Risk factors and outcomes of melanoma in children and adolescents: A retrospective multicenter study.

BACKGROUND: Pediatric melanoma presents with distinct clinical features compared to adult disease. OBJECTIVE: Characterize risk factors and negative outcomes in pediatric melanoma. METHODS: Multicenter retrospective study of patients under 20 years diagnosed with melanoma between January 1, 1995 and June 30, 2015 from 11 academic medical centers. RESULTS: Melanoma was diagnosed in 317 patients, 73% of whom were diagnosed in adolescence (age ≥11). Spitzoid (31%) and superficial spreading (26%) subtypes were most common and 11% of cases arose from congenital nevi. Sentinel lymph node biopsy was performed in 68% of cases and positive in 46%. Fatality was observed in 7% of cases. Adolescent patients with melanoma were more likely to have family history of melanoma (P = .046) compared to controls. LIMITATIONS: Retrospective nature, cohort size, control selection, and potential referral bias. CONCLUSION: Pediatric melanoma has diverse clinical presentations. Better understanding of these cases and outcomes may facilitate improved risk stratification of pediatric melanoma.

Vaccines and use of immunosuppressive and immunomodulatory therapy in a pediatric dermatology clinic-A single institution experience.

Vaccine type and timing are critical issues to prevent unintended infections in those on immunosuppressive therapies. We retrospectively chart reviewed patients at Children's Wisconsin Pediatric Dermatology Clinic on immunosuppressives and immunomodulators between 11/1/2012 and 6/1/2020 and found that approximately 76% of patient encounters do not have documented vaccine counseling in the medical chart before initiation of immunosuppressives and immunomodulators. As age increased, vaccine counseling was less likely to be documented (odds ratio: 0.89; 95% confidence interval: 0.84-0.95, p = .001). In addition, 13 patient encounters (4%) were not up to date with live vaccines before immunosuppressive or immunomodulating therapy. There is an opportunity to improve clinical processes to ensure documentation of vaccination status and vaccine counseling before starting immunosuppressive and immunomodulator medications in a pediatric dermatology clinic.

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

Importance: Morphea is an insidious inflammatory disorder of the skin and deeper tissues. Determining disease activity is challenging yet important to medical decision-making and patient outcomes. Objective: To develop and validate a scoring tool, the Morphea Activity Measure (MAM), to evaluate morphea disease activity of any type or severity that is easy to use in clinical and research settings. Design, Setting, and Participants: This pilot diagnostic study was conducted from September 9, 2019, to March 6, 2020, in 2 phases: development and validation. During the development phase, 14 morphea experts (dermatologists and pediatric dermatologists) used a Delphi consensus method to determine items that would be included in the MAM. The validation phase included 8 investigators who evaluated the tool in collaboration with 14 patients with pediatric morphea (recruited from a referral center [Medical College of Wisconsin]) during a 1-day in-person meeting on March 6, 2020. Main Outcomes and Measures: During the development phase, online survey items were evaluated by experts in morphea using a Likert scale (score range, 0-10, with 0 indicating not important and 10 indicating very important); agreement was defined as a median score of 7.0 or higher, disagreement as a median score of 3.9 or lower, and no consensus as a median score of 4.0 to 6.9. During the validation phase, reliability (interrater and intrarater agreement using intraclass correlation coefficients), validity (using the content validity index and κ statistics as well as correlations with the modified Localized Scleroderma Severity Index and the Physician Global Assessment of Activity using Spearman ρ coefficients), and viability (using qualitative interviews of investigators who used the MAM tool) were evaluated. Descriptive statistics were used for quantitative variables. Data on race and ethnicity categories were collected but not analyzed because skin color was more relevant for the purposes of this study. Results: Among 14 survey respondents during the development phase, 9 (64.3%) were pediatric dermatologists and 5 (35.7%) were dermatologists. After 2 rounds, a final tool was developed comprising 10 items that experts agreed were indicative of morphea activity (new lesion in the past 3 months, enlarging lesion in the past 3 months, linear lesion developing progressive atrophy in the past 3 months, erythema, violaceous rim or color, warmth to the touch, induration, white-yellow or waxy appearance, shiny white wrinkling, and body surface area). The validation phase was conducted with 14 patients (median age, 14.5 years [range, 8.0-18.0 years]; 8 [57.1%] female), 2 dermatologists, and 6 pediatric dermatologists. Interrater and intrarater agreement for MAM total scores was good, with intraclass correlation coefficients of 0.844 (95% CI, 0.681-0.942) for interrater agreement and 0.856 (95% CI, 0.791-0.901) for intrarater agreement. Correlations between the MAM and the modified Localized Scleroderma Severity Index (Spearman ρ = 0.747; P < .001) and the MAM and the Physician Global Assessment of Activity (Spearman ρ = 0.729; P < .001) were moderately strong. In qualitative interviews, evaluators agreed that the tool was easy to use, measured morphea disease activity at a single time point, and should be responsive to changes in morphea disease activity over multiple time points. Conclusions and Relevance: In this study, the MAM was found to be a reliable, valid, and viable tool to measure pediatric morphea activity. Further testing to assess validity in adults and responsiveness to change is needed.

Dealing with new members: Team members' reactions to newcomer's attractiveness and sex.

We examine how team members respond to the inclusion of new members' physical attractiveness and sex. Drawing on Social Exchange Theory, we argue and show that incumbent team members engage in three behaviors (mimicry, ingratiation, and challenging) in response to the inclusion of more or less attractive male or female members in their team. Using a multilevel experimental design, we show that existing team members mimic newcomers who are higher on physical attractiveness and that the effect is more pronounced when there is a sex match (i.e., existing males mimic new males more). Furthermore, they ingratiate toward the physically attractive newcomers who are also committed to the task. In addition, we find that existing team members challenge physically attractive females who are committed to the task. Our findings suggest that the basic combinations of primary cues of newcomers' characteristics affect intrateam behaviors and produce different outcomes across sexes for attractiveness. By shifting the attention to the effect that newcomers have on team behaviors, the study provides novel insights for scholars that help move the discussion of team membership changes beyond the traditional accounts of new member socialization and team effectiveness. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

A sixteen-year single-center retrospective chart review of Spitz nevi and spitzoid neoplasms in pediatric patients.

BACKGROUND: Spitzoid neoplasms in pediatric patients pose an interesting challenge for clinicians. More data on the clinical, histologic, and molecular characteristics of these lesions are necessary to distinguish features that may portend recurrence or malignant behavior to help determine future treatment guidelines in pediatric patients. METHODS: Institutional Review Board approval was obtained from Children's Hospital of Wisconsin to conduct a retrospective analysis of spitzoid neoplasms. Patients with biopsied or excised spitzoid neoplasms between 01/01/2000 and 08/01/2016 were included. Pertinent clinical and histologic data were collected. Atypical, unusual, or diagnostically uncertain lesions were selected for re-review. RESULTS: 266 lesions from 264 patients were included. 243 were classified as benign (91.35%), 22 as atypical (8.27%), and 1 as spitzoid melanoma (0.38%). No clinical or histologic variables were found to be statistically significant between the benign Spitz, atypical Spitz, and spitzoid melanoma cohorts. No known deaths occurred. CONCLUSION: Our findings highlight the extreme variability of spitzoid neoplasms clinically and histologically. Importantly, this study demonstrates that the vast majority of spitzoid neoplasms in pediatric populations are benign and supports conservative management of spitzoid lesions in children.

Low risk of clinically important central nervous system dysraphism in a cohort study of 69 patients with isolated aplasia cutis congenita of the head.

BACKGROUND: Aplasia cutis congenita of the head may be associated with underlying fusion defects in the skin, soft tissues, muscle, or bone. The risk of central nervous system dysraphism in patients with aplasia cutis congenita is not known; however, knowledge of underlying structural defects can inform management considerations. METHODS: This retrospective review investigated the risk of cranial central nervous system dysraphism in children presenting with aplasia cutis congenita of the head, who presented between 1/1/2000 and 6/15/2016. Inclusion criteria were subjects with aplasia cutis congenita of the head who received CT or MR imaging of the head. RESULTS: We identified a total of 69 subjects with aplasia cutis congenita affecting the head and who received imaging. The most common location of the aplasia cutis congenita lesion was the vertex scalp (49.3%). The hair collar sign was present in 27.5% of patients. Twelve of 69 patients (17.4%) demonstrated abnormalities of the bone, vasculature, or brain on head imaging. Only one patient had a diagnosis of encephalocele that required neurosurgical intervention. There was a statistical association between the hair collar sign and the presence of abnormal imaging findings (P = .029), with a negative predictive value of 89.4%. CONCLUSIONS: The incidence of central nervous system dysraphism in patients with aplasia cutis congenita of the head appears to be low, and it may not be necessary to image the head of each child presenting with this skin lesion. The hair collar sign may be a marker of underlying defects.

Ethical champions, emotions, framing, and team ethical decision making.

Research has offered a pessimistic (although limited) view regarding the effectiveness of ethical champions in teams and the social consequences they are likely to experience. To challenge this view, we conducted two multimethod (quantitative/qualitative) experimental studies in the context of entrepreneurial team decision-making to examine whether and how an ethical champion can shape team decision ethicality and whether ethical champions experience interpersonal costs. In Study 1, we found that confederate ethical champions influenced team decisions to be more ethical by increasing team ethical awareness. Focusing on the emotional expressions of ethical champions, we found that sympathetic and angry ethical champions both increased team decision ethicality but that angry ethical champions were more disliked. Analysis of team interaction videos further revealed moral disengagement in team discussions and the emergence of nonconfederate ethical champions who used business frames to argue for the ethical decision. Those emergent phenomena shifted our focus, in Study 2, to how ethical champions framed the issues and the mediating processes involved. We found that ethical champions using ethical frames not only increased team ethical awareness but also consequently reduced team moral disengagement, resulting in more ethical team decisions. Ethical champions using business frames also improved team decision ethicality, but by increasing the perceived business utility of the ethical decision. (PsycINFO Database Record (c) 2020 APA, all rights reserved).

The Effect of Interactions between Item Discrimination and Item Difficulty on Fit Statistics.

Residual-based fit statistics are among the most common indicators of fit to the Rasch model. There is considerable discussion in the literature of the efficacy of item fit statistics in detecting measurement disturbances. However, to date there has been no investigation of whether these fit statistics are robust to interactions between item discrimination and item difficulty. This study uses simulations to investigate whether interaction effects occur for fit statistics commonly used with the Rasch model. It is found that when the parameters are estimated with the Rasch model, the values of certain item fit statistics vary depending on the interaction between location and discrimination. Specifically, in the study, OUTFIT MNSQ and INFIT MNSQ provide a relatively consistent index of item discrimination across a range of item difficulties, whereas the t-statistic and the log transformed fit residual vary in a systematic fashion that depends on item location.

Risk Factors and Outcomes of Nonmelanoma Skin Cancer in Children and Young Adults.

OBJECTIVE: To identify risk factors associated with nonmelanoma skin cancer (NMSC) occurrence and survival in children. STUDY DESIGN: This was a multicenter, retrospective, case-control study of patients <20 years of age diagnosed with NMSC between 1995 and 2015 from 11 academic medical centers. The primary outcome measure was frequency of cases and controls with predisposing genetic conditions and/or iatrogenic exposures, including chemotherapy, radiation, systemic immunosuppression, and voriconazole. RESULTS: Of the 124 children with NMSC (40 with basal cell carcinoma, 90 with squamous cell carcinoma), 70% had at least 1 identifiable risk factor. Forty-four percent of the cases had a predisposing genetic condition or skin lesion, and 29% had 1 or more iatrogenic exposures of prolonged immunosuppression, radiation therapy, chemotherapy, and/or voriconazole use. Prolonged immunosuppression and voriconazole use were associated with squamous cell carcinoma occurrence (cases vs controls; 30% vs 0%, P = .0002, and 15% vs 0%, P = .03, respectively), and radiation therapy and chemotherapy were associated with basal cell carcinoma occurrence (both 20% vs 1%, P < .0001). Forty-eight percent of initial skin cancers had been present for >12 months prior to diagnosis and 49% of patients were diagnosed with ≥2 skin cancers. At last follow-up, 5% (6 of 124) of patients with NMSC died. Voriconazole exposure was noted in 7 cases and associated with worse 3-year overall survival (P = .001). CONCLUSIONS: NMSC in children and young adults is often associated with a predisposing condition or iatrogenic exposure. High-risk patients should be identified early to provide appropriate counseling and management.

The Impact of Levels of Discrimination on Vertical Equating in the Rasch Model.

Aligning scales in vertical equating carries a number of challenges for practitioners in contexts such as large-scale testing. This paper examines the impact of high and low discrimination on the results of vertical equating when the Rasch model is applied. A simulation study is used to show that different levels of discrimination introduce systematic error into estimates. A second simulation study shows that for the purpose of vertical equating, items with high or low discrimination contain information about translation constants that contains systematic error. The impact of differential item discrimination on vertical equating is examined and subsequently illustrated in terms of a real data set from a large-scale testing program, with vertical links between grade 3 and 5 numeracy tests. Implications of the results for practitioners conducting vertical equating with the Rasch model are identified, including monitoring progress over time. Implications for other item response models are also discussed.

CARD14-associated papulosquamous eruption: A spectrum including features of psoriasis and pityriasis rubra pilaris.

BACKGROUND: Heterozygous mutations in caspase recruitment domain family member 14 gene (CARD14) have been shown to be associated with psoriasis and familial pityriasis rubra pilaris (PRP). Many subjects with CARD14 mutations display features of both disorders, which can result in diagnostic uncertainty. In addition, these eruptions are often recalcitrant to conventional psoriasis therapies such as methotrexate, oral retinoids, and tumor necrosis factor-α inhibitors. OBJECTIVE: We sought to describe the clinical characteristics, family history, and response to therapy in subjects with papulosquamous eruptions due to mutations in CARD14. METHODS: Subjects were referred for genetic testing as part of a registry of subjects with inherited disorders of keratinization. DNA was isolated from blood or saliva, and multiplex targeted sequencing or whole exome sequencing was performed. Clinical histories of subjects with CARD14 mutations were reviewed. RESULTS: We identified 15 kindreds with CARD14-associated papulosquamous eruption (CAPE). Characteristic features of CAPE include early age of onset; prominent involvement of the cheeks, chin, and ears; family history of psoriasis or PRP; minimal response to conventional topical and systemic psoriasis therapies; and improvement with ustekinumab. LIMITATIONS: Relatively small sample size. CONCLUSIONS: Many subjects with CARD14 mutations display characteristics of both psoriasis and PRP. We propose the term CARD14-associated papulosquamous eruption to describe this spectrum of disease. Subjects with clinical features suggestive of CAPE should undergo CARD14 sequencing and may benefit from treatment with ustekinumab.

Mimickers of Infantile Hemangiomas.

Infantile hemangiomas (IHs) are the most common tumors of infancy and usually follow a typical course of growth and involution. We report four soft tissue tumors that were referred to the pediatric dermatology clinic as IHs and the process by which they were diagnosed and treated. Clinicians should be aware of presentations of these uncommon, but serious soft tissue tumors. Many of these mimickers have a vastly different clinical prognosis, and early intervention to limit sequelae is crucial. Biopsy of atypical lesions should be considered early in the diagnostic process since they have varied prognosis and treatment strategies.

Cardiorenal Anemia Syndrome and Survival among Heart Failure Patients in Tanzania: A Prospective Cohort Study.

BACKGROUND: Cardiorenal anemia syndrome (CRAS) is an evolving global epidemic associated with increased morbimortality and cost of care. The management of patients with CRAS remains a challenging undertaking worldwide and the lack of evidence-based clinical guidelines adds to the challenge. We aimed to explore the prevalence and survival rates of heart failure patients with CRAS in Tanzania. METHODS: We screened 789 patients and consecutively recruited 463 who met the inclusion criteria. Each participant underwent an interview, physical examination, anthropometric measurements, anemia, renal functions and echocardiographic assessment. All participants were followed until death or for up-to 180 days, whichever came first. Bivariate comparison and subsequent Cox proportional-hazards regression model were used to compare the CRAS and non-CRAS groups with respect to the primary end point. RESULTS: The mean age of participants was 46.4 ± 18.9 years, and 56.5% were women. Overall, 51.9% of participants had renal insufficiency, 72.8% were anemic and 44.4% had CRAS. During a mean follow-up of 103 ± 75 days, 57.8% of participants died. Patients with CRAS displayed a higher mortality rate (73.5%) compared to those free of CRAS (45.8%), (p < 0.001). During multivariate analysis in a cox regression model of 21 potential predictors of mortality; renal dysfunction (HR 1.9; 95% CI 1.0-3.5; p = 0.03), severe anemia (HR 1.8; 95% CI 1.0-3.1; p = 0.04), hyponatremia (HR 2.2; 95% CI 1.3-3.7; p = 0.004) and rehospitalization (HR 4.3; 95% CI 2.2-8.4; p < 0.001) proved to be the strongest factors. CONCLUSION: Cardiorenal anemia syndrome is considerably prevalent and is associated with an increase in mortality amongst patients with heart failure. In view of this, timely, aggressive and collaborative measures to improve renal functions and/or correct anemia are crucial in the management of CRAS patients. Furthermore, these findings call for guideline committees to revise and/or develop evidence-based recommendations for management of patients with CRAS.

Building a global teledermatology collaboration.

Skin disease is common in low-resource countries and is associated with significant morbidity. The disease burden is often heightened by lack of access to adequate diagnosis and treatment. Teledermatology is a growing healthcare delivery modality that allows access to subspecialty care at a distance. This article describes how a low-cost teledermatology program was launched through collaboration between the Medical College of Wisconsin and Hillside Healthcare International. Several factors are required for a teledermatology program to be successful, beginning with a partnership between two entities that targets a locally identified need and is mutually beneficial to invested partners. The program should utilize the expertise of each partner, be based on an agreed upon process with clearly defined objectives, and protect patient privacy. After a program is implemented, adaptation to address challenges and best meet the needs of all parties involved will allow for continued success and sustainability. This process can serve as a model for other programs desiring to establish similar teledermatology partnerships in an academic setting.

Lichen planus and other lichenoid dermatoses: Kids are not just little people.

Lichenoid dermatoses, a group of inflammatory skin conditions with characteristic clinical and histopathologic findings, range from common to rare. Classic lichen planus typically presents as pruritic, polygonal, violaceous flat-topped papules and plaques; many variants in morphology and location also exist. Other lichenoid dermatoses share similar clinical presentations and histopathologic findings. These include lichenoid drug eruption, lichen planus-like keratosis, lichen striatus, lichen nitidus, and keratosis lichenoides chronica. Epidemiologic characteristics vary among each lichenoid disorder. While classic lichen planus is considered a disease of adults, other lichenoid dermatoses may be more common in younger populations. The literature contains an array of reports on the variations in presentation and successful management of lichen planus and lichenoid dermatoses among diverse populations. Familiarity with the characteristics of each lichenoid dermatosis, rare or common within each patient population, is key to accomplishing timely recognition and effective management.