An Isolated Cutaneous Nodule: Dupilumab-Induced Sarcoid-Like Reaction.

Dupilumab has recently been recognized as a potential trigger for drug-induced sarcoid-like reactions (DISR). This phenomenon may become more prevalent with increased utilization of this drug for a multitude of skin and atopic conditions. We present a unique case of a patient developing a solitary cutaneous nodule on her left forearm following dupilumab initiation. Histopathology and MRI studies confirmed that this nodule had features of a sarcoid granuloma. Six months following dupilumab discontinuation, the patient's granuloma resolved. This case demonstrates that dupilumab can induce cutaneous-limited autoimmune disease and stresses the importance of prompt recognition of dupilumab-induced sarcoid-like reactions for appropriate diagnosis and treatment.

Approach to the Atypical Wound.

The heterogeneity of atypical wounds can present diagnostic and therapeutic challenges; however, as the prevalence of atypical wounds grows worldwide, prompt and accurate management is increasingly an essential skill for dermatologists. Addressing the underlying cause of an atypical wound is critical for successful outcomes. An integrated approach with a focus on pain management and patient engagement is recommended to facilitate enduring wound closure. Advances in treatment, in addition to further research and clinical training, are necessary to address the expanding burden of atypical wounds.

Inflammatory and vaso-occlusive ulcers: Part I - clinical presentation and diagnosis.

In this CME, we review two specific categories of ulcers: inflammatory (where inflammation is the primary pathologic process leading to ulceration) and vaso-occlusive (where occlusion is the primary process). Inflammatory ulcers include pyoderma gangrenosum and vasculitides, whereas livedoid vasculopathy, calciphylaxis and Martorell ulcers are vaso-occlusive ulcers. Determining the causes of ulcers in these conditions may require laboratory evaluation, biopsy and imaging.

Inflammatory and vaso-occlusive ulcers: Part II - Management.

In the second part of this CME, we present an approach for the management of inflammatory and vaso-occlusive ulcers and highlight the need for further research in this field. The three overarching principles for management are etiology-specific treatment, ulcer care, and consideration of patient comorbidities and risk factors for poor healing. Both etiology-specific treatment and management of patient comorbidities and risk factors often require collaboration with providers from other specialties. Ulcer care is governed by TIME, or tissue debridement, infection control, management of moisture imbalance and epithelial edge advancement. As wound healing is a dynamic process, management should be adapted to changes in the status of the ulcer.

Recent Advances in Treatment of Systemic Sclerosis and Morphea.

Systemic sclerosis (SSc) and morphea are autoimmune sclerosing diseases that cause significant morbidity, and in the case of SSc, mortality. The pathogenesis of both SSc and morphea share vascular dysfunction, auto-reactive T cells and Th2-associated cytokines, such as interleukin 4, and overproduction of transforming growth factor beta (TGFß). TGFß stimulates fibroblast collagen and extra-cellular matrix production. Although morphea and SSc have similar pathogenic pathways and histological findings, they are distinct diseases. Recent advances in treatment of morphea, skin sclerosis in SSc, and interstitial lung disease in SSc are focused on targeting known pathogenic pathways.

Eosinophilic Annular Erythema Responding to Doxycycline.

Eosinophilic annular erythema (EAE) is a rare skin disease characterized by relapsing and remitting pruritic, annular erythematous plaques and tissue eosinophilia. A 39-year-old male presented with a mildly pruritic, relapsing, and remitting urticarial rash. A biopsy revealed superficial and deep perivascular dermatitis with numerous eosinophils and some neutrophils, with an absence of flame figures. Based on clinical and histopathologic findings, the patient was given a diagnosis of eosinophilic annular erythema. Treatment was initiated with doxycycline 100 mg twice daily. The patient reported substantial improvement at three months and sustained clearance at one year, remaining on doxycycline well tolerated throughout. To our knowledge, no cases of EAE improving with doxycycline have been reported in the literature and, thus, our findings highlight a potential new therapy to consider in a patient with EAE.

A national survey comparing practice patterns and residency training satisfaction for categorical dermatology versus combined internal medicine and dermatology trained physicians.

Combined internal medicine and dermatology (med-derm) training programs were created to advance complex medical dermatology and inpatient dermatology care. A prior study demonstrated that compared to categorical dermatology residents, med-derm residents had less program satisfaction, yet indicated a stronger desire to pursue careers in academia. No follow-up data on practice patterns after training has been reported. We aimed to characterize differences in residency program satisfaction and practice patterns between physicians trained in categorical dermatology compared to med-derm residency programs. We surveyed physicians who graduated from combined med-derm programs along with their counterparts, from six institutions, that either currently or historically had a combined med-derm training, from 2008-2017. Fifty-five percent of med-derm and forty-one percent of categorical-trained physicians responded. The practice patterns between the two groups were similar. A quarter of med-derm physicians continued to provide general internal medicine services. Categorical trained physicians were significantly more satisfied with their training (P=0.03) and performed more excisions on the head/neck (P=0.02). The combined graduates had significantly greater confidence in multidisciplinary care (P=0.003), prescribed more biologic (P<0.001) and non-biologic immunosuppressive agents (P=0.002), and volunteered more for the underserved patients in their communities (P=0.04). Although few differences in overall practice patterns between categorical and med-derm trained graduates were appreciated, med-derm graduates seem more comfortable with multidisciplinary care and may care for more medically complex patients requiring immunosuppression.

Treatment-Resistant Edematous Annular Plaques and Mild Leukopenia in a Man in His 60s.

A man in his 60s presented with a 5-year history of diffuse erythematous, edematous annular plaques, low-grade fevers, and mild leukopenia. What is your diagnosis?

Development and Validation of the Morphea Activity Measure in Patients With Pediatric Morphea.

Importance: Morphea 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. Objective: To 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 Participants: This 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 Measures: During 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. Results: Among 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 < .001) and the MAM and the Physician Global Assessment of Activity (Spearman ρ = 0.729; P < .001) were moderately strong. In qualitative interviews, evaluators agreed that the tool was easy to use, measured morphea disease activity at a single time point, and should be responsive to changes in morphea disease activity over multiple time points. Conclusions and Relevance: In 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.

Monkeypox outbreak, vaccination, and treatment implications for the dermatologic patient: Review and interim guidance from the Medical Dermatology Society.

Rapid human-to-human transmission of monkeypox has created a public health emergency requiring prompt, multidisciplinary attention. Dermatologists are at the forefront of diagnosis due to the disease-defining skin lesions. Moreover, patients with pre-existing skin disease and those who are on immunosuppressive medications for skin disease may be at increased risk of severe infection. In this review, a panel of authors with expertise in complex medical dermatology and managing patients on immunosuppression reviews the literature and provides initial guidance for diagnosis and management in dermatology practices. Though there are knowledge gaps due to a lack of controlled studies, we support use of replication-deficit vaccines in all dermatologic patients who meet qualifying risk or exposure criteria. We offer strategies to optimize vaccine efficacy in patients with immunosuppression. We discuss alternative post-exposure treatments and their safety profiles. Finally, we outline supportive care recommendations for cutaneous manifestations of monkeypox. Large scale epidemiologic investigations and clinical trials will ultimately revise and extend our guidance.

A Case of Tofacitinib-Induced Lymphomatoid Papulosis With Ocular Involvement.

ABSTRACT: Janus kinase (JAK) inhibitors are being prescribed with increasing regularity in dermatology. We report on a patient who initiated treatment with tofacitinib for refractory erythema elevatum diutinum and subsequently developed a novel cutaneous outbreak characterized by firm violaceous papules on the trunk and extremities along with conjunctival injection and periorbital inflammation. Biopsy of affected tissue from both the cutaneous and ophthalmologic sources demonstrated increased numbers of CD30+ large atypical cells amid a mixed inflammatory cell infiltrate, consistent with lymphomatoid papulosis. A review of the literature reveals a plausible mechanism for the induction of persistent JAK signaling in the presence of a JAK inhibitor. We discuss this mechanism in depth because it pertains to this patient and recommend continued vigilance with the use of these immunologic agents.

American College of Rheumatology White Paper on Antimalarial Cardiac Toxicity.

Hydroxychloroquine (HCQ) and chloroquine (CQ) are well-established medications used in treating systemic lupus erythematosus and rheumatoid arthritis, as well as skin conditions such as cutaneous lupus erythematosus. In rare cases, arrhythmias and conduction system abnormalities, as well as cardiomyopathy, have been reported in association with HCQ/CQ use. Recently, however, the corrected QT interval (QTc)-prolonging potential of these medications, and risk of torsade de pointes (TdP) in particular, have been highlighted in the setting of their experimental use for COVID-19 infection. This report was undertaken to summarize the current understanding of HCQ/CQ cardiac toxicity, describe QTc prolongation and TdP risks, and discuss areas of priority for future research. A working group of experts across rheumatology, cardiology, and dermatology performed a nonsystematic literature review and offered a consensus-based expert opinion. Current data clearly indicate that HCQ and CQ are invaluable medications in the management of rheumatic and dermatologic diseases, but they are associated with QTc prolongation by directly affecting cardiac repolarization. Prescribing clinicians should be cognizant of this small effect, especially in patients taking additional medications that prolong the QTc interval. Long-term use of HCQ/CQ may lead to a cardiomyopathy associated with arrhythmias and heart failure. Risk and benefit assessment should be considered prior to initiation of any medication, and both initial and ongoing risk-benefit assessments are important with regard to prescription of HCQ/CQ. While cardiac toxicity related to HCQ/CQ treatment of rheumatic diseases is rarely reported, it can be fatal. Awareness of the potential adverse cardiac effects of HCQ and CQ can increase the safe use of these medications. There is a clear need for additional research to allow better understanding of the cardiovascular risk and safety profile of these therapies used in the management of rheumatic and cutaneous diseases.

A Multidisciplinary Collaborative Approach to Retinal Toxic Effects Screening for Dermatology Patients Taking Antimalarials.

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.

A virtual faculty exchange program enhances dermatology resident education in the COVID-19 era: a survey study.

One of the many consequences of the COVID-19 pandemic was the cancelation of the 2020 American Academy of Dermatology Annual Meeting. This conference historically features lectures from world-renowned experts in all areas of dermatology, thus providing an important educational experience for dermatology residents. We hypothesized that the cancellation of this meeting produced a substantial educational loss for dermatology residents. To mitigate this impact, we developed a virtual faculty exchange program and surveyed dermatology residents' perspectives on its implementation. All participating residents found the virtual faculty exchange useful and would recommend it to other residents/programs. Moreover, all residents wanted to participate in more faculty exchange sessions as well as incorporate them throughout the academic year. Additionally, this educational program eliminated the potential cost of >$15,000 in flights and >24 metric tons of carbon emissions. This virtual faculty exchange program is a viable tool to enhance dermatology resident education in the COVID-19 era.

American College of Rheumatology, American Academy of Dermatology, Rheumatologic Dermatology Society, and American Academy of Ophthalmology 2020 Joint Statement on Hydroxychloroquine Use With Respect to Retinal Toxicity.

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 <2% for usage up to 10 years. Widespread adoption of more sensitive testing techniques, such as optical coherence tomography and automated visual fields, by eye care providers will allow the detection of early toxicity and thus preserve the patient's visual function. Baseline testing is advised to rule out confounding disease when a patient is started on HCQ. Annual screening with sensitive tests should begin no more than 5 years after treatment initiation. Providers should be sensitive to the medical value of HCQ, and not stop the drug for uncertain indications. It is important to note that effective communication among prescribing physicians, patients, and eye care providers will optimize the utility and safety of HCQ.