Publications by authors named "Megha Tollefson"

108 Publications

Clinical Features, Prognostic Factors, and Treatment Interventions for Ulceration in Patients With Infantile Hemangioma.

JAMA Dermatol 2021 Mar 31. Epub 2021 Mar 31.

Department of Dermatology, School of Medicine, University of California, San Francisco.

Importance: Ulceration is a common complication of infantile hemangioma (IH), which leads to substantial morbidity. Ulceration in IH has not been systematically studied since the advent of β-blocker therapy for IH.

Objectives: To examine treatment interventions used for ulceration in IH and identify clinical prognostic indicators of healing time.

Design, Setting, And Participants: A retrospective, multicenter cohort study was conducted on 436 consecutive patients with a clinical diagnosis of ulcerated IH and available clinical photographs. Patients receiving care at tertiary referral centers evaluated between 2012 and 2016 were included; statistical and data analysis were performed from February 7 to April 27, 2020.

Exposures: Clinical characteristics, treatment interventions, course, complications, and resource use were analyzed. Treatment interventions for ulceration in IH included local (wound care, topical), systemic (β-blocker, corticosteroids), and procedural (pulsed-dye laser).

Main Outcomes And Measures: The primary end point was time to complete or nearly complete ulceration healing. Clinical characteristics were analyzed to determine the responses to most common interventions and prognostic factors for healing of ulceration.

Results: Of the 436 patients included in the study, 327 were girls (75.0%); median age at ulceration was 13.7 weeks (interquartile range, 8.86-21.30 weeks). The median heal time was 4.79 weeks (95% CI, 3.71-5.86 weeks) with wound care alone, 5.14 weeks (95% CI, 4.57-6.00 weeks) with timolol, 6.36 weeks (95% CI, 5.57-8.00 weeks) with a systemic β-blocker, and 7.71 weeks (95% CI, 6.71-10.14 weeks) with multimodal therapy. After adjusting for IH size, a dose of propranolol less than or equal to 1 mg/kg/d was associated with shorter healing time compared with higher propranolol doses (hazard ratio, 2.04; 95% CI, 1.11 to 3.73; P = .02). Size of the IH was identified as a significant prognostic factor for healing time in multivariable analysis. Increasing size of IH portends a proportionately longer time to heal of the ulceration.

Conclusions And Relevance: Despite the use of β-blockers, this cohort study found that a subset of patients with IH ulceration continued to experience prolonged IH healing times. Larger IH size appears to be a poor prognostic factor for time to heal. For patients requiring systemic therapy, initiation of propranolol at lower doses (≤1 mg/kg/d) should be considered.
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http://dx.doi.org/10.1001/jamadermatol.2021.0469DOI Listing
March 2021

The Epidemiology of Psoriatic Arthritis over 5 Decades: A Population-Based Study.

Arthritis Rheumatol 2021 Mar 28. Epub 2021 Mar 28.

Division of Rheumatology, Mayo Clinic, Rochester, MN, United States.

Objective: To determine the incidence of psoriatic arthritis (PsA) in a US population and describe trends in incidence and mortality over 5 decades.

Methods: The previously identified population-based cohort of Olmsted County, Minnesota residents ≥18 years of age who fulfilled PsA criteria during 1970-1999 was extended to include patients with incident PsA in 2000-2017. Age- and sex-specific incidence rates and point prevalence, adjusted to 2010 US white population, were reported.

Results: There were 164 incident cases of PsA in 2000-17, with a mean age of 46.4 (SD=12.0) years and 47% females. The overall age- and sex-adjusted annual incidence of PsA per 100,000 population was 8.5 (95% CI 7.2-9.8), and higher in males (9.3, 95% CI 7.4-11.3) than females (7.7, 95% CI 5.9-9.4) in 2000-2017. Overall incidence was highest in the age range 40-59 years. The incidence rate was relatively stable in 2000-2017 with no evidence of increase overall or in males, but a modest increase of 3% per year in females, compared to 1970-1999 where a 4%/yr rise in incidence was observed. Point prevalence was 181.8 per 100,000 (95% CI 156.5-207.1) in 2015. The percentage of females increased from 39% in 1970-1999 and 41% in 2000-2009 to 54% in 2010-2017 (p=0.08). Overall survival in PsA did not differ from general population (SMR= 0.85, 95% CI 0.61-1.15).

Conclusion: The incidence of PsA in this predominantly white US population was stable in 2000-2017 in contrast to previous years. However, an increasing proportion of females was found in this study.
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http://dx.doi.org/10.1002/art.41741DOI Listing
March 2021

Body site distribution of pediatric-onset morphea and association with extracutaneous manifestations.

J Am Acad Dermatol 2021 Mar 6. Epub 2021 Mar 6.

Departments of Dermatology and Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Background: The distribution of pediatric-onset morphea and site-based likelihood for extracutaneous complications has not been well characterized.

Objective: To characterize the lesional distribution of pediatric-onset morphea and to determine the sites with the highest association of extracutaneous manifestations.

Methods: A retrospective cross-sectional study was performed. Using clinical photographs, morphea lesions were mapped onto body diagrams using customized software.

Results: A total of 823 patients with 2522 lesions were included. Lesions were more frequent on the superior (vs inferior) anterior aspect of the head and extensor (vs flexor) extremities. Linear morphea lesions were more likely on the head and neck, whereas plaque and generalized morphea lesions were more likely on the trunk. Musculoskeletal complications were more likely with lesions on the extensor (vs flexor) extremity (odds ratio [OR], 2.0; 95% confidence interval [CI], 1.2-3.4), whereas neurologic manifestations were more likely with lesions on the anterior (vs posterior) (OR, 2.8; 95% CI, 1.7-4.6) and superior (vs inferior) aspect of the head (OR, 2.3; 95% CI, 1.6-3.4).

Limitations: Retrospective nature and the inclusion of only patients with clinical photographs.

Conclusion: The distribution of pediatric-onset morphea is not random and varies with body site and within individual body sites. The risk stratification of extracutaneous manifestations by body site may inform decisions about screening for extracutaneous manifestations, although prospective studies are needed.
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http://dx.doi.org/10.1016/j.jaad.2021.03.017DOI Listing
March 2021

The spectrum of pediatric scarring alopecia: A retrospective review of 27 patients seen at Mayo Clinic.

Pediatr Dermatol 2021 Mar 1. Epub 2021 Mar 1.

Department of Dermatology, Mayo Clinic, Rochester, MN, USA.

Background/objective: There are few studies examining pediatric scarring alopecia. The objective of this study is to characterize the clinicopathologic findings, comorbidities, and treatment outcomes of pediatric patients with scarring alopecia.

Methods: Retrospective review of patients under age 18 diagnosed with scarring alopecia at Mayo Clinic from 01/01/1992 through 02/05/2019.

Results: 27 patients met inclusion criteria with a mean age of 11.2 years and a racial breakdown of 85.2% (23) White, 11.1% (3) Black, and 3.7% (1) Multiracial. Clinical scarring was noted in most (23, 85.2%). Biopsy confirmed the diagnosis in most (24, 88.9%). The most common diagnoses were folliculitis decalvans (6, 22.2%), lichen planopilaris (6, 22.2%), aplasia cutis congenita (4, 14.8%), tinea capitis (4, 14.8%), and morphea (3, 11.1%). Comorbid depression (6, 22.2%) and anxiety (6, 22.2%) were prevalent. Of the patients who received follow-up, most who pursued treatment achieved stabilization (55.5%) or slowing of progression (27.8%), with 44.4% of those treated experiencing regrowth. Mean time to stabilization in the treated population was 19.6 months. Two patients did not pursue treatment, but received follow-up and these untreated patients did not experience hair regrowth.

Conclusions: Most patients presented with clinically evident primary scarring alopecia. Biopsy may confirm the diagnosis. Active treatment should be pursued, and successful treatment often requires combination therapies. Time to stabilization often takes years. Screening for depression and anxiety should be pursued.
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http://dx.doi.org/10.1111/pde.14543DOI Listing
March 2021

Hidradenitis Suppurativa in the Pediatric Population: An International, Multicenter, Retrospective, Cross-sectional Study of 481 Pediatric Patients.

JAMA Dermatol 2021 Feb 24. Epub 2021 Feb 24.

Section of Dermatology, Division of Pediatric Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Canada.

Importance: Hidradenitis suppurativa (HS) in pediatric patients has been understudied. Increased awareness and recognition of HS prevalence in children demand efforts to better understand this condition.

Objective: To describe the demographics, clinical features, treatment, associated comorbidities, and outcomes in a large cohort of pediatric patients with HS.

Design, Setting, And Participants: International, multicenter, retrospective medical record review of pediatric patients (aged 1-18 years) with a clinical diagnosis of HS carried out in 10 dermatology clinics across the US, Canada, Israel, Australia, and Italy from January 1996 to January 2017.

Main Outcomes And Measures: Patient demographics, clinical features, severity, associated comorbidities, and treatments in pediatric patients with HS.

Results: This cross-sectional study included 481 patients diagnosed with HS. Overall, 386 (80%) were girls. The mean (SD) age of disease onset was 12.5 (2.9) years, and the mean (SD) age at diagnosis was 14.4 (3.5) years. Family history of HS was present in 111 of 271 (41%) patients. First signs/symptoms reported at disease onset were cyst/abscess in 229 of 481 (48%), pain/tenderness in 118 of 481 (25%), and papules/pustules in 117 of 481 (24%). At initial dermatologic assessment, 233 of 481 (48%) patients already had evidence of skin scarring. Disease severity (Hurley staging) was documented in 288 of 481 (60%) patients (47% stage 1, 45% stage 2 and 8% stage 3). Comorbid conditions were reported in 406 of 481 (85%) patients, the most common being obesity (263/406 [65%]) and acne vulgaris (118/406 [29%]). Complications occurred in 378 of 481 (79%) patients, the most common of which were scars or contractures (301/378 [80%]).

Conclusions And Relevance: The findings of this study indicate that there is a gap in recognizing and diagnosing pediatric HS. Pediatric patients with HS are likely to present with other comorbidities. Prospective observational and interventional studies are needed to better understand clinical course and optimal treatments for pediatric HS.
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http://dx.doi.org/10.1001/jamadermatol.2020.5435DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7905696PMC
February 2021

Trigeminal nerve electrophysiological findings in hemifacial atrophy: A systematic literature review and retrospective chart review.

Clin Neurophysiol Pract 2021 23;6:50-55. Epub 2021 Jan 23.

Department of Neurology, Mayo Clinic, Phoenix, AZ, USA.

Objective: Hemifacial atrophy (HFA) is a rare disorder characterized by progressive unilateral wasting facial soft tissue, muscle, and/or bone. Trigeminal nerve abnormalities may contribute to or result from disease pathophysiology. We aimed to gain further insights into the role of trigeminal pathophysiology along the HFA severity spectrum.

Methods: A systematic literature review was performed according to PRISMA standards. Retrospective cases of HFA from the literature and Mayo Clinic EMG database were pooled for descriptive and semi-quantitative analysis.

Results: Overall, 13 total HFA patients were identified through literature and database reviews. Trigeminal nerve testing was abnormal in 9/13 (69%), exclusively in moderate-severe cases. Abnormalities suggested a peripheral (7/9, 78%) or mixed central/peripheral (2/9, 22%) localization. Trigeminal nerve abnormalities were not identified in any of the 4 cases with mild disease severity.

Conclusion: Moderate to severe cases of HFA were associated with electrophysiological trigeminal abnormalities. No abnormalities were seen in mild cases of HFA.

Significance: Trigeminal nerve electrophysiology may serve as a biomarker of moderate-severe disease progression, likely reflecting the consequences of progressive soft tissue atrophy.
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http://dx.doi.org/10.1016/j.cnp.2020.12.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7881166PMC
January 2021

Diagnostic Delay in Psoriatic Arthritis: A Population Based Study.

J Rheumatol 2021 Feb 15. Epub 2021 Feb 15.

Division of Rheumatology, Mayo Clinic, Rochester, MN; Department of Health Sciences Research, Mayo Clinic, Rochester, MN; Departments of Medicine/Rheumatology and Biostatistics, Epidemiology and Informatics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA; Robert D. and Patricia E. Kern Center for the Science of Health Care Delivery, Mayo Clinic, Rochester, MN; Departments of Dermatology and Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN. Financial support: This study was made possible using the resources of the Rochester Epidemiology Project, which is supported by the National Institute on Aging of the National Institutes of Health under Award Number R01AG034676, and Grant Number UL1 TR002377 from the National Center for Advancing Translational Sciences (NCATS), a component of the National Institutes of Health. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Paras Karmacharya is supported by T32 AR56950 grant from the National Institute of Arthritis and Musculoskeletal and Skin Diseases for the Musculoskeletal Research Training Program. Alí Duarte-García is supported by CDC (grant number U01 U01DP006491), the Rheumatology Research Foundation Scientist Development Award, the Robert D. and Patricia E. Kern Center for the Science of Health Care Delivery, the Women's Health Career Enhancement Award and the Eaton Family Career Development Award. Conflict of interest: Dr. Ogdie has served as a consultant for AbbVie, Amgen, BMS, Celgene, Corrona, Janssen, Lilly, Novartis, and Pfizer (less than 10,000 each) and has received grants from Novartis and Pfizer to Penn and from Amgen to Forward (grants more than 10,000). Dr. Davis has received consulting fees and/or honoraria from AbbVie and Sanofi-Genzyme (less than $10,000 each) and research support from Pfizer. No other disclosures relevant to this article. Corresponding author: Paras Karmacharya, Division of Rheumatology, Mayo Clinic College of Medicine, 200 First Street S.W. Rochester, MN 55905. Email:

Objective: To examine demographic and clinical characteristics associated with diagnostic delay in psoriatic arthritis (PsA).

Methods: We characterized a retrospective, population-based cohort of incident adult (≥18 years) PsA patients from Olmsted County, MN from 2000-17. All patients met classification criteria. Diagnostic delay was defined as the time from any patient-reported PsA-related joint symptom to a physician diagnosis of PsA. Factors associated with delay in PsA diagnosis were identified through logistic regression models.

Results: Of the 164 incident PsA cases from 2000-17, 162 had a physician or rheumatologist diagnosis. Mean (SD) age was 41.5 (12.6) and 46% were females. Median time from symptom onset to physician diagnosis was 2.5 years (interquartile range: 0.5 to 7.3). By six months, 38 (23%) received a diagnosis of PsA, 56 (35%) by one year and 73 (45%) by two years after symptom onset. No significant trend in diagnostic delay was observed over calendar time. Earlier age at onset of PsA symptoms, higher body mass index, and enthesitis were associated with a diagnostic delay of >2 years, while sebopsoriasis was associated with a lower likelihood of delay.

Conclusion: In our study, more than half of PsA patients had a diagnostic delay of >2 years, and no significant improvement in time to diagnosis was noted between 2000-17. Patients with younger age at PsA symptom onset, higher BMI, or enthesitis before diagnosis were more likely to have a diagnostic delay of >2 year while patients with sebopsoriasis were less likely to have a diagnostic delay.
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http://dx.doi.org/10.3899/jrheum.201199DOI Listing
February 2021

Heck's disease occurring after Epstein-Barr virus-associated smooth muscle tumors in an immunosuppressed child.

Pediatr Dermatol 2021 Jan 4;38(1):257-259. Epub 2020 Dec 4.

Department of Dermatology, Mayo Clinic, Rochester, MN, USA.

A 10-year-old Guatemalan girl with past medical history of Epstein-Barr virus-associated smooth muscle tumors (EBV-SMT) and combined immunodeficiency presented for evaluation of painful intraoral lesions. On examination, she was noted to have multiple, white to flesh-colored, soft, flat-topped papules, and plaques on the buccal and labial mucosa. Human papillomavirus type 13 was detected on PCR with PGMY primers of previously biopsied buccal tissue, confirming a diagnosis of Heck's disease (multifocal epithelial hyperplasia). We present an immunosuppressed, pediatric patient with two rare, virus-associated neoplastic disorders that have not been previously reported to occur in the same individual.
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http://dx.doi.org/10.1111/pde.14463DOI Listing
January 2021

Consensus Statement for the Management and Treatment of Port-Wine Birthmarks in Sturge-Weber Syndrome.

JAMA Dermatol 2021 01;157(1):98-104

Department of Dermatology, University of California, Irvine School of Medicine, Irvine.

Importance: Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome involving the skin, brain, and eyes. Consensus recommendations for management are lacking.

Objective: To consolidate the current literature with expert opinion to make recommendations that will guide treatment and referral for patients with port-wine birthmarks (PWBs).

Evidence Review: In this consensus statement, 12 nationally peer-recognized experts in dermatology with experience treating patients with SWS were assembled. Key topics and questions were formulated for each group and included risk stratification, optimum treatment strategies, and recommendations regarding light-based therapies. A systematic PubMed search was performed of English-language articles published between December 1, 2008, and December 1, 2018, as well as other pertinent studies identified by the expert panel. Clinical practice guidelines were recommended.

Findings: Treatment of PWBs is indicated to minimize the psychosocial impact and diminish nodularity and potentially tissue hypertrophy. Better outcomes may be attained if treatments are started at an earlier age. In the US, pulsed dye laser is the standard for all PWBs regardless of the lesion size, location, or color. When performed by experienced physicians, laser treatment can be safe for patients of all ages. The choice of using general anesthesia in young patients is a complex decision that must be considered on a case-by-case basis.

Conclusions And Relevance: These recommendations are intended to help guide clinical practice and decision-making for patients with SWS and those with isolated PWBs and may improve patient outcomes.
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http://dx.doi.org/10.1001/jamadermatol.2020.4226DOI Listing
January 2021

The anatomic distribution of isolated and syndrome-associated port-wine stain.

Pediatr Dermatol 2021 Jan 10;38(1):77-82. Epub 2020 Nov 10.

Department of Dermatology, Mayo Clinic Rochester, Rochester, MN, USA.

Background/objectives: To determine the role of sex in port-wine stain (PWS) distribution and describe the epidemiologic and anatomic differences between syndrome-associated and non-syndrome-associated PWS using modern criteria.

Methods: A retrospective review of PWS patients aged 18 years and younger from 1995 to 2018 seen in the Department of Dermatology at an academic tertiary referral center. Cases were reviewed for sex, anatomic location, and presence of associated syndrome. 4,527 records were reviewed on the basis of ICD billing codes for congenital vascular malformations, with 516 meeting inclusion criteria.

Results: 516 patients were included in the analysis: 234 (45.4%) men and 282 (54.6%) women. A female preponderance of Sturge-Weber syndrome (18 of 23, 78%, P = .03) and a trend toward more female-isolated PWS (149 of 269, 55%, P = .72) were found. No lateral predominance observed for isolated PWS was found: 112(41.6%) limited left-side lesions and 113(42%) limited right-side lesions (P = .41). A trend toward Klippel-Trenaunay syndrome (KTS)-associated PWS occurring more commonly isolated to the left side (76 (45.5%) vs 59 (35.12%) P = .29) was found. Nine percent of SWS patients had a PWS on the body. Five percent of KTS patients had a facial PWS. The lower limb was the most common location overall of body PWS with 33.8% of isolated PWS and 81.5% of KTS patients having a lower limb lesion.

Conclusions: Female children were more likely to be diagnosed with SWS, and a trend toward more isolated PWS in women was found. No lateral predominance of isolated PWS was found, but KTS-associated PWS was more common on the left. A considerable proportion of lesions do not appear in anatomic locations traditionally considered typical in the setting of associated syndromes, which underscores the importance of conducting a complete physical examination and adhering to diagnostic criteria for those syndromes.
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http://dx.doi.org/10.1111/pde.14392DOI Listing
January 2021

Hair Loss.

Pediatr Rev 2020 Nov;41(11):570-584

Department of Dermatology and.

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http://dx.doi.org/10.1542/pir.2019-0009DOI Listing
November 2020

Spinal Neurovascular Malformations in Klippel-Trenaunay Syndrome: A Single Center Study.

Neurosurgery 2021 02;88(3):515-522

Department of Radiology, Mayo Clinic, Rochester, Minnesota.

Background: A number of studies have demonstrated spinal anomalies associated with Klippel-Trenaunay syndrome (KTS). To date, there are no large consecutive series examining the prevalence and subtype distribution of spinal neurovascular malformations in patients with KTS.

Objective: To report the spectrum and incidence of spinal neurovascular manifestations in the KTS population.

Methods: This was a cross-sectional study. Consecutive patients with definite KTS as defined by International Society for the Study of Vascular Anomalies criteria who underwent spinal neuroimaging at our institution were included. All studies were evaluated by a staff neuroradiologist and a senior radiology resident for the presence of developmental venous anomalies, cavernous malformations (CMs), and arteriovenous shunts (AVS).

Results: A total of 116 patients with definite KTS who underwent spinal neuroimaging were included. A total of 23 neurovascular anomalies were found in 19 patients (16.4%), including 4 patients with multiple anomalies. These included 5 patients with spinal cord CMs (4.3%), 14 patients with a paraspinal or epidural venous malformation (12.1%), and 4 patients with an AVS (3.4%). Of the AVS, 3 were epidural arteriovenous fistulas, 1 of which likely formed de novo in an epidural venous malformation. One was a conus medullaris arteriovenous malformation.

Conclusion: Our study cohort of 116 KTS patients demonstrated a wide spectrum of spinal neurovascular anomalies with a relatively high prevalence. Potential phenotypic descriptions of KTS should include the possibility for spinal neurovascular anomalies.
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http://dx.doi.org/10.1093/neuros/nyaa457DOI Listing
February 2021

Pediatric maculopapular cutaneous mastocytosis: Retrospective review of signs, symptoms, and associated conditions.

Pediatr Dermatol 2021 Jan 17;38(1):159-163. Epub 2020 Oct 17.

Department of Dermatology, Emory University, Atlanta, GA, USA.

Background/objectives: Though maculopapular cutaneous mastocytosis is the most common form of pediatric mastocytosis, it remains unclear which patients will experience severe symptoms. We sought to better define the presentation and the cutaneous and systemic signs and symptoms in patients with maculopapular cutaneous mastocytosis.

Methods: We analyzed retrospective data on 227 patients diagnosed with maculopapular cutaneous mastocytosis prior to age 15 years from five US clinical sites. We collected data on signs, symptoms, age of onset, and laboratory testing.

Results: Median age of onset of maculopapular cutaneous mastocytosis was 3 months, with 94% of patients presenting prior to age 2 (range 0-15 years). Patients presenting before age 2 had significantly lower serum tryptase level (P = .019). Greater number of skin lesions (P = .006), number of reported skin signs and symptoms (P < .001), and higher tryptase levels (P < .001) were associated with more systemic symptoms.

Conclusion: Children with maculopapular cutaneous mastocytosis, who have greater skin involvement, higher serum tryptase level, and more skin signs and symptoms, are more likely to have systemic symptoms.
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http://dx.doi.org/10.1111/pde.14399DOI Listing
January 2021

Intracranial and extracranial vascular manifestations of patients with a clinical diagnosis of Klippel-Trenaunay syndrome.

Neuroradiology 2021 Mar 16;63(3):409-415. Epub 2020 Oct 16.

Department of Radiology, Mayo Clinic, 200 1st St. SW, Rochester, MN, 55905, USA.

Background And Purpose: While numerous reports have demonstrated intracranial CNS anomalies associated with Klippel-Trenaunay syndrome, to our knowledge, there has not been a large consecutive study examining these anomalies. The aim of this study was to determine the spectrum of intracranial neurovascular manifestations in patients with a clinical diagnosis of Klippel-Tranaunay syndrome.

Methods: Consecutive patients with a clinical diagnosis of Klippel-Trenaunay syndrome, as defined by the International Society for the Study of Vascular Anomalies, who underwent brain contrast-enhanced CT/computed tomography angiography, MRI/magnetic resonance angiography, or digital subtraction angiography at our institution from 2000 to 2019 were included. Studies were evaluated by a neuroradiologist and a senior radiology resident for the presence of cavernous malformations, developmental venous anomalies, venous sinus developmental abnormalities, craniofacial venous malformations, intraosseous venous malformations, and intracranial/extracranial venous abnormalities.

Results: Fifty patients with definite KTS were included. Thirty-four neurovascular anomalies were found in 17 patients (34.0%), including 8 with multiple anomalies. Nine patients had developmental venous anomalies (18.0%), 7 had craniofacial venous malformations (14.0), 6 had venous sinus developmental abnormalities (12.0%), 7 had intraosseous venous malformations (14.0%), and 2 had cavernous malformations (4.0%), and 9 patients had both intracranial venous abnormalities and craniofacial or calvarial findings (13.0%).

Conclusion: Our findings demonstrate that Klippel-Trenaunay syndrome can involve a wide spectrum of intracranial neurovascular anomalies predominantly involving the venous system.
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http://dx.doi.org/10.1007/s00234-020-02560-3DOI Listing
March 2021

Use of spironolactone to treat acne in adolescent females.

Pediatr Dermatol 2021 Jan 3;38(1):72-76. Epub 2020 Oct 3.

Department of Dermatology, Mayo Clinic, Rochester, MN, USA.

Background/objectives: Studies assessing the utility of spironolactone for treating acne in adolescent females are lacking. Thus, we sought to examine spironolactone's role in treating this patient population.

Methods: A retrospective review was performed to determine the efficacy of spironolactone treatment in adolescent females seen at Mayo Clinic in Rochester, Minnesota, from 2007 to 2017.

Results: In a cohort of 80 pediatric patients with a median age of 19 years (range, 14-20 years), 64 patients (80%) experienced improvement of acne on treatment with spironolactone (median dose, 100 mg daily) with a favorable side effect profile. Approximately a quarter of patients (22.5%) had a complete response; more than half (58.8%) had a complete response or a partial response greater than 50%. Initial and maximal responses were observed at a median of 3 months and 5 months, respectively. Patients received treatment with spironolactone for a median duration of 7 months (range, 3-45 months) with limited side effects.

Conclusions: Spironolactone demonstrated efficacy in treating acne in adolescent females and is a safe long-term alternative to systemic antibiotics in these patients.
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http://dx.doi.org/10.1111/pde.14391DOI Listing
January 2021

Laryngotracheal Reconstruction in a Patient With a Central Conducting Lymphatic Anomaly.

Laryngoscope 2021 04 9;131(4):E1339-E1341. Epub 2020 Sep 9.

Department of Otolaryngology-Head and Neck Surgery, Stanford University; Aerodigestive and Airway Reconstruction Program, Lucile Packard Children's Hospital, Palo Alto, California, U.S.A.

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http://dx.doi.org/10.1002/lary.29071DOI Listing
April 2021

Bundled intervention to improve patient safety by reducing skin specimen-related errors in a tertiary dermatology practice.

J Am Acad Dermatol 2021 Apr 7;84(4):1146-1148. Epub 2020 Jul 7.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2020.06.1020DOI Listing
April 2021

Characterization of vascular stains associated with high flow.

J Am Acad Dermatol 2021 Mar 27;84(3):654-660. Epub 2020 Jun 27.

Department of Dermatology, Vagelos College of Physicians & Surgeons, Columbia University Medical Center, New York, New York; Department of Pediatrics, Vagelos College of Physicians & Surgeons, Columbia University Medical Center, New York, New York. Electronic address:

Background: High-flow vascular stains (HFVS) are lesions that have the appearance of capillary malformations/port wine stains but are associated with increased arterial flow.

Objective: To identify features of HFVS that differentiate them from typical "slow-flow" port wine stains.

Methods: Retrospective multicenter cohort study of HFVS evaluated across 7 centers was conducted. HFVS were characterized by clinical features (warmth, thrill, rapid capillary refill), radiologic findings (fast flow), or mutations associated with capillary malformation-arteriovenous malformation syndrome. Investigators reviewed photographs.

Results: The study reviewed 70 patients with HFVS (47 multifocal and 23 solitary). Most were flat (77%), warm to the touch (60%), and red or pink-red in color (35%), with heterogeneous color saturation (73%) and well-defined borders (71%). Regional soft tissue swelling/overgrowth was common (47%). Head and neck location was most common (38%). Among 34 HFVS with photographic review over time, all demonstrated changes in appearance.

Limitations: Retrospective design, recall bias, lack of standardized time points or visual analog scale, and image variability.

Conclusion: Heterogeneity of stain color saturation, warmth to touch, peripheral pallor, and overgrowth/soft tissue swelling help distinguish HFVS from port wine stains. Darkening of color and increased border demarcation may develop over time. These findings raise suspicion for HFVS and provide an indication to assess for extracutaneous involvement.
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http://dx.doi.org/10.1016/j.jaad.2020.06.985DOI Listing
March 2021

Dermatologic disorders in transgender patients: A retrospective cohort of 442 patients.

J Am Acad Dermatol 2020 Nov 25;83(5):1516-1518. Epub 2020 Jun 25.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2020.06.074DOI Listing
November 2020

Deep Learning for Dermatologists: Part I Fundamental Concepts.

J Am Acad Dermatol 2020 May 17. Epub 2020 May 17.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota; Mayo Clinic Office of Artificial Intelligence in Dermatology (AIDE).

Artificial intelligence (AI) is generating substantial interest in the field of medicine. One form of artificial intelligence, deep learning, has led to rapid advances in automated image analysis. In 2017, an algorithm demonstrated the ability to diagnose certain skin cancers from clinical photographs with the accuracy of an expert dermatologist. Subsequently, deep learning has been applied to a range of dermatology applications. Though experts will never be replaced by AI, it will certainly impact the specialty of dermatology. In this first article of a two-part series, the basic concepts of deep learning will be reviewed with the goal of laying the groundwork for effective communication between clinicians and technical colleagues. In part two of the series, the clinical applications of deep learning in dermatology will be reviewed considering limitations and opportunities.
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http://dx.doi.org/10.1016/j.jaad.2020.05.056DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7669702PMC
May 2020

Deep learning for dermatologists: Part II. Current applications.

J Am Acad Dermatol 2020 May 16. Epub 2020 May 16.

Mayo Clinic Office of Artificial Intelligence in Dermatology, Rochester, Minnesota; Department of Health Sciences Research, Division of Digital Health Sciences, Mayo Clinic, Rochester, Minnesota.

Because of a convergence of the availability of large data sets, graphics-specific computer hardware, and important theoretical advancements, artificial intelligence has recently contributed to dramatic progress in medicine. One type of artificial intelligence known as deep learning has been particularly impactful for medical image analysis. Deep learning applications have shown promising results in dermatology and other specialties, including radiology, cardiology, and ophthalmology. The modern clinician will benefit from an understanding of the basic features of deep learning to effectively use new applications and to better gauge their utility and limitations. In this second article of a 2-part series, we review the existing and emerging clinical applications of deep learning in dermatology and discuss future opportunities and limitations. Part 1 of this series offered an introduction to the basic concepts of deep learning to facilitate effective communication between clinicians and technical experts.
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http://dx.doi.org/10.1016/j.jaad.2020.05.053DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7669658PMC
May 2020

Systemic immunosuppressive therapy for inflammatory skin diseases in children: Expert consensus-based guidance for clinical decision-making during the COVID-19 pandemic.

Pediatr Dermatol 2020 May 16;37(3):424-434. Epub 2020 May 16.

Department of Dermatology, University of California San Francisco School of Medicine, San Francisco, California, USA.

Background/objectives: The COVID-19 pandemic has raised questions about the approach to management of systemic immunosuppressive therapies for dermatologic indications in children. Change to: Given the absence of data to address concerns related to SARS-CoV-2 infection and systemic immunosuppressive therapies in an evidence-based manner, a Pediatric Dermatology COVID-19 Response Task Force (PDCRTF) was assembled to offer time-sensitive guidance for clinicians.

Methods: A survey was distributed to an expert panel of 37 pediatric dermatologists on the PDCRTF to assess expert opinion and current practice related to three primary domains of systemic therapy: initiation, continuation, and laboratory monitoring.

Results: Nearly all respondents (97%) reported that the COVID-19 pandemic had impacted their decision to initiate immunosuppressive medications. The majority of pediatric dermatologists (87%) reported that they were pausing or reducing the frequency of laboratory monitoring for certain immunosuppressive medications. In asymptomatic patients, continuing therapy was the most popular choice across all medications queried. The majority agreed that patients on immunosuppressive medications who have a household exposure to COVID-19 or test positive for new infection should temporarily discontinue systemic and biologic medications, with the exception of systemic steroids, which may require tapering.

Conclusions: The ultimate decision regarding initiation, continuation, and laboratory monitoring of immunosuppressive therapy during the pandemic requires careful deliberation, consideration of the little evidence available, and discussion with families. Consideration of an individual's adherence to COVID-19 preventive measures, risk of exposure, and the potential severity if infected must be weighed against the dermatological disease, medication, and risks to the patient of tapering or discontinuing therapies.
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http://dx.doi.org/10.1111/pde.14202DOI Listing
May 2020

Management of infantile hemangiomas during the COVID pandemic.

Pediatr Dermatol 2020 May 16;37(3):412-418. Epub 2020 May 16.

University of Minnesota, Minneapolis, Minnesota, USA.

The COVID-19 pandemic has caused significant shifts in patient care including a steep decline in ambulatory visits and a marked increase in the use of telemedicine. Infantile hemangiomas (IH) can require urgent evaluation and risk stratification to determine which infants need treatment and which can be managed with continued observation. For those requiring treatment, prompt initiation decreases morbidity and improves long-term outcomes. The Hemangioma Investigator Group has created consensus recommendations for management of IH via telemedicine. FDA/EMA-approved monitoring guidelines, clinical practice guidelines, and relevant, up-to-date publications regarding initiation and monitoring of beta-blocker therapy were used to inform the recommendations. Clinical decision-making guidelines about when telehealth is an appropriate alternative to in-office visits, including medication initiation, dosage changes, and ongoing evaluation, are included. The importance of communication with caregivers in the context of telemedicine is discussed, and online resources for both hemangioma education and propranolol therapy are provided.
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http://dx.doi.org/10.1111/pde.14196DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7262142PMC
May 2020

Limited Utility of Repeated Vital Sign Monitoring During Initiation of Oral Propranolol for Complicated Infantile Hemangioma.

J Am Acad Dermatol 2020 Apr 11. Epub 2020 Apr 11.

School of Medicine and Public Health, University of Wisconsin.

Background: Initial propranolol recommendations for infantile hemangioma published in 2013 were intended as provisional best practices to be updated as evidence-based data emerged.

Methods: A retrospective multicenter study was performed to evaluate utility of prolonged monitoring after first propranolol dose and escalation(s). Inclusion criteria included diagnosis of hemangioma requiring propranolol of ≥0.3 mg/kg/dose, age <2 years, and heart rate (HR) monitoring for ≥1 hour. Data collected included demographics, dose, vital signs and adverse events.

Results: 783 subjects met inclusion criteria; median age at initiation was 112 days. None of the 1148 episodes of prolonged monitoring warranted immediate intervention or drug discontinuation. No symptomatic bradycardia or hypotension occurred during monitoring. Mean HR change from baseline to 1 hour was -8.19 +/- 15.54 and baseline to 2 hours was -9.24 +/- 15.84 bpm. Three preterm subjects had dose adjustments due to prescriber concerns about asymptomatic vital sign changes. No significant difference existed in pre-treatment HR or in HR change between those with later adverse events during treatment and those without.

Conclusion: Prolonged monitoring for initiation and escalation of oral propranolol rarely changed management and did not predict future adverse events. Few serious adverse events occurred during therapy; none were cardiovascular.
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http://dx.doi.org/10.1016/j.jaad.2020.04.013DOI Listing
April 2020

Drug reaction with eosinophilia and systemic symptoms (DRESS) in the pediatric population: A systematic review of the literature.

J Am Acad Dermatol 2020 Nov 2;83(5):1323-1330. Epub 2020 Apr 2.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a drug-induced hypersensitivity reaction that can have fatal complications. Although substantial data exist regarding DRESS in adults, to our knowledge, a systematic review of available literature has not been performed in children.

Objective: To review available data on DRESS in the pediatric population.

Methods: A systematic literature review was performed for pediatric (aged <18 years) patients with DRESS.

Results: We included 82 articles with 148 patients; of these, 97.9% experienced a skin rash, and the liver was the second most common organ involved (84.5%). Among 143 patients for which a treatment regimen was reported, 85.3% were treated with systemic steroids. Intravenous immunoglobulin alone failed to improve symptoms in 5 patients who were initially misdiagnosed, whereas those treated with intravenous immunoglobulin and steroids (2.7%) showed rapid clinical improvement. The mortality rate was low (3.0%). Complications included multiorgan failure and acute respiratory distress syndrome.

Limitations: Limitations included limited availability of data for statistical analysis.

Conclusion: Pediatric DRESS commonly involves the liver. With treatment, the prognosis is commonly good, but serious complications may occur. Corticosteroids, possibly in conjunction with intravenous immunoglobulin in severe cases, may serve as an effective, valuable treatment of pediatric DRESS.
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http://dx.doi.org/10.1016/j.jaad.2020.03.081DOI Listing
November 2020

A Comparison of Psoriasis Severity in Pediatric Patients Treated With Methotrexate vs Biologic Agents.

JAMA Dermatol 2020 04;156(4):384-392

Department of Dermatology, Radboud University, Nijmegen, the Netherlands.

Importance: Few studies have compared the use of methotrexate and biologics, the most commonly used systemic medications for treatment of moderate to severe psoriasis in children.

Objective: To assess the real-world, 6-month reduction in psoriasis severity and long-term drug survival (rate and duration of adherence to a specific drug) of methotrexate vs biologics in plaque psoriasis in children.

Design, Setting, And Participants: A retrospective medical records review was conducted at 20 European and North American centers. Treatment response was based on site-reported Psoriasis Area and Severity Index (PASI) and/or Physician Global Assessment (PGA) scores at baseline and within the first 6 months of treatment. Participants included all 234 consecutively seen children with moderate to severe psoriasis who received at least 3 months of methotrexate or biologics from December 1, 1990, to September 16, 2014, with sufficient data for analysis. Data analysis was performed from December 14, 2015, to September 1, 2016.

Main Outcomes And Measures: PASI, with a range from 0 to 72 (highest score indicating severe psoriasis), and/or PGA, with a scale of 0 (clear), 1 (minimal), 2 (mild), 3 (moderate), 4 (severe), and 5 (very severe).

Results: Of 234 pediatric patients (103 boys [44.0%]; 131 girls [56.0%]) treated with methotrexate and/or biologics, 163 patients (69.7%) exclusively received methotrexate, 47 patients (20.1%) exclusively received biologics, and 24 children (10.2%) received methotrexate and biologics sequentially. Of the latter cohort, 23 children were treated initially with methotrexate. Mean (SD) age at initiation was 11.6 (3.7) years for methotrexate and 13.3 (2.9) years for biologics (73.2% for etanercept) (P = .002). Among patients evaluated by a scoring method at 6-month follow-up, 75% or greater improvement in PASI (PASI75) was achieved in 12 of 30 patients (40.0%) receiving methotrexate and 20 of 28 patients (71.4%) receiving biologics, and PGA was clear/almost clear (PGA 0/1) in 41 of 115 patients (35.6%) receiving methotrexate and 18 of 37 patients (48.6%) receiving biologics. Achieving PASI75 and/or PGA 0/1 between baseline and 6 months was more likely with biologics than methotrexate (PASI75: odds ratio [OR], 4.56; 95% CI, 2.02-10.27; P < .001; and PGA 0/1: OR, 2.00; 95% CI, 0.98-4.00; P = .06). Decreased mean PASI and PGA scores were associated with biologics more than with methotrexate (PASI effect, -3.13; 95% CI, -4.33 to -1.94; P < .001; and PGA effect, -0.31; 95% CI, -0.56 to -0.06; P = .02). After 1, 3, and 5 years of use, overall drug survival rates for methotrexate were 77.5%, 50.3%, and 35.9%, and for biologics, the rates were 83.4%, 64.3%, and 57.1%, respectively. Biologics were associated with a better confounder-corrected drug survival than methotrexate (hazard ratio [HR], 2.23; 95% CI, 1.21-4.10; P = .01). Discontinuation owing to lack of response was comparable (HR, 1.64; 95% CI, 0.80-3.36; P = .18).

Conclusions And Relevance: Methotrexate and biologics appear to be associated with improvement in pediatric psoriasis, although biologics seem to be associated with greater reduction in psoriasis severity scores and higher drug survival rates than methotrexate in the real-world setting. Additional studies directly comparing these medications should be performed for confirmation.
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http://dx.doi.org/10.1001/jamadermatol.2019.4835DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7042803PMC
April 2020

Oral erosions associated with surreptitious marijuana vaping in an adolescent boy.

Pediatr Dermatol 2020 Mar 22;37(2):347-349. Epub 2020 Jan 22.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota.

A 15-year-old boy presented with painful ulcerations affecting the oral mucosa that were eventually attributed to marijuana vaping. In this case report, we highlight cannabis vaping as a potential cause of oral erosions due to injury and chronic inflammation of the oral mucosa.
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http://dx.doi.org/10.1111/pde.14101DOI Listing
March 2020

Treatment of superficial vascular anomalies with topical sirolimus: A multicenter case series.

Pediatr Dermatol 2020 Mar 19;37(2):272-277. Epub 2020 Jan 19.

Division of Pediatric Dermatology, University of Minnesota, Minneapolis, Minnesota.

Background: Systemic sirolimus (rapamycin) has recently been found effective in treating complex vascular anomalies by reducing the size and associated complications. Many vascular anomalies have a cutaneous component, and thus, we sought to determine whether topical administration of sirolimus may be an effective therapy, as data on the use of topical sirolimus are limited.

Objective: We reviewed the efficacy and tolerability of topical formulations of sirolimus in the treatment of various simple and combined vascular malformations and tumors.

Methods: Eighteen patients with any vascular anomaly treated exclusively with topical sirolimus were retrospectively reviewed.

Results: Eleven patients had combined venous lymphatic malformations, three had tufted angiomas, two had a lymphatic malformation, one had a venous malformation, and one had a verrucous venous malformation. All (100%) patients reported some degree of improvement and 50% of patients reported marked improvement in one or more symptoms, most commonly blebs and lymphatic drainage, and bleeding.

Limitations: The retrospective nature, small number of patients, and differences in topical preparations limit the broad application of the results.

Conclusion: Topical sirolimus appears to be a safe and useful non-invasive therapy that is well-tolerated in the treatment of the cutaneous portion of a variety of vascular anomalies.
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http://dx.doi.org/10.1111/pde.14104DOI Listing
March 2020

Sclerotherapy for Venous Malformations of Head and Neck: Systematic Review and Meta-Analysis.

Neurointervention 2020 Mar 16;15(1):4-17. Epub 2020 Jan 16.

Department of Radiology and Vascular Centers, Mayo Clinic, Rochester, MN, USA.

We performed a systematic review and meta-analysis of studies performing sclerotherapy for treatment of venous malformations (VMs) of the face, head and neck. It is our hope that data from this study could be used to better inform providers and patients regarding the benefits and risks of percutaneous sclerotherapy for treatment of face, head and neck VMs. We searched PubMed, MEDLINE, and EMBASE from 2000-2018 for studies evaluating the safety and efficacy of percutaneous sclerotherapy of neck, face and head VMs. Two independent reviewers selected studies and abstracted data. The primary outcomes were complete and partial resolution of the VM. Data were analyzed using random-effects meta-analysis. Thirty-seven studies reporting on 2,067 patients were included. The overall rate of complete cure following percutaneous sclerotherapy with any agent was 64.7% (95% confidence interval [CI], 57.4-72.0%). Sodium tetradecyl sulfate had the lowest complete cure rate at 55.5% (95% CI, 36.1-74.9%) while pingyangmycin had the highest cure rate at 82.9% (95% CI, 71.1-94.7%). Overall patient satisfaction rates were 91.0% (95% CI, 86.1-95.9%). Overall quality of life improvement was 78.9% (95% CI, 67.0-90.8%). Overall permanent morbidity/mortality was 0.8% (95% CI, 0.3-1.3%) with no cases of mortality. Our systematic review and meta-analysis of 37 studies and over 2,000 patients found that percutaneous sclerotherapy is a very safe and effective treatment modality for treatment of VMs of the head, neck and face.
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http://dx.doi.org/10.5469/neuroint.2019.00213DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7105094PMC
March 2020

Acquired Beauty Mark?: New Black Macule on the Face.

Mayo Clin Proc 2020 01;95(1):197-198

Mayo Clinic Department of Dermatology, Mayo Clinic, Rochester, MN; Department of Pediatrics and Adolescent Medicine, Mayo Clinic, Rochester, MN.

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http://dx.doi.org/10.1016/j.mayocp.2019.11.001DOI Listing
January 2020