Publications by authors named "Robert G Micheletti"

83 Publications

Cutaneous Manifestations of COVID-19: Characteristics, Pathogenesis, and the Role of Dermatology in the Pandemic.

Cutis 2021 Apr;107(4):209-215

Drs. Alam, Lewis, Steele, Rosenbach, and Micheletti are from the Department of Dermatology, University of Pennsylvania, Philadelphia. Dr. Harp is from New York-Presbyterian/Weill Cornell Medical Center, New York.

Cutaneous manifestations of COVID-19-SARS-CoV-2-are common and varied. Morbilliform, vesicular, and urticarial eruptions may be nonspecific initial features of the disease. Chilblainlike lesions on the fingers or toes typically occur as part of a resolution phase, signifying a milder course, whereas livedoid lesions and retiform purpura are associated with coaguloapthy and more severe disease. Additionally, a severe Kawasaki-like multisystem inflammatory syndrome rarely is seen in children. This diverse range of cutaneous manifestations in COVID-19 reflects a spectrum of host immunologic responses to SARS-CoV-2 and may inform disease pathophysiology.
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http://dx.doi.org/10.12788/cutis.0230DOI Listing
April 2021

Dermatologic support for oncology: Quantifying the consultative services received by hospitalized oncology patients.

J Am Acad Dermatol 2021 May 2. Epub 2021 May 2.

Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:

Hospitalized oncology patients often require multidisciplinary care. Inpatient consultative dermatologists can provide expertise in the management of cutaneous complications that patients with cancer may experience. The goal of this study was to quantify the types of consults received by hospitalized oncology patients to better understand the utilization of dermatology consults in this population. Hospital billing codes were used to identify inpatient oncology patients and the types of consults they received at a single quaternary care hospital center. Between July 1, 2015, and January 31, 2020, 14,175 patients were admitted to an oncology service for more than 24 hours, and 5,243 (37%) of these patients received at least 1 consultation during their hospital admission. These patients received a total of 10,492 consults from 101 different services. Dermatology had the fifth-highest number of consults (n = 623; 5.9%). Among patients receiving consults, 608 (11.6%) received inpatient dermatology consults. Infectious disease was the service with the most consults (n = 1,485; 14.2%) and was also the service most commonly co-consulted with dermatology (n = 262; 42.1%). The inpatient consultative dermatology service is highly utilized among hospitalized oncology patients, suggesting that expertise in dermatologic care is valued by oncology teams.
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http://dx.doi.org/10.1016/j.jaad.2021.04.088DOI Listing
May 2021

Long-term Physical and Psychological Outcomes of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis.

JAMA Dermatol 2021 May 5. Epub 2021 May 5.

Hennepin Healthcare, University of Minnesota Medical School, Minneapolis.

Importance: Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is known to cause multiple end-organ complications in its acute phase, but less is known about the long-term association with patients' mental health and quality of life.

Objective: To examine the chronic physical and psychological sequelae affecting patients with SJS/TEN.

Design, Setting, And Participants: A survey study conducted at 11 academic health centers in the US evaluated 121 adults diagnosed with SJS/TEN by inpatient consultive dermatologists between January 1, 2009, and September 30, 2019.

Interventions: Patients completed a survey that included the following validated questionnaires: Patient Health Questionnaire-9 (PHQ-9), Generalized Anxiety Disorder-7 (GAD-7), Primary Care Post-Traumatic Stress Disorder Screen (PC-PTSD), and the 12-item Short Form Health Survey (SF-12). The survey also included questions created by the study team regarding fear, patient education, and long-term sequelae relevant to SJS/TEN.

Main Outcomes And Measures: Primary outcome measures were the percentage of patients reporting long-term physical sequelae; the percentage of patients with positive results on PHQ-9, GAD-7, and PC-PTSD screening; and the numeric score on the SF-12 (score of 50 defined as average physical and mental well-being).

Results: A total of 121 individuals (73 women [60.3%]; mean [SD] age, 52.5 [17.1] years) completed the survey (response rate, 29.2%). The most common long-term physical sequelae reported were cutaneous problems (102 of 121 [84.3%]), ocular problems (72 of 121 [59.5%]), and oral mucosal problems (61 of 120 [50.8%]). A total of 53.3% (64 of 120) of the respondents had results indicating depression on the PHQ-9, 43.3% (52 of 120) showed signs of anxiety on the GAD-7, and 19.5% had results indicating PTSD on the PC-PTSD. The mean (SD) SF-12 Physical Component Summary score was 42.4 (22.8), and the mean Mental Component Summary score was 46.1 (20.9). A total of 28.2% (33 of 117) of the respondents were unable to work, 68.1% (81 of 119) were fearful of taking new medications, and 30.0% (36 of 120) avoided taking prescribed medications for a diagnosed medical condition.

Conclusions And Relevance: This survey study found that long-term physical sequelae, depression, and anxiety appear to be common in patients with SJS/TEN, with implications for health and well-being. Improved awareness of these complications may assist health professionals in offering medical care, counseling, and support to patients with SJS/TEN.
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http://dx.doi.org/10.1001/jamadermatol.2021.1136DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8100906PMC
May 2021

Advances in cutaneous vasculitis research and clinical care.

Ann Transl Med 2021 Mar;9(5):439

Departments of Dermatology and Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Vasculitis is characterized by inflammation and destruction of blood vessels, resulting in downstream ischemic tissue damage. Diagnosis of vasculitis is a careful exercise in clinical-pathologic correlation, depending upon the clinical manifestations, organs involved, the size of affected blood vessels, imaging, and laboratory findings. While some vasculitis subtypes may be confined to the skin, serious internal organ involvement or underlying disease states may also occur. Accordingly, the skin plays an important role in the diagnostic process and may be prognostically important in some cases, signifying more severe systemic disease. The skin also provides opportunities for tissue-based translational research, improving understanding of disease pathophysiology. Dermatologists, therefore, play a critical role in evaluating vasculitis and helping to advance vasculitis clinical care and research. Recent updates in vasculitis nomenclature and terminology, evidence-based diagnosis, pathogenesis, and investigations of targeted therapies are changing vasculitis research and leading to fundamental shifts in disease management. Treatment advances favoring evidence-based and targeted, rather than broadly immunosuppressive, therapies are in development, while a multicenter trial for skin-limited vasculitis is ongoing. Collaborative multidisciplinary research networks are key to current and future advances in vasculitis research. In this review, we describe recent developments in vasculitis clinical care and research, starting with a discussion of efforts to develop diagnostic and classification criteria, followed by updates on the evaluation and treatment of vasculitis.
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http://dx.doi.org/10.21037/atm-20-6395DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8033321PMC
March 2021

Reply.

Arthritis Rheumatol 2021 Jun 30;73(6):1089. Epub 2021 Mar 30.

University of Pennsylvania, Philadelphia, PA.

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http://dx.doi.org/10.1002/art.41661DOI Listing
June 2021

Pregnancy in Hidradenitis Suppurativa-Patient Perspectives and Practice Gaps.

JAMA Dermatol 2021 Feb;157(2):227-230

Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia.

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http://dx.doi.org/10.1001/jamadermatol.2020.5162DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7807387PMC
February 2021

Is There a Role for Therapeutic Drug Monitoring in Patients with Hidradenitis Suppurativa on Tumor Necrosis Factor-α Inhibitors?

Am J Clin Dermatol 2021 Mar;22(2):139-147

Temerty Faculty of Medicine, University of Toronto, Toronto, ON, Canada.

Tumor necrosis factor-α inhibitors, adalimumab and infliximab, are at the forefront of biologic therapy for the management of moderate-to-severe hidradenitis suppurativa, with adalimumab as currently the only approved medication for this condition. In treating patients, primary or secondary lack of response (also termed suboptimal response) is a major burden for both patients and healthcare systems and is a challenge with biologics in part owing to the development of anti-drug antibodies following treatment. To overcome this, therapeutic drug monitoring may be conducted proactively or reactively to a patient's suboptimal response guided by measurements of trough serum drug concentrations and levels of anti-drug antibodies. While strong evidence to support the utility of therapeutic drug monitoring exists in patients with inflammatory bowel disease, current information is limited in the context of hidradenitis suppurativa. We sought to summarize the available evidence and to present the role of therapeutic drug monitoring and other dose optimization strategies in improving clinical response in patients with hidradenitis suppurativa treated with tumor necrosis factor-α inhibitors.
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http://dx.doi.org/10.1007/s40257-020-00579-zDOI Listing
March 2021

Diagnosis and management of Stevens-Johnson syndrome/toxic epidermal necrolysis.

Clin Dermatol 2020 Nov - Dec;38(6):607-612. Epub 2020 Jun 30.

Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA; Department Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA. Electronic address:

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare, immunologically mediated cutaneous adverse reaction characterized by mucous membrane and epidermal detachment, with a mortality ranging from 15% to 25%. Risk factors for the development of SJS/TEN include immune dysregulation, active malignancy, and genetic predisposition. Medications are the most common cause, particularly antimicrobials, antiepileptics, allopurinol, and nonsteroidal anti-inflammatory medications. Drug-specific CD8 T-cells and natural killer cells are thought to be the major inducers of keratinocyte apoptosis via release of soluble cytotoxic mediators, including Fas ligand, perforin/granzyme, tumor necrosis factor, and granulysin. When SJS/TEN is suspected clinically, appropriate therapy should be instituted without delay. All patients should be managed initially in an intensive care unit or burn unit under a multidisciplinary team of physicians experienced in the care of patients with SJS/TEN. Available data support the use of various pharmacologic agents to halt disease progression and improve outcomes, but no single drug has been found to be superior or beneficial for all patients. Future research should focus on developing a better understanding of the genetic susceptibility and immunopathophysiology of the disease, as well as novel diagnostic and therapeutic targets to improve patient outcomes.
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http://dx.doi.org/10.1016/j.clindermatol.2020.06.016DOI Listing
January 2021

Authors' reply to the comment "High-dose, high-frequency infliximab: A novel treatment paradigm for hidradenitis suppurativa".

J Am Acad Dermatol 2021 Apr 27;84(4):e203-e204. Epub 2020 Nov 27.

Division of Dermatology, Department of Internal Medicine, Albert Einstein College of Medicine, Bronx, New York. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2020.11.041DOI Listing
April 2021

Improving Outcomes for Patients With Epidermal Necrolysis.

JAMA Dermatol 2020 12;156(12):1289-1290

Department of Dermatology, Brigham and Women's Hospital, Harvard University of School of Medicine, Boston, Massachusetts.

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http://dx.doi.org/10.1001/jamadermatol.2020.3655DOI Listing
December 2020

Low utility of radiologic imaging in evaluating cutaneous small-vessel vasculitis: A multi-institutional retrospective study.

J Am Acad Dermatol 2021 Apr 1;84(4):1197-1199. Epub 2020 Oct 1.

Department of Dermatology, Harvard Medical School/Brigham and Women's Hospital, Boston, Massachusetts. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2020.09.051DOI Listing
April 2021

Nutritional dermatoses in the hospitalized patient.

Cutis 2020 Jun;105(6):296;302-308;E1;E2;E3;E4;E5

Department of Dermatology, University of Pennsylvania Perelman School of Medicine, Philadelphia, USA.

Cutaneous disease may be the first manifestation of an underlying nutritional deficiency, highlighting the importance of early recognition by dermatologists. Undernutrition occurs when there is an imbalance between nutrient intake and metabolic demand. Many hospitalized patients are in catabolic states due to chronic illness, infection, malabsorption, or medication. These patients are at an increased risk for undernutrition and therefore associated cutaneous disease. This review details the risk factors for nutritional deficiency, illustrates the presentations of cutaneous disease, reviews diagnostic workups, and provides suggestions for supplementation in the undernourished patient.
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June 2020

Management of cutaneous vasculitis.

Presse Med 2020 Oct 6;49(3):104033. Epub 2020 Jul 6.

Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, University of Toronto, 60 Murray Street, Ste 2-220, Toronto, Ontario, M5T 3L9, Canada. Electronic address:

Cutaneous vasculitis encompasses cutaneous components of systemic vasculitides, skin-limited variants of systemic vasculitides, such as IgA vasculitis or cutaneous polyarteritis nodosa, and single-organ cutaneous vasculitis, as individualized in 2012 in the Chapel Hill Consensus Conference Nomenclature. In this article, we focus on the management of skin-limited and single-organ vasculitides, often referred to, in clinical practice, as isolated "cutaneous leukocyctoclastic vasculitis", terms which may correspond to histological findings or descriptions, but are imprecise and not specific. Since most cases of isolated cutaneous vasculitis are self-limited and resolve spontaneously over 3 to 4 weeks, most patients require no systemic treatment. For those with severe, intractable, or chronic and recurring vasculitis, systemic therapy can be indicated and should be tailored to the severity of the disease. High-quality literature is lacking to guide management. Oral glucocorticoids may be required for a short period of time for painful, ulcerative, or otherwise severe disease in order to speed resolution. Among drugs which are reasonable longer-term options are colchicine, dapsone, azathioprine or hydroxychloroquine. Additional studies, including an ongoing multicenter randomized trial, are needed to determine the most effective therapies for skin-limited vasculitis.
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http://dx.doi.org/10.1016/j.lpm.2020.104033DOI Listing
October 2020

Navigating immunosuppression in a pandemic: A guide for the dermatologist from the COVID Task Force of the Medical Dermatology Society and Society of Dermatology Hospitalists.

J Am Acad Dermatol 2020 Oct 19;83(4):1150-1159. Epub 2020 Jun 19.

Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts; Division of Rheumatology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.

Dermatologists treating immune-mediated skin disease must now contend with the uncertainties associated with immunosuppressive use in the context of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic. Although the risk of infection with many commonly used immunosuppressive agents remains low, direct data evaluating the safety of such agents in coronavirus disease 2019 (COVID-19) are scarce. This article reviews and offers guidance based on currently available safety data and the most recent COVID-19 outcome data in patients with immune-mediated dermatologic disease. The interdisciplinary panel of experts emphasizes a stepwise, shared decision-making approach in the management of immunosuppressive therapy. The goal of this article is to help providers minimize the risk of disease flares while simultaneously minimizing the risk of iatrogenic harm during an evolving pandemic.
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http://dx.doi.org/10.1016/j.jaad.2020.06.051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7303642PMC
October 2020

Calcinosis Cutis in the Setting of Chronic Skin Graft-Versus-Host Disease.

JAMA Dermatol 2020 07;156(7):814-817

Dermatology Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland.

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http://dx.doi.org/10.1001/jamadermatol.2020.1157DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7344382PMC
July 2020

Cutaneous Manifestations of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Arthritis Rheumatol 2020 10 28;72(10):1741-1747. Epub 2020 Aug 28.

University of Pennsylvania, Philadelphia.

Objective: Cutaneous manifestations of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), are poorly characterized. This report describes the dermatologic features of AAV and their association with systemic manifestations of vasculitis.

Methods: A cross-sectional study identifying and comparing the cutaneous manifestations of AAV was performed using data from a large, international, collaborative effort in order to collect comprehensive clinical data on patients with vasculitis.

Results: Data from 1,184 patients with AAV from 130 centers worldwide were available. Cutaneous manifestations were common in all AAV subtypes: GPA (223 of 656, or 34%), MPA (85 of 302, or 28%), and EGPA (106 of 226, or 47%). The most frequent cutaneous manifestation in AAV (all types) was petechiae/purpura, which was observed in 181 patients (15%). Allergic and nonspecific manifestations, such as pruritus, urticaria, and maculopapular rash, were more common in EGPA than in other disease subtypes (all P < 0.01). Skin biopsy, while underutilized (performed in 22-44% of patients), was frequently found to be an effective test suitable for diagnosis of AAV (diagnostic in 68-94% of patients). Compared to patients without cutaneous manifestations, those with skin lesions more frequently had severe systemic manifestations of vasculitis (such as alveolar hemorrhage and glomerulonephritis), specifically patients with GPA or EGPA and cytoplasmic/anti-proteinase 3 (anti-PR3) ANCA-positive or ANCA-negative patients (hazard ratio >1.9 for all), but not those with MPA or perinuclear/antimyeloperoxidase ANCAs.

Conclusion: Cutaneous manifestations are common and varied in AAV and are associated with disease severity in patients with GPA, EGPA, cytoplasmic/anti-PR3 ANCA, or ANCA-negative disease. These findings underscore the potential diagnostic and prognostic importance of the cutaneous examination in the evaluation and management of AAV.
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http://dx.doi.org/10.1002/art.41310DOI Listing
October 2020

Protocol for a randomized multicenter study for isolated skin vasculitis (ARAMIS) comparing the efficacy of three drugs: azathioprine, colchicine, and dapsone.

Trials 2020 Apr 28;21(1):362. Epub 2020 Apr 28.

Divison of Rheumatology and Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, PA, USA.

Background: Skin-limited forms of vasculitis, while lacking systemic manifestations, can persist or recur indefinitely, cause pain, itch, or ulceration, and be complicated by infection or scarring. High-quality evidence on how to treat these conditions is lacking. The aim of this comparative effectiveness study is to determine the optimal management of patients with chronic skin-limited vasculitis.

Methods: ARAMIS is a multicenter, sequential, multiple assignment randomized trial with an enrichment design (SMARTER) aimed at comparing the efficacy of three drugs-azathioprine, colchicine, and dapsone-commonly used to treat various forms of isolated skin vasculitis. ARAMIS will enroll patients with isolated cutaneous small or medium vessel vasculitis, including cutaneous small vessel vasculitis, immunoglobulin A (IgA) vasculitis (skin-limited Henoch-Schönlein purpura), and cutaneous polyarteritis nodosa. Patients not responding to the initial assigned therapy will be re-randomized to one of the remaining two study drugs (Stage 2). Those with intolerance or contraindication to a study drug can be randomized directly into Stage 2. Target enrollment is 90 participants, recruited from international centers affiliated with the Vasculitis Clinical Research Consortium. The number of patients enrolled directly into Stage 2 of the study will be capped at 10% of the total recruitment target. The primary study endpoint is the proportion of participants from the pooled study stages with a response to therapy at month 6, according to the study definition.

Discussion: ARAMIS will help identify effective agents for skin-limited forms of vasculitis, an understudied group of diseases. The SMARTER design may serve as an example for future trials in rare diseases.

Trial Registration: ClinicalTrials.gov: NCT02939573. Registered on 18 October 2016.
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http://dx.doi.org/10.1186/s13063-020-04285-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7189702PMC
April 2020

Long-term sequelae from Stevens-Johnson syndrome/toxic epidermal necrolysis in a large retrospective cohort.

J Am Acad Dermatol 2021 Mar 11;84(3):784-786. Epub 2020 Apr 11.

Departments of Dermatology and Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, Massachusetts. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2020.04.020DOI Listing
March 2021

SJS/TEN 2019: From science to translation.

J Dermatol Sci 2020 Apr 7;98(1):2-12. Epub 2020 Mar 7.

Memorial Sloan Kettering Cancer Center, New York, NY, USA.

Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are potentially life-threatening, immune-mediated adverse reactions characterized by widespread erythema, epidermal necrosis, and detachment of skin and mucosa. Efforts to grow and develop functional international collaborations and a multidisciplinary interactive network focusing on SJS/TEN as an uncommon but high burden disease will be necessary to improve efforts in prevention, early diagnosis and improved acute and long-term management. SJS/TEN 2019: From Science to Translation was a 1.5-day scientific program held April 26-27, 2019, in Vancouver, Canada. The meeting successfully engaged clinicians, researchers, and patients and conducted many productive discussions on research and patient care needs.
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http://dx.doi.org/10.1016/j.jdermsci.2020.02.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7261636PMC
April 2020

Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults.

J Am Acad Dermatol 2020 Jun 7;82(6):1553-1567. Epub 2020 Mar 7.

Royal North Shore Hospital, University of Sydney, Sydney, New South Wales, Australia.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions with high morbidity and mortality. Supportive care management of SJS/TEN is highly variable. A systematic review of the literature was performed by dermatologists, ophthalmologists, intensivists, and gynecologists with expertise in SJS/TEN to generate statements for supportive care guideline development. Members of the Society of Dermatology Hospitalists with expertise in SJS/TEN were invited to participate in a modified, online Delphi-consensus. Participants were administered 9-point Likert scale questionnaires regarding 135 statements. The RAND/UCLA Appropriateness Method was used to evaluate and select proposed statements for guideline inclusion; statements with median ratings of 6.5 to 9 and a disagreement index of ≤1 were included in the guideline. For the final round, the guidelines were appraised by all of the participants. Included are an evidence-based discussion and recommendations for hospital setting and care team, wound care, ocular care, oral care, urogenital care, pain management, infection surveillance, fluid and electrolyte management, nutrition and stress ulcer prophylaxis, airway management, and anticoagulation in adult patients with SJS/TEN.
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http://dx.doi.org/10.1016/j.jaad.2020.02.066DOI Listing
June 2020

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

JAMA Dermatol 2020 03;156(3):270-279

Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.

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.
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http://dx.doi.org/10.1001/jamadermatol.2019.4221DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6990734PMC
March 2020

Creation of a Registry to Address Knowledge Gaps in Hidradenitis Suppurativa and Pregnancy.

JAMA Dermatol 2020 03;156(3):353

Division of Dermatology, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles.

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http://dx.doi.org/10.1001/jamadermatol.2019.4162DOI Listing
March 2020

Erythematous plaques and nodules on the abdomen and groin.

Cutis 2019 Oct;104(4):E24-E26

Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA.

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October 2019

A survey-based study of diagnostic and treatment concordance in standardized cases of cellulitis and pseudocellulitis via teledermatology.

J Am Acad Dermatol 2020 05 15;82(5):1221-1223. Epub 2019 Oct 15.

Division of Dermatology, Department of Internal Medicine, The Ohio State University, Columbus, Ohio. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2019.09.084DOI Listing
May 2020

High-dose, high-frequency infliximab: A novel treatment paradigm for hidradenitis suppurativa.

J Am Acad Dermatol 2020 May 4;82(5):1094-1101. Epub 2019 Oct 4.

Division of Dermatology, Department of Internal Medicine, Albert Einstein College of Medicine, Bronx, New York. Electronic address:

Background: The permanent disfigurement associated with hidradenitis suppurativa (HS) necessitates early aggressive disease intervention. Although limited data support the use of infliximab (IFX) in HS, the efficacy of high-dose, high-frequency IFX has yet to be defined.

Objective: To evaluate the efficacy of IFX 7.5 to 10 mg/kg, with a maintenance frequency every 4 weeks.

Methods: Prospective analysis of 42 patients initiating IFX 7.5 mg/kg every 4 weeks (IFX 7.5) and 16 patients receiving dose escalation to IFX 10 mg/kg every 4 weeks (IFX 10) between March 1, 2018, and February 28, 2019. The primary outcome measure (clinical response) was the proportion of patients with Physician Global Assessment of clear, minimal, or mild (score of 0-2) HS with at least a 2-grade improvement from baseline scores.

Results: The proportion of patients achieving a clinical response after initiating IFX 7.5 was 20 of 42 (47.6%) at week 4 and 17 of 24 (70.8%) at week 12. For patients receiving dose escalation to IFX 10 because of incomplete initial response, 6 of 16 (37.5%) achieved clinical response at week 4 and 6 of 12 (50%) at week 12.

Conclusions: Initiation of IFX 7.5 every 4 weeks, with possible dose escalation to IFX 10, if needed, provides optimal mitigation of HS-related disease activity.
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http://dx.doi.org/10.1016/j.jaad.2019.09.071DOI Listing
May 2020

Numerous Pink-Purple Papules in a Middle-aged Man.

JAMA Dermatol 2019 Nov;155(11):1308-1309

Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia.

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http://dx.doi.org/10.1001/jamadermatol.2019.2949DOI Listing
November 2019

Low-dose methotrexate as rescue therapy in patients with hidradenitis suppurativa and pyoderma gangrenosum developing human antichimeric antibodies to infliximab: A retrospective chart review.

J Am Acad Dermatol 2020 02 18;82(2):507-510. Epub 2019 Sep 18.

Departments of Dermatology and Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2019.09.012DOI Listing
February 2020

The ABCD-10 Risk Prediction Model for In-Hospital Mortality Among Patients With Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis-Reply.

JAMA Dermatol 2019 Sep;155(9):1088-1089

Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia.

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http://dx.doi.org/10.1001/jamadermatol.2019.0998DOI Listing
September 2019