Successful Treatment of Keloids and Hypertrophic Scars With Systemic and Intralesional Dupilumab.

Keloids and hypertrophic scars negatively impact the quality of life for millions of people in the world. Unfortunately, though many thera-peutic approaches are used to treat scars, they are often limited in efficacy with high rates of recurrence. Lately, a better understanding of the immune dysregulation of several dermatologic conditions has led to the emergence of multiple cytokine-targeted therapies for numerous conditions. Several studies have implicated T helper 2 (Th2) immune dysregulation in the development of scars and keloids, with interleukins (IL)-4 and -13 identified as pro-fibrotic mediators. Dupilumab is an IL-4 receptor alpha antagonist that inhibits the ex-pression of both IL-4 and -13. Herein, we describe a 44-year-old woman who developed numerous disfiguring hypertrophic scars and keloids after suffering from a severe herpes zoster infection. Given the number of scars, intralesional corticosteroid injections were not feasible. Therefore, treatment with systemic dupilumab was initiated. Many scars flattened, several even developing a cigarette-paper-like texture due to rapid involution. The largest and most recalcitrant keloid was further treated with intralesional dupliumab injec-tions every 2 weeks with an even more dramatic improvement noted in 2 months. To our knowledge, this is the first report of treating multiple keloids and hypertrophic scars with both systemic and intralesional dupilumab. Dermatologists may want to consider treating keloids that cover a large area with systemic dupilumab, a therapy with an established, reassuring safety profile. The most recalcitrant areas may further benefit from concentrating dupilumab by intralesional delivery. J Drugs Dermatol. 2023;22(12):1220-1222.  doi:10.36849/JDD.6385.

International Guideline for Idiopathic Inflammatory Myopathy-Associated Cancer Screening: an International Myositis Assessment and Clinical Studies Group (IMACS) initiative.

Adult-onset idiopathic inflammatory myopathy (IIM) is associated with an increased cancer risk within the 3 years preceding and following IIM onset. Evidence- and consensus-based recommendations for IIM-associated cancer screening can potentially improve outcomes. This International Guideline for IIM-Associated Cancer Screening provides recommendations addressing IIM-associated cancer risk stratification, cancer screening modalities and screening frequency. The international Expert Group formed a total of 18 recommendations via a modified Delphi approach using a series of online surveys. First, the recommendations enable an individual patient's IIM-associated cancer risk to be stratified into standard, moderate or high risk according to the IIM subtype, autoantibody status and clinical features. Second, the recommendations outline a 'basic' screening panel (including chest radiography and preliminary laboratory tests) and an 'enhanced' screening panel (including CT and tumour markers). Third, the recommendations advise on the timing and frequency of screening via basic and enhanced panels, according to risk status. The recommendations also advise consideration of upper or lower gastrointestinal endoscopy, nasoendoscopy and 18F-FDG PET-CT scanning in specific patient populations. These recommendations are aimed at facilitating earlier IIM-associated cancer detection, especially in those who are at a high risk, thus potentially improving outcomes, including survival.

A systematic review and meta-analysis to inform cancer screening guidelines in idiopathic inflammatory myopathies.

OBJECTIVES: To identify clinical factors associated with cancer risk in the idiopathic inflammatory myopathies (IIMs) and to systematically review the existing evidence related to cancer screening. METHODS: A systematic literature search was carried out on Medline, Embase and Scopus. Cancer risk within the IIM population (i.e. not compared with the general population) was expressed as risk ratios (RR) for binary variables and weighted mean differences (WMD) for continuous variables. Evidence relating to cancer screening practices in the IIMs were synthesized via narrative review. RESULTS: Sixty-nine studies were included in the meta-analysis. DM subtype (RR 2.21), older age (WMD 11.19), male sex (RR 1.53), dysphagia (RR 2.09), cutaneous ulceration (RR 2.73) and anti-transcriptional intermediary factor-1 gamma positivity (RR 4.66) were identified as being associated with significantly increased risk of cancer. PM (RR 0.49) and clinically amyopathic DM (RR 0.44) subtypes, Raynaud's phenomenon (RR 0.61), interstitial lung disease (RR 0.49), very high serum creatine kinase (WMD -1189.96) or lactate dehydrogenase (WMD -336.52) levels, and anti-Jo1 (RR 0.45) or anti-EJ (RR 0.17) positivity were identified as being associated with significantly reduced risk of cancer. Nine studies relating to IIM-specific cancer screening were included. CT scanning of the thorax, abdomen and pelvis appeared to be effective in identifying underlying asymptomatic cancers. CONCLUSION: Cancer risk factors should be evaluated in patients with IIM for risk stratification. Screening evidence is limited but CT scanning could be useful. Prospective studies and consensus guidelines are needed to establish cancer screening strategies in IIM patients.

Dermatomyositis: An Update on Diagnosis and Treatment.

Dermatomyositis is a rare inflammatory disease with characteristic cutaneous findings and varying amounts of systemic involvement. Patients may present with skin disease alone, have concomitant muscle disease, or have extracutaneous manifestations such as pulmonary disease or an associated malignancy. Given such diverse presentations, dermatomyositis is both a diagnostic and therapeutic challenge. However, a prompt diagnosis is of utmost importance to institute adequate therapy and screen patients for an associated malignancy. Dermatologists should play a crucial role in the diagnosis and management of patients with dermatomyositis as cutaneous disease tends to be chronic, negatively impact quality of life, and be more recalcitrant to therapy. In this review, we discuss diagnosis, with a focus on myositis-specific antibodies and their associated phenotypes. We also review therapies available for this often refractory skin disease.

A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria.

Importance: Necrobiotic xanthogranuloma (NXG) is a non-Langerhans cell histiocytosis classically associated with paraproteinemia attributable to plasma-cell dyscrasias or lymphoproliferative disorders. Despite the morbidity of NXG, the literature is limited to case reports and small studies, and diagnostic criteria are lacking. Objective: To evaluate the characteristics of NXG and propose diagnostic criteria. Design, Setting, and Participants: This multicenter cross-sectional study was conducted at tertiary academic referral centers and followed by a systematic review and a consensus exercise. The multicenter cohort included patients with NXG diagnosed at the Brigham and Women's and Massachusetts General Hospitals (2000-2018), the University of Iowa Hospitals and Clinics (2000-2018), and the University of Pennsylvania Health System (2008-2018). The systematic review was conducted in 2018 and included patients with NXG identified in the Cochrane, Ovid EMBASE, PubMed, and Web of Science databases. The consensus exercise was conducted by 8 board-certified dermatologists to identify diagnostic criteria. Main Outcomes and Measures: Demographic factors, comorbidities, clinical features, and treatment response. Results: Of 235 included patients with NXG (34 from the multicenter cohort and 201 from the systematic review results), the mean (SD) age at presentation was 61.6 (14.2) years; 147 (62.6%) were female. Paraproteinemia was detected in 193 patients (82.1%), most often IgG-κ (117 patients [50.0%]). A malignant condition was detected in 59 patients (25.1%), most often multiple myeloma (33 patients [14.0%]). The overall rate of paraproteinemia and/or a malignant condition was 83.8% (197 patients). In the multicenter cohort, evolution of paraproteinemia into multiple myeloma was observed up to 5.7 years (median [range], 2.4 [0.1-5.7] years) after NXG presentation. Cutaneous lesions consisted of papules, plaques, and/or nodules, typically yellow or orange in color (113 of 187 [60.4%]) with a periorbital distribution (130 of 219 [59.3%]). The eye was the leading site of extracutaneous involvement (34 of 235 [14.5%]). In the multicenter cohort, intravenous immunoglobulin had the best treatment response rate (9 of 9 patients [100%]), followed by antimalarial drugs (4 of 5 patients [80%]), intralesional triamcinolone (6 of 8 patients [75%]), surgery (3 of 4 patients [75%]), chemotherapy (8 of 12 patients [67%]), and lenalidomide or thalidomide (5 of 8 patients [63%]). The consensus exercise yielded 2 major criteria, which were (1) clinical and (2) histopathological features consistent with NXG, and 2 minor criteria, consisting of (1) paraproteinemia, plasma-cell dyscrasia, and/or other associated lymphoproliferative disorder and (2) periorbital distribution of cutaneous lesions. In the absence of foreign body, infection, or another identifiable cause, fulfillment of both major and at least 1 minor criterion were proposed to establish the diagnosis of NXG. Conclusions and Relevance: Necrobiotic xanthogranuloma is a multisystem disorder associated with paraproteinemia and malignant conditions. The proposed diagnostic criteria may advance clinical research and should be validated.

Augmented reality in dermatology: Are we ready for AR?

Augmented reality (AR) refers to a group of technologies that capture, analyze, and superimpose digital information onto the real world. This information gives health care providers unique and useful perspectives that can enhance patient care. AR has been utilized in selected scenarios in health care for several decades, notably laparoscopic surgery and vein finding. In recent years, improved wireless technologies, computing power, and analytics are leading to rapid growth in the AR industry. Novel health care-specific use cases are rapidly being introduced with the potential to widely affect clinical care, particularly in dermatology because of the visual nature of the field. In this article, we define AR, profile clinical and educational uses of AR in dermatology, and discuss key policy considerations for the safe and appropriate use of this emerging technology.

Clinical Features and Comorbidities of Patients With Necrobiosis Lipoidica With or Without Diabetes.

Importance: Necrobiosis lipoidica (NL) is a rare granulomatous condition. Current knowledge of its key features is based on a limited number of studies and case reports, leading to wide variability in the characterization of its defining features, with limited comparison of patients with or without diabetes. Objective: To evaluate the epidemiologic characteristics, clinical features, and disease associations of NL in patients with or without type 1 or 2 diabetes. Design, Setting, and Participants: This multicenter retrospective review included 236 patients aged 15 to 84 years who were evaluated and received a diagnosis of NL at the University of Pennsylvania Health System between January 1, 2008, and July 15, 2018; University of Iowa Hospitals and Clinics between January 1, 2000, and June 15, 2018; and Brigham and Women's Hospital and Massachusetts General Hospital between January 1, 2000, and February 15, 2018. Main Outcomes and Measures: Patient demographics, clinical features, medical comorbidities, and biopsy status. Results: Of the 236 patients with NL, 200 were women and 36 were men, and 182 were white, with a median age at presentation of 50.0 years (interquartile range, 33.0-59.0 years). The diagnosis was biopsy proven in 156 patients (66.1%). Of the 230 patients with location specified, 225 (97.8%) had NL on the lower legs. A total of 138 patients with NL (58.5%; 95% CI, 52.7%-65.3%) had diabetes. The median hemoglobin A1c for patients with diabetes was 8.00% (interquartile range, 6.68%-9.50%) (to convert hemoglobin A1c to proportion of total hemoglobin, multiply by 0.01). Patients with diabetes were significantly younger than patients without diabetes (median age, 45.0 vs 52.0 years; P = .005), and slightly less likely to be female (112 of 138 [81.2%] vs 87 of 96 [90.6%]; P = .046), but lesion characteristics were otherwise comparable. Other notable comorbidities included obesity in 95 of 184 patients (51.6%; 95% CI, 44.4%-58.9%), hypertension in 104 of 230 patients (45.2%), dyslipidemia in 98 of 225 patients (43.6%), and thyroid disease in 56 of 229 patients (24.5%). Conclusions and Relevance: This study of NL supports its associations with diabetes as well as obesity, hypertension, dyslipidemia, and thyroid disease. Younger age and female sex were observed more frequently in patients with diabetes. Otherwise, NL lesions in patients with or without diabetes shared many clinical features, suggesting that risk factors outside of elevated blood glucose may play an important role in the disease. Future studies should evaluate these associations with the goal of further elucidating NL's underlying pathophysiologic characteristics.

Anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis: A concise review with an emphasis on distinctive clinical features.

Melanoma differentiation-associated gene 5 (MDA5) is a recently described autoantigen target in a subset of patients with dermatomyositis. Anti-MDA5 dermatomyositis is characterized by a unique mucocutaneous and systemic phenotype that includes cutaneous and oral ulceration, painful palmar papules, alopecia, panniculitis, arthritis, a lower incidence of myositis, and, importantly, an elevated risk of interstitial lung disease with a potentially fatal course. Because the clinical features can differ substantially from those typically observed in cutaneous dermatomyositis, the diagnosis is often overlooked, which might negatively affect patient outcomes. This review aims to familiarize the clinician with the distinctive clinical features of anti-MDA5 dermatomyositis in order to enhance its recognition and to facilitate an appropriate screening and management strategy.