Publications by authors named "Anita Haggstrom"

51 Publications

Neonatal lupus with left bundle branch block and cardiomyopathy: a case report.

BMC Cardiovasc Disord 2020 07 29;20(1):352. Epub 2020 Jul 29.

Indiana University School of Medicine, Indianapolis, IN, 46202, USA.

Background: Cardiac manifestations of neonatal lupus include an array of structural and conduction abnormalities due to placental transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies. Late-onset neonatal lupus cardiomyopathies, occurring outside the neonatal period, is an infrequently reported manifestation with unknown pathophysiology and poorly defined treatment regimens. Due to the rarity of this condition, additional studies and case reports are required to better understand and manage late-onset neonatal lupus cardiomyopathies.

Case Presentation: A 4-week-old female, born to a mother with known anti-SSA/Ro and anti-SSB/La autoantibodies, presents with classic cutaneous manifestations for neonatal lupus and is found to have left bundle branch block, severely dilated cardiomyopathy with an ejection fraction of 25%, and a thin echogenic dyskinetic ventricular septum. Weekly second trimester and 30-week fetal echocardiograms showed no signs of structural or conduction abnormalities. There were no histologic signs of inflammation on cardiac tissue biopsy. After a complicated hospital course, she was successfully treated with biventricular pacemaker, intravenous immunoglobulin, and plasmapheresis.

Conclusions: We present a case of late-onset neonatal lupus with severe dilated cardiomyopathy, a dyskinetic ventricular septum, and left bundle branch block. To our knowledge, the dyskinetic ventricular septum has never been reported and left bundle branch block is rarely reported in NL. This case further validates the need for long term cardiac follow up for patients born with NL, even if lacking cardiac manifestations in the peripartum period. We characterize a unique presentation of a rare clinical entity, highlighting the diagnostic challenges, and describe a successful treatment course.
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http://dx.doi.org/10.1186/s12872-020-01637-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7391615PMC
July 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.

Northwestern University Feinberg School of Medicine, Chicago, Illinois.

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

Segmental infantile hemangioma and concomitant hypertension in three African American neonates.

Pediatr Dermatol 2020 May 13;37(3):524-526. Epub 2020 Feb 13.

Department of Dermatology and Pediatrics, University of Minnesota, Minneapolis, Minnesota.

We present three African American infants with segmental, ulcerated infantile hemangiomas and concomitant, persistent hypertension. When treated with beta-blocker therapy, the hemangiomas decreased in size and the ulcerations resolved, but there was no impact on the elevated blood pressure in one of our patients. We failed to identify any associations between infantile hemangioma and hypertension in the literature.
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http://dx.doi.org/10.1111/pde.14119DOI Listing
May 2020

Seborrhea herpeticum: cutaneous herpes simplex virus infection within infantile seborrheic dermatitis.

Cutis 2019 Nov;104(5):295-296

Department of Dermatology, Indiana University School of Medicine, Indianapolis, USA.

Eczema herpeticum has been well described in the setting of atopic dermatitis (AD) and other dermatoses. We present the case of a 2-month-old infant boy with cutaneous herpes simplex virus (HSV) infection within existing diffuse infantile seborrheic dermatitis. Providers should be aware that cutaneous HSV can be confined to a seborrheic distribution and may represent underlying epidermal dysfunction secondary to seborrheic dermatitis.
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November 2019

Bathing suit ichthyosis: Two Burmese siblings and a review of the literature.

Pediatr Dermatol 2020 Jan 20;37(1):165-170. Epub 2019 Oct 20.

Departments of Dermatology, Indiana University School of Medicine, Indianapolis, IN, USA.

Bathing suit ichthyosis (BSI) is a subtype of autosomal recessive congenital ichthyosis (ARCI) characterized by the development of large platelike scales mainly limited to the trunk. It is caused by temperature sensitive variants in transglutaminase 1, encoded by the gene TGM1. We describe a rare case of intrafamilial variation in phenotypic expressivity in two Burmese siblings with BSI that demonstrates the heterogeneity of the disorder within the same family and even in the same individual across time. We also present a concise review of the genotypic spectrum of BSI from 54 cases reported in the literature as evidence that both environmental and additional genetic factors can significantly alter the clinical phenotype.
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http://dx.doi.org/10.1111/pde.14030DOI Listing
January 2020

Drug reaction with eosinophilia and systemic symptoms: Pediatric case series and literature review.

Pediatr Dermatol 2019 Nov 18;36(6):887-892. Epub 2019 Aug 18.

Department of Dermatology, Indiana University School of Medicine, Indianapolis, IN, USA.

Background: Pediatric Drug reaction with eosinophilia and systemic symptoms (DRESS) is an uncommon disease that can be difficult to diagnose. This case series and literature review highlights the clinical features of pediatric DRESS and underscores the differential diagnoses, culprit medications, and need for clinical follow-up to detect associated autoimmune sequelae.

Objective: To describe the clinical and laboratory features of pediatric DRESS, identify associated culprit medications, and discuss the natural history of disease.

Methods: Ten cases of pediatric DRESS were identified in the electronic medical record by searching the inpatient dermatology consultation list at Indiana University between 2013 and 2018. Clinical and laboratory data were collected including demographics, differential diagnoses, culprit medications, resolution of disease, and autoimmune sequelae.

Results: Pediatric patients with DRESS presented at a mean age of 11.5 years and demonstrated a mean time from drug initiation to onset of symptoms of 4 weeks. The most common inciting drugs included antibiotics (62.5%) followed by antiepileptics (37.5%). Rash and transaminitis resolved by 3 weeks, and 20% of patients, all female, developed autoimmune sequelae including Hashimoto's thyroiditis and an undifferentiated connective tissue disorder and occurred at an average of 14.5 weeks after diagnosis.

Limitations: This was a small retrospective study of an uncommon clinical diagnosis at a single institution.

Conclusions: Pediatric DRESS was most commonly caused by antibiotics which are being increasingly recognized in the literature as the predominant culprit medications. The development of autoimmune sequelae is a notable consequence that can present weeks after illness and may preferentially affect female patients.
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http://dx.doi.org/10.1111/pde.13949DOI Listing
November 2019

Epidermolysis bullosa simplex-generalized severe type due to keratin 5 p.Glu477Lys mutation: Genotype-phenotype correlation and in silico modeling analysis.

Pediatr Dermatol 2019 Jan 4;36(1):132-138. Epub 2018 Dec 4.

Epidermolysis Bullosa Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Background/objectives: Epidermolysis bullosa is a group of diseases caused by mutations in skin structural proteins. Availability of genetic sequencing makes identification of causative mutations easier, and genotype-phenotype description and correlation are important. We describe six patients with a keratin 5 mutation resulting in a glutamic acid to lysine substitution at position 477 (p.Glu477Lys) who have a distinctive, severe and sometimes fatal phenotype. We also perform in silico modeling to show protein structural changes resulting in instability.

Methods: In this case series, we collected clinical data from six patients with this mutation identified from their national or local epidermolysis bullosa databases. We performed in silico modeling of the keratin 5-keratin 14 coil 2B complex using CCBuilder and rendered with Pymol (Schrodinger, LLC, New York, NY).

Results: Features include aplasia cutis congenita, generalized blistering, palmoplantar keratoderma, onychodystrophy, airway and developmental abnormalities, and a distinctive reticulated skin pattern. Our in silico model of the keratin 5 p.Glu477Lys mutation predicts conformational change and modification of the surface charge of the keratin heterodimer, severely impairing filament stability.

Conclusions: Early recognition of the features of this genotype will improve care. In silico analysis of mutated keratin structures provides useful insights into structural instability.
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http://dx.doi.org/10.1111/pde.13722DOI Listing
January 2019

Late growth of infantile hemangiomas in children >3 years of age: A retrospective study.

J Am Acad Dermatol 2019 Feb 5;80(2):493-499. Epub 2018 Oct 5.

University of California, San Francisco, California.

Background: The proliferative phase of infantile hemangiomas (IHs) is usually complete by 9 months of life. Late growth beyond age 3 years is rarely reported.

Objective: To describe the demographic and clinic characteristics of a cohort of patients with late growth of IH, defined as growth in a patient >3 years of age.

Methods: A multicenter, retrospective cohort study.

Results: In total, 59 patients, 85% of which were female, met the inclusion criteria. The mean first episode of late growth was 4.3 (range 3-8.5) years. Head and neck location (55/59; 93%) and presence of deep hemangioma (52/59; 88%) were common characteristics. Posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities (PHACE) syndrome was noted in 20 of 38 (53%) children with segmental facial IH. Systemic therapy (corticosteroid or β-blocker) was given during infancy in 58 of 59 (98%) and 24 of 59 (41%) received systemic therapy (β-blockers) for late IH growth.

Limitations: The retrospective nature and ascertainment by investigator recall are limitations of the study.

Conclusion: Late IH growth can occur in children after 3 years of age. Risk factors include head and neck location, segmental morphology, and involvement of deep dermal/subcutaneous tissues.
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http://dx.doi.org/10.1016/j.jaad.2018.07.061DOI Listing
February 2019

Localized infantile hemangiomas of the face and scalp: Predilection for the midline and periorbital and perioral skin.

Pediatr Dermatol 2018 Nov 31;35(6):774-779. Epub 2018 Aug 31.

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

Background/objectives: Infantile hemangiomas are common vascular tumors. Identifying sites of predilection may provide insight into pathogenesis. Previous studies have suggested a predilection for the boundary of facial metameres. The objective was to observe patterns of localized hemangiomas on the face and scalp, determine sites of predilection, and place these patterns in a developmental context.

Methods: A retrospective review of photographic archives at 10 Hemangioma Investigator Group pediatric dermatology centers identified localized infantile hemangiomas of the face and scalp. Heat map software was used to identify areas of predilection. Dot maps were used to assess frequency, and densities of infantile hemangiomas were compared between facial units using t-testing. The scalp was divided into quintiles to assess relative frequencies.

Results: Four thousand one hundred fifty-three focal face and scalp infantile hemangiomas were mapped, of which 2962 (71%) were mapped to a frontal facial template. On the face, 73.8% (2186/2962) of hemangiomas occurred along the midline axis or perpendicularly across the ocular axis in a cross-shaped area of predilection intersecting at the glabella. Scalp hemangiomas show a predilection for the midline, with 149/295 (50.5%) noted on the top of the scalp at the midline (P < 0.001). Localized hemangiomas do not demonstrate a preferential laterality.

Conclusion: The distribution of localized infantile hemangiomas of the face and scalp is not random. There is preferential involvement of the midline face and scalp and the ocular axis. The regions corresponding to the boundaries between the embryonic facial segments, including the maxillary and mandibular metameres, are not accentuated in the distribution of infantile hemangiomas.
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http://dx.doi.org/10.1111/pde.13626DOI Listing
November 2018

Compound heterozygous mutations in desmoplakin associated with skin fragility, follicular hyperkeratosis, alopecia, and nail dystrophy.

Pediatr Dermatol 2018 Jul 6;35(4):e218-e220. Epub 2018 Apr 6.

Department of Dermatology, School of Medicine, Indiana University, Indianapolis, IN, USA.

Desmoplakin mutations are associated with a wide variety of phenotypes affecting the skin, nails, hair, and heart. A 21-month-old boy was born with multiple erosions resembling epidermolysis bullosa, complete alopecia, nail dystrophy, palmoplantar keratoderma, and areas of follicular hyperkeratosis. He was found to have two heterozygous mutations in the desmoplakin gene: c.478 C>T in exon 4 (p.Arg160X) and c.3630T>A in exon 23 (Tyr1210X). This case expands the clinical spectrum associated with desmoplakin mutations and highlights a mutation in exon 23 that has not been previously reported in the literature.
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http://dx.doi.org/10.1111/pde.13498DOI Listing
July 2018

Prenatal Risk Factors for PHACE Syndrome: A Study Using the PHACE Syndrome International Clinical Registry and Genetic Repository.

J Pediatr 2017 11 1;190:275-279. Epub 2017 Sep 1.

Departments of Dermatology and Pediatrics, Section of Pediatric Dermatology, Medical College of Wisconsin, Milwaukee, WI.

The cause of PHACE syndrome is unknown. In a study of 218 patients, we examined potential prenatal risk factors for PHACE syndrome. Rates of pre-eclampsia and placenta previa in affected individuals were significantly greater than in the general population. No significant risk factor differences were detected between male and female subjects.
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http://dx.doi.org/10.1016/j.jpeds.2017.06.055DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5690843PMC
November 2017

Peters anomaly in PHACE syndrome.

J AAPOS 2017 Aug 13;21(4):331-333. Epub 2017 Jul 13.

Department of Dermatology, Indiana University Health, Indianapolis, Indiana. Electronic address:

PHACE syndrome is a rare neurocutaneous disorder, with a complex pathogenesis. It presents with a large facial hemangioma associated with anomalies of the posterior fossa of the brain, arterial anomalies, cardiac anomalies, coarctation of the aorta, and eye anomalies. Ocular abnormalities are rare. We report the first published case of an infant with PHACE syndrome and Peters anomaly.
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http://dx.doi.org/10.1016/j.jaapos.2017.04.010DOI Listing
August 2017

Utility of the Hemangioma Severity Scale as a Triage Tool and Predictor of Need for Treatment.

Pediatr Dermatol 2017 Jan 16;34(1):78-83. Epub 2016 Dec 16.

Department of Dermatology, School of Medicine, Indiana University, Indianapolis, Indiana.

Background/objectives: Infantile hemangiomas (IHs) are commonly encountered in primary care and most often remain asymptomatic, resolving without sequelae. Certain characteristics are associated with a greater risk of complications, associated anomalies, and disfigurement. The heterogeneous presentation poses a clinical challenge for physicians in determining the need for treatment and subspecialty referral. This study aims to evaluate the utility of the previously published Hemangioma Severity Scale (HSS) to predict the need for treatment.

Methods: This retrospective study included 106 patients with IHs seen in the Indiana University Dermatology Clinic in 2011. Data from electronic medical records and clinical photographs taken at patients' initial visits were used to score the hemangiomas using the HSS. Treatments used over 9 to 14 months of follow-up were recorded.

Results: Four HSS score subgroups were identified. Higher HSS scores correlated with the need for treatment; 98% of patients with HSS scores of 10 or greater received local or systemic therapy. Higher HSS scores also correlated with greater frequency of complications and risks of associated structural anomalies and permanent disfigurement. Scores did not correlate with sex, age at initial presentation, history of bleeding or pain, or IH size.

Conclusions: The HSS may be a useful tool for primary care physicians in identifying high-risk IHs that may benefit from therapy. This easy-to-use scale can improve clinical outcomes by identifying which patients need intervention to minimize complications. IHs with total HSS scores of 6 or greater should be referred for subspecialty evaluation.
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http://dx.doi.org/10.1111/pde.13032DOI Listing
January 2017

Neurodevelopmental Outcomes in Children with PHACE Syndrome.

Pediatr Dermatol 2016 Jul 13;33(4):415-23. Epub 2016 Jun 13.

Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin.

Background: Practitioners who work with children with posterior fossa, facial hemangiomas, arterial anomalies, cardiovascular anomalies, and abnormalities of the eye (PHACE) syndrome need information about neurodevelopmental outcomes to provide appropriate anticipatory guidance and education for parents. This study aimed to determine the neurodevelopmental outcomes in children with PHACE syndrome and identify which children may be at greatest risk for delays.

Methods: Children with a diagnosis of PHACE syndrome (ages 4-18 yrs) were recruited from the PHACE Syndrome International Clinical Registry and Genetic Repository. Participants (n = 25) underwent a neurodevelopmental evaluation at a children's hospital tertiary care referral center between 2009 and 2013. Children completed standardized neurocognitive tests assessing multiple domains. Parents completed standardized questionnaires assessing behavioral and emotional functioning.

Results: Results were analyzed according to cohort and individual subject. Mean scores for the cohort did not differ significantly from test norms in most domains. The only subtest that the cohort scored lower on than test norms was Word Structure, a language task. Forty-four percent of the sample scored within the normal range in all domains, 28% had one score in the at-risk range (1-2 standard deviations [SDs] below the mean), 12% had two or more scores in the at-risk range, and 16% had at least one score in the impaired range (>2 SDs below the mean).

Conclusion: Although most children in this cohort of patients with PHACE syndrome did not have significant neurodevelopmental deficits, a subset of patients had delays in multiple areas. Practitioners who work with these children should routinely ask about neurocognitive and developmental skills. Children with more severe phenotypes should be referred for appropriate evaluations and intervention services.
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http://dx.doi.org/10.1111/pde.12870DOI Listing
July 2016

Rebound Growth of Infantile Hemangiomas After Propranolol Therapy.

Pediatrics 2016 Apr 7;137(4). Epub 2016 Mar 7.

Departments of Dermatology and.

Background And Objectives: Propranolol is first-line therapy for problematic infantile hemangiomas (IHs). Rebound growth after propranolol discontinuation is noted in 19% to 25% of patients. Predictive factors for rebound are not completely understood and may alter the management approach. The goal of the study was to describe a cohort of patients with IHs treated with propranolol and to identify predictors for rebound growth.

Methods: A multicenter retrospective cohort study was conducted in patients with IHs treated with propranolol. Patient demographic characteristics, IH characteristics, and specifics of propranolol therapy were obtained. Episodes of rebound growth were recorded. Patients' responses to propranolol were evaluated through a visual analog scale.

Results: A total of 997 patients were enrolled. The incidence of rebound growth was 231 of 912 patients (25.3%). Mean age at initial rebound was 17.1 months. The odds of rebound among those who discontinued therapy at <9 months was 2.4 (odds ratio [OR]: 2.4; 95% confidence interval [CI]: 1.3 to 4.5; P = .004) compared with those who discontinued therapy between 12 to 15 months of life. Female gender, location on head and neck, segmental pattern, and deep or mixed skin involvement were associated with rebound on univariate analysis. With multivariate analysis, only deep IHs (OR: 3.3; 95% CI: 1.9 to 6.0; P < .001) and female gender (OR: 1.7; 95% CI: 1.1 to 2.6; P = .03) were associated. Of those with rebound growth, 83% required therapeutic modification including 62% of patients with modifications in their propranolol therapy.

Conclusions: Rebound growth occurred in 25% of patients, requiring modification of systemic therapy in 15%. Predictive factors for rebound growth included age of discontinuation, deep IH component, and female gender. Patients with these predictive factors may require a prolonged course of therapy.
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http://dx.doi.org/10.1542/peds.2015-1754DOI Listing
April 2016

Infantile Hemangiomas in Twins: A Prospective Cohort Study.

Pediatr Dermatol 2016 Mar-Apr;33(2):178-83. Epub 2016 Feb 11.

Department of Dermatology, Oregon Health & Science University, Portland, Oregon.

Background: Twins have a higher-than-expected risk of infantile hemangiomas (IHs), but the exact reasons for this association are not clear. Comparing concordant and discordant twin pairs might help elucidate these factors and yield more information about IH risk factors.

Methods: A prospective cohort study of twin pairs from 12 pediatric dermatology centers in the United States, Canada, Argentina, and Spain was conducted. Information regarding maternal pregnancy history, family history of vascular birthmarks, zygosity (if known), and pregnancy-related information was collected. Information regarding twins (N = 202 sets) included birthweight, gestational age (GA), presence or absence of IHs, numbers and subtypes of IHs, presence of other birthmarks, and other medical morbidities.

Results: Two hundred two sets of twins were enrolled. Concordance for IH was present in 37% of twin pairs. Concordance for IH was inversely related to gestational age (GA), present in 42% of GA of 32 weeks or less, 36% of GA of 33 to 36 weeks, and 32% of GA of 37 weeks or more. Twins of GA of 34 weeks or less were more than two and a half times as likely to be concordant as those of GA of 35 weeks or more (odds ratio (OR) = 2.66, 95% confidence interval (CI) = 1.42-4.99; p < 0.01). In discordant twins, lower birthweight conferred a high risk of IH; of the 64 sets of twins with 10% or greater difference in weight, the smaller twin had IH in 62.5% (n = 40) of cases, versus 37.5% (n = 24) of cases in which the higher-birthweight twin was affected. Zygosity was reported in 188 twin sets (93%). Of these, 78% were dizygotic and 22% monozygotic. There was no statistically significant difference in rates of concordance between monozygotic twins (43%, 18/42) and dizygotic twins (36%, 52/146) (p = 0.50). In multivariate analysis comparing monozygotic and dizygotic twins, adjusting for effects of birthweight and sex, the likelihood of concordance for monozygotic was not appreciably higher than that for dizygotic twins (OR = 1.14, 95% CI = 0.52-2.49). Female sex also influenced concordance, confirming the effects of female sex on IH risk. The female-to-male ratio was 1.7:1 in the entire cohort and 1.9:1 in those with IH. Of the 61 concordant twin sets with known sex of both twins, 41% were female/female, 43% were female/male, and 16% were male/male.

Conclusions: These findings suggest that the origin of IHs is multifactorial and that predisposing factors such as birthweight, sex, and GA may interact with one another such that a threshold is reached for clinical expression.
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http://dx.doi.org/10.1111/pde.12781DOI Listing
January 2017

Development and Validation of a Quality-of-Life Instrument for Infantile Hemangiomas.

J Invest Dermatol 2015 Jun 23;135(6):1533-1539. Epub 2015 Jan 23.

Department of Dermatology and Pediatrics, Indiana University School of Medicine, Indianapolis, Indiana, USA. Electronic address:

Infantile hemangiomas (IH) are common tumors for which there is no validated disease-specific instrument to measure the quality of life in infants and their parents/caregivers during the critical first months of life. This study prospectively developed and validated a quality-of-life instrument for patients with IH and their parents/caregivers and correlated demographic and clinical features to the effects on the quality of life. A total of 220 parents/caregivers completed the 35-item Infantile Hemangioma Quality-of-Life (IH-QoL) instrument and provided demographic information. The dimensionality of the items was evaluated using factor analysis, with results suggesting four factors: child physical symptoms, child social interactions, parent emotional functioning, and parent psychosocial functioning. Each factor fit the Rasch measurement model with acceptable fit index (mean square <1.4) and demonstrated excellent internal consistency, with alpha ranging from 0.76 to 0.88. The final instrument consists of four scales with a total of 29 items. Content validity was verified by analyzing parents' responses to an open-ended question. Test-retest reliability at a 48-hour interval was supported by a total IH-QoL intraclass correlation coefficient of 0.84. Certain clinical characteristics of hemangioma, including those located on the head and neck, in the proliferative stage, and requiring treatment, are associated with a greater impact on QoL.
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http://dx.doi.org/10.1038/jid.2015.18DOI Listing
June 2015

The "biker-glove" pattern of segmental infantile hemangiomas on the hands and feet.

J Am Acad Dermatol 2014 Sep 11;71(3):542-7. Epub 2014 Jun 11.

Department of Dermatology and Pediatrics, Indiana University, Indianapolis, Indiana. Electronic address:

Background: Infantile hemangiomas (IH) on the extremities have not been systematically studied.

Objective: We sought to describe the clinical characteristics and distribution patterns of IH affecting acral surfaces and to explore the relationship among these patterns, limb development, and IH pathogenesis.

Methods: This was a retrospective multicenter cohort study. Photographic archives from 4 tertiary pediatric dermatology referral centers were searched for patients with IH larger than 1 cm and involving 1 or more digit. Hemangioma location, distribution, and morphologic subtype were recorded. Medical records were reviewed for demographic and clinical data.

Results: In all, 73 patients were identified. The most common IH pattern resembled that of a "biker glove" (73%), followed by localized IH on the distal digits (14%), segmental IH extending over the distal digits (8%), and intermediate patterns (5%). Overall, 63% of acral IH were segmental, 26% indeterminate, and 11% localized. Five patients had associated structural anomalies. Complications were noted in 33% of cases.

Limitations: Limitations were retrospective study design; selection bias based on recall and photography; documentation and follow-up were not standardized across institutions; and treatment information may not reflect current approaches.

Conclusion: Acral IH display specific patterns and are associated with a relatively high risk of ulceration.
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http://dx.doi.org/10.1016/j.jaad.2014.04.062DOI Listing
September 2014

Characteristics of noninvoluting congenital hemangioma: a retrospective review.

J Am Acad Dermatol 2014 May 12;70(5):899-903. Epub 2014 Mar 12.

Department of Dermatology, Indiana University School of Medicine, Indianapolis, Indiana. Electronic address:

Background: Noninvoluting congenital hemangioma (NICH) is a distinct vascular tumor of infancy.

Objective: We describe the clinical characteristics, histopathology, imaging, and natural history of NICH and compare our findings with previous reports.

Methods: We conducted a retrospective review of charts and photographic databases from 2 vascular anomaly centers over a 15-year period.

Results: Thirty cases of NICH were identified. All patients had fully formed vascular lesions at birth that demonstrated a nonprogressive course. The trunk and lower extremities were preferred sites and there was a female predominance. Thirteen of 30 patients reported pain. Focal necrosis and scarring was seen in a minority. Doppler studies, when performed, confirmed high vascular flow. Microscopic evaluation of 4 excised lesions showed lobular areas of endothelial cell proliferation directly adjacent to ectatic malformed vessels. Immunohistochemical studies demonstrated absence of glucose transporter-1 protein expression in every case. Wilms tumor-1 positivity was observed in lobular areas. The larger vessels did not stain with Wilms tumor-1, but some displayed D2-40 positivity.

Limitations: Patients were referred to university-based pediatric vascular anomaly centers, with potential bias toward more severe or extensive cases.

Conclusions: This retrospective study highlights the unique clinical and histopathologic features of NICH.
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http://dx.doi.org/10.1016/j.jaad.2014.01.860DOI Listing
May 2014

Congenital agminated segmental nevi of the chest.

Dermatol Online J 2013 Sep 14;19(9):19626. Epub 2013 Sep 14.

Indiana University School of Medicine.

Grouped patterns of pigmented lesions are infrequent. Of the several reports of agminated nevi, most have been Spitz nevi or blue nevi. The distribution of these nevi is often segmental, following a dermatome or the lines of Blaschko. Most segmental nevi are not agminated and develop early in childhood [1]. We describe a rare case of congenital agminated segmental nevi on the chest.
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September 2013

Pyoderma gangrenosum associated with an aseptic splenic abscess in a patient with neurofibromatosis.

Pediatr Dermatol 2015 Jan-Feb;32(1):113-7. Epub 2013 Mar 28.

Indiana University School of Medicine, Indianapolis, Indiana.

Pyoderma gangrenosum (PG) is a painful, ulcerating neutrophilic dermatosis commonly associated with a variety of underlying systemic conditions. We report a child with neurofibromatosis-1 (NF-1) and an aseptic splenic abscess who developed multifocal PG in areas of iatrogenic skin trauma. There is no clinical evidence or theoretical basis to suggest a causal relationship between NF-1 and PG. Systemic corticosteroid and cyclosporine therapy led to complete resolution of the lesions.
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http://dx.doi.org/10.1111/pde.12129DOI Listing
October 2015

Top-accessed article: topical rapamycin.

JAMA Dermatol 2013 Feb;149(2):203

Department of Dermatology, Indiana University School of Medicine, Indianapolis, IN 46202, USA.

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http://dx.doi.org/10.1001/jamadermatol.2013.750DOI Listing
February 2013

Initiation and use of propranolol for infantile hemangioma: report of a consensus conference.

Pediatrics 2013 Jan 24;131(1):128-40. Epub 2012 Dec 24.

Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.

Infantile hemangiomas (IHs) are common neoplasms composed of proliferating endothelial-like cells. Despite the relative frequency of IH and the potential severity of complications, there are currently no uniform guidelines for treatment. Although propranolol has rapidly been adopted, there is significant uncertainty and divergence of opinion regarding safety monitoring, dose escalation, and its use in PHACE syndrome (PHACE = posterior fossa, hemangioma, arterial lesions, cardiac abnormalities, eye abnormalities; a cutaneous neurovascular syndrome characterized by large, segmental hemangiomas of the head and neck along with congenital anomalies of the brain, heart, eyes and/or chest wall). A consensus conference was held on December 9, 2011. The multidisciplinary team reviewed existing data on the pharmacologic properties of propranolol and all published reports pertaining to the use of propranolol in pediatric patients. Workgroups were assigned specific topics to propose protocols on the following subjects: contraindications, special populations, pretreatment evaluation, dose escalation, and monitoring. Consensus protocols were recorded during the meeting and refined after the meeting. When appropriate, protocol clarifications and revision were made and agreed upon by the group via teleconference. Because of the absence of high-quality clinical research data, evidence-based recommendations are not possible at present. However, the team agreed on a number of recommendations that arose from a review of existing evidence, including when to treat complicated IH; contraindications and pretreatment evaluation protocols; propranolol use in PHACE syndrome; formulation, target dose, and frequency of propranolol; initiation of propranolol in infants; cardiovascular monitoring; ongoing monitoring; and prevention of hypoglycemia. Where there was considerable controversy, the more conservative approach was selected. We acknowledge that the recommendations are conservative in nature and anticipate that they will be revised as more data are made available.
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http://dx.doi.org/10.1542/peds.2012-1691DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3529954PMC
January 2013

Pediatric segmental eccrine spiradenomas: a case report and review of the literature.

Pediatr Dermatol 2013 Nov-Dec;30(6):e285-6. Epub 2012 May 21.

School of Medicine, Indiana University, Indianapolis, IndianaAssociates in Dermatology, Westlake, OhioDepartment of Dermatology, Indiana University, Indianapolis, Indiana.

Eccrine spiradenoma is a rare benign adnexal tumor that usually occurs as a solitary nodule, although there are reports of multiple eccrine spiradenomas occurring in a segmental, linear, blaschkoid, or zosteriform pattern. Segmental eccrine spiradenomas have rarely been reported with onset occurring in childhood or adolescence. We describe a case of segmental eccrine spiradenomas occurring on the neck and mandible of a child.
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http://dx.doi.org/10.1111/j.1525-1470.2012.01777.xDOI Listing
August 2014

Measuring the severity of infantile hemangiomas: instrument development and reliability.

Arch Dermatol 2012 Feb;148(2):197-202

Department of Dermatology, Indiana University School of Medicine, Indianapolis, IN 46202, USA.

Objectives: To develop instruments that measure the severity of infantile hemangiomas (Hemangioma Severity Scale [HSS]) and the complications of infantile hemangiomas for longitudinal use (Hemangioma Dynamic Complication Scale [HDCS]).

Design: Instrument development and reliability study.

Setting: Academic research.

Participants: The HSS and the HDCS were developed through the collaborative effort of members of the Hemangioma Investigator Group Research Core, an expert multi-institutional research group. After development of the scales, 13 pediatric dermatologists used the HSS to score 20 different hemangiomas. In addition, 12 pediatric dermatologists used the HDCS to score hemangioma-related complications for 24 clinical scenarios. Interrater and intrarater reliability was measured for both scales.

Main Outcome Measures: Interrater and intrarater reliability.

Results: For the HSS, interrater reliability and intrarater reliability exceeded 99%. Similarly, the HDCS had a high rate of interrater agreement; for individual items, agreement among raters was 67% to 100%, with most clinical scenarios demonstrating greater than 90% agreement. Intrarater reliability was excellent for all individual items of the HDCS.

Conclusion: The HSS and the HDCS are reliable scales that can be used to measure the severity of infantile hemangiomas, including the severity of complications for longitudinal use.
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http://dx.doi.org/10.1001/archdermatol.2011.926DOI Listing
February 2012

Treatment outcomes of secondarily impetiginized pediatric atopic dermatitis lesions and the role of oral antibiotics.

Pediatr Dermatol 2012 May-Jun;29(3):289-96. Epub 2011 Dec 9.

Department of Dermatology, Indiana University School of Medicine, 550 N. University Blvd., suite 3240, Indianapolis, IN 46202, Indiana, USA.

Patients with atopic dermatitis (AD) are predisposed to infection with Staphylococcus aureus, which worsens their skin disease; it has been postulated that the lack of antimicrobial peptides due to aberrant allergic inflammation in skin with AD could mediate this enhanced bacterial susceptibility. We sought to characterize the amounts of S. aureus and biological products found in infected AD lesions and whether treatment with topical corticosteroids and oral cephalexin as the only antimicrobial improved outcomes. Fifty-nine children with clinically and S. aureus-positive impetiginized lesions of AD were enrolled in this study. A lesion was graded clinically using the Eczema Area and Severity Index, and wash fluid was obtained from the lesion for quantitative bacterial culture and antibiotic sensitivities and measurement of bacterial products and cytokines. Subjects were re-evaluated 2 weeks after treatment. Improvement in the clinical and inflammatory characteristics of impetiginized lesions were noted, even in the 15% of lesions infected with Methicillin-resistant S. aureus (MRSA). In a subgroup of subjects whose lesions did not contain S. aureus 2 weeks after initiating treatment, beta-defensin levels were higher at both visits than in normal skin. Treatment of uncomplicated impetiginized pediatric AD with topical corticosteroids and cephalexin results in significant clinical improvement, even in subjects infected with MRSA. We propose that the inhibition of abnormal inflammation by the treatment regimen, resulting in the high levels of defensins, is involved in the improvement of AD and that systemic antibiotics do not appear to be necessary in secondary impetiginized AD.
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http://dx.doi.org/10.1111/j.1525-1470.2011.01661.xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310266PMC
September 2012

Cutaneous manifestations of connective tissue disease.

Adolesc Med State Art Rev 2011 Apr;22(1):35-53

Indiana University School of Medicine, Indianapolis, IN, USA.

There is evidence that early treatment of connective tissue in adolescence improves clinical outcomes; thus, recognition of the cutaneous manifestation of CTD is critical. This review summarizes the clinical features that are unique to children and adolescents in cutaneous (CLE) and systemic lupus erythematosus (SLE), juvenile dermatomyositis (JDM), juvenile systemic scleroderma (JSS), juvenile localized scleroderma (JLS), and juvenile inflammatory arthritis (JIA).
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April 2011

Clinical spectrum and risk of PHACE syndrome in cutaneous and airway hemangiomas.

Arch Otolaryngol Head Neck Surg 2011 Jul;137(7):680-7

Department of Dermatology, Indiana University, 550 N University Blvd, UH 3240, Indianapolis, IN 46202, USA.

Objective: To describe the clinical presentation and risk of PHACE syndrome in infants with large facial hemangiomas and concomitant airway hemangiomas.

Design: The study involved a case series of infants with cutaneous hemangiomas and airway hemangiomas extracted from a prospective multicenter cohort study. Data regarding clinical features, diagnosis, treatment, and clinical course were obtained from medical charts and physician intake forms. All patients were evaluated for PHACE syndrome using a standardized protocol.

Setting: Six academic pediatric dermatology clinics.

Patients: The study included 17 patients younger than 1 year who were diagnosed as having large (>22 cm(2)) facial hemangiomas and airway hemangiomas.

Results: Thirteen patients (76%) had hemangiomas in the bilateral mandibular distribution. Other observed facial patterns included limited involvement of the lip and chin, unilateral reticular frontotemporal and preauricular hemangiomas, and large unilateral hemifacial hemangiomas. Fourteen patients (82%) had symptomatic airway involvement. All symptomatic patients had subglottic airway hemangiomas. The airway hemangioma was circumferential in 10 patients (58%) and more focal in distribution in 7 patients (42%). All patients were treated with oral prednisolone. Eleven patients required additional multimodal therapy. Eight patients (47%) met the criteria for PHACE syndrome.

Conclusions: Airway hemangiomas represent a potentially fatal complication of infantile hemangiomas. Our data highlight cutaneous presentations in patients with subglottic hemangiomas and large (>22 cm(2)) cutaneous hemangiomas. PHACE syndrome was detected in 8 such patients (47%) in our series.
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http://dx.doi.org/10.1001/archoto.2011.113DOI Listing
July 2011

Prospective study of the frequency of hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas.

Pediatr Dermatol 2011 May-Jun;28(3):245-53. Epub 2011 Apr 26.

Section of Dermatology, Children's Mercy Hospitals and Clinics, Kansas City, Missouri, USA.

Multiple cutaneous infantile hemangiomas have been associated with hepatic hemangiomas. Screening of infants with five or more cutaneous infantile hemangiomas with abdominal ultrasound is often recommended. The aim of this study was to determine the frequency with which hepatic hemangiomas occur in infants with five or more cutaneous infantile hemangiomas compared to those with one to four cutaneous infantile hemangiomas and to characterize the clinical features of these hepatic hemangiomas. A multicenter prospective study of children with cutaneous infantile hemangiomas was conducted at pediatric dermatology clinics at Hemangioma Investigator Groups sites in the United States, Canada, and Spain between October 2005 and December 2008. Data were collected, and abdominal ultrasonography was performed on infants younger than 6 months old with five or more cutaneous infantile hemangiomas and those with one to four cutaneous infantile hemangiomas. Twenty-four (16%) of the 151 infants with five or more cutaneous infantile hemangiomas had hepatic hemangiomas identified on abdominal ultrasound, versus none of the infants with fewer than five (p = 0.003). Two of the 24 infants with hepatic hemangiomas received treatment specifically for their hepatic hemangiomas. Infants with five or more cutaneous infantile hemangiomas have a statistically significantly greater frequency of hepatic hemangiomas than those with fewer than 5. These findings support the recommendation of five or more cutaneous infantile hemangiomas as a threshold for screening infants younger than 6 months old for hepatic hemangiomas but also demonstrate that the large majority of these infants with hepatic hemangiomas do not require treatment.
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http://dx.doi.org/10.1111/j.1525-1470.2011.01420.xDOI Listing
September 2011