in lesional skin of patients with dermatomyositis. mDCs colocalized with IFN-β by immunofluorescence and laser-capture microdissection revealed increased IFN-β mRNA expression by mDCs in lesional skin of patients with dermatomyositis. In blood, both mDCs and plasmacytoid dendritic cells were major producers of IFN-β in patients with dermatomyositis, whereas plasmacytoid dendritic cells predominately released IFN-β in healthy controls (P0.01). mDCs were significantly increased in the skin of hydroxychloroquine nonresponders compared with that in the skin of responders (P0.05). mDCs cells appear to play an important role in dermatomyositis pathogenesis and IFN-β production.

The Dermatomyositis Delphi Criteria Project created 25 provisional clinical, laboratory and contextual classification criteria after an extensive literature search, three rounds of consensus exercises and nominal group discussions. These criteria will be subjected to a case-control validation study to create a combination of items that will define a more inclusive cohort of DM patients with skin-predominant disease for clinical research. Several measurement properties that need to be assessed prior to the conduct of a multicenter prospective validation study include the final purpose of the criteria set, population and disease characteristics of cases and controls for study entry, sources of samples, definition of criteria items, methods of item ranking and reduction, and consideration of criteria set validity against a comparator backdrop “gold standard” criteria. An expert committee Delphi and several online discussions among participants from the fields of dermatology, and adult and pediatric rheumatology have been conducted in order to address such issues. Constant evaluation of the methodologic process is vital to produce classification criteria which are valid and reliable to identify patients with DM and delineate these patients from those with mimicker diseases.

Cutaneous lupus erythematosus (CLE) can present with or without features of systemic lupus erythematosus (SLE), with estimates of the incidence of isolated skin disease almost equaling the incidence of those with systemic disease. However, despite the impact CLE has on a patient's quality of life (QoL), there has been no US Food and Drug Administration (FDA) approved treatment for the disease in the past 50 years. In addition, patients with skin predominant LE are often excluded from clinical SLE trials. In the rare trials that include patients with skin predominant LE, disease activity and progression in the skin are often difficult to evaluate using multi-organ outcome measures. The need for new therapies for CLE and the lack of focus on skin outcomes has led to the development of the Cutaneous Lupus Disease Area and Severity Index (CLASI), a validated organ-specific outcome measure that is not only responsive to change in disease activity and damage but also correlated to changes in a patient's QoL. This paper will emphasize the extensive validation studies performed in developing the CLASI, as well as the importance of clinical trials using the CLASI to address the need for improved therapies for patients with lupus skin manifestations.

Cutaneous Lupus Erythematosus (CLE) and Dermatomyositis (DM) are cutaneous autoimmune diseases that have been among the least systematically studied, due in part to the lack of validated outcome instruments in the past. More recent epidemiologic studies have elucidated the incidence and prevalence of these diseases and their subtypes. In addition, the advent of validated clinical outcome measures, including the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), has led to an objective means of measuring activity and damage of the disease. These outcome measures have established the framework for evaluating responsiveness and therapeutic efficacy in clinical trials as well as longitudinal studies to study disease course.

A 57-year-old African American woman presented to dermatology following a two-year history of a pruritic rash initially involving the face which later progressed to her forearms, back, ears, dorsal hands, and abdomen. Physical exam revealed erythematous papules overlying the metacarpal and interphalangeal joints, periungual telangiectasias, periorbital edema, violaceous erythema of the upper eyelids as well as areas of dyspigmentation (A). There were 1-2 mm skin-colored hyperkeratotic follicular papules on the extensor arms, upper back, and abdomen, and hyperpigmented follicular plugging on the chest (B).

Cutaneous lupus erythematosus (CLE) is a disfiguring and potentially disabling disease that causes significant morbidity in patients. Antimalarials are an important class of medication used to treat this disease and have been the first-line systemic therapy since the 1950s. Quinacrine, in particular, is used as an adjuvant therapy to other antimalarials for improved control of CLE. Quinacrine is currently unavailable in the USA, which has taken away an important component of the treatment regimen of patients with CLE. This paper reviews the evidence of available local and systemic therapies in order to assist providers in choosing alternative treatments for patients who previously benefited from quinacrine therapy.

BACKGROUND: Multiple strategies have been used to evaluate minimal important change (MIC) of Eczema Area and Severity Index (EASI) and Scoring Atopic Dermatitis (SCORAD). The meaningfulness of these MIC is not well established across all AD severities. OBJECTIVES: Determine the MIC of % and absolute improvement of EASI and SCORAD scores in adults and children with AD. METHODS: We performed a prospective dermatology practice-based study using questionnaires and evaluation by a dermatologist (n=826). An anchor-based approach was used to determine thresholds for % and absolute MIC of EASI, SCORAD and O-SCORAD at follow-up from baseline. RESULTS: One-grade improvements of Physician’s Global Assessment (PGA) and Validated Investigator’s Global Assessment of AD (vIGA-AD) were associated with 50%, 35%, and 35% decreases of EASI, SCORAD and objective-SCORAD, respectively. The thresholds for % MIC of EASI (Kruskal-Wallis test, P=0.61), SCORAD (P=0.07) and O-SCORAD (P=0.09) were similar across baseline AD severity. One-grade improvements of PGA and vIGA-AD were associated with 14.0 and 14.9-point decreases of EASI; 19.9 and 14.9-point decreases of SCORAD; 15.5 and 17.4-point decreases of O-SCORAD. The thresholds for absolute MIC of EASI (P