The stigma of skin disease.

PURPOSE OF REVIEW: The effects of skin disease on stigmatization are important but understudied in the pediatric population. Given the highly visible nature of dermatologic conditions, stigmatization is a common problem that requires significant attention in patients with skin diseases. In this review, we examine the recent literature addressing stigmatization of patients suffering from common dermatologic diseases with the goal to increase clinician awareness of these issues and identify new avenues for future research. RECENT FINDINGS: A number of studies have examined the impact of skin disease on psychosocial well being and quality of life. Although some skin diseases are often overlooked medically and considered to be primarily cosmetic issues, the long-term consequences of skin diseases on psychosocial health, especially in pediatric patients, can be profound. SUMMARY: The precipitating factors for stigma vary widely depending on age, sex, and culture. In order to effectively reduce the impact of pediatric skin diseases on psychosocial health, physicians should be able to identify specific characteristics that may increase risks for stigmatization in chidlren. Carefully monitoring psychosocial development in pediatric patients with dermatological conditions in addition to proactively guiding patients and families to appropriate resources can benefit the child's development and overall long-term well being.

Influence of infantile hemangioma severity and activity on QoL of patients and their parents: A cross-sectional study.

BACKGROUND: Quality of life (QoL) data are lacking in children with infantile hemangioma (IH) and their parents/caregivers. Available data are conflicting. OBJECTIVES: To determine QoL of (parents of) patients with IH in the proliferative phase related to IH-severity and activity. METHODS: Parents of 59 IH-patients (≤ 6 months) were asked to fill in the Dutch IH-specific QoL-questionnaire (D-IH-QoL) within 1 month after their first visit to our tertiary referral center. Hemangioma Severity Scale (HSS) score and Hemangioma Activity Score (HAS) were assessed. RESULTS: D-IH-QoL-scores were low; mean: 22.6 (range 1-56; max 116). Mean HSS score and HAS score were 8.4 (range 2-28) and 4.0 (range 1.5-6), respectively. Higher HSS scores correlated with worse QoL (ρ = .358; P = .005). HAS scores did not correlate with QoL scores. CONCLUSION: An IH only seems to have limited influence on QoL in young children and their parents in the first month of their first doctor's visit. QoL is more affected in more severe IH. Physicians should be alert to the impact of IH, optionally guided by the HSS score.

The Role of Surgery in the Management of Infantile Hemangiomas: What is the Best Timing?

Surgery for the management of infantile hemangiomas has become commonplace. Surgical technique articles are plentiful; however, little has been written about the timing of surgery. Knowledge of the biology of the tumors, data from developmental psychology, and the utility of facial reconstruction provide guidelines for timing of surgical intervention.

Psychosocial Impact of Vascular Anomalies on Children and Their Families.

Vascular anomalies are divided into tumors and malformations based on their clinical and cytologic attributes. Vascular malformations are further subcategorized as low-flow lymphatic, venous, capillary, or mixed lesions and as high-flow arteriovenous malformations. Treatment is reserved for vascular anomalies that are symptomatic or cosmetically disfiguring, and surgical and nonsurgical treatment options are widely varied with variable outcomes.

Quality of life in children with infantile hemangioma: a case control study.

BACKGROUND: Infantile hemangioma (IH) is the most common vascular tumor in children. It is controversial whether IHs has effects on the quality of life (QOL) in patients of whom IH poses no threat or potential for complication. Thus, we conducted this study to evaluate the q QOL in patients with IH and find the predictors of poor QOL. METHODS: The PedsQL 4.0 Genetic Core Scales and the PedsQL family information form were administered to parents of children with IH and healthy children both younger than 2-year-old. The quality-of-life instrument for IH (IH-QOL) and the PedsQL 4.0 family impact module were administered to parents of children with IH. We compared the PedsQL 4.0 Genetic Core Scales (GCIS) scores of the two groups. Multiple step-wise regression analysis was used to determine factors that influenced QOL in children with IH and their parents. RESULTS: Except for physical symptom, we found no significant difference in GCIS between patient group and healthy group (P = 0.409). The internal reliability of IH-QOL was excellent with the Cronbach's alpha coefficient for summary scores being 0.76. Multiple step-wise regression analysis showed that the predictors of poor IH-QOL total scores were hemangioma size, location, and mother's education level. The predictors of poor FIM total scores were hemangioma location and father's education level. The predictors of poor GCIS total scores were children's age, hemangioma location and father's education level. CONCLUSION: The findings support the feasibility and reliability of the Chinese version of IH-QOL to evaluate the QOL in children with IH and their parents. Hemangioma size, location and education level of mother are important impact factors for QOL in children with IH and their parents.

Statistical Analysis of Influences on the Psychosocial Status of Children With Hemangiomas and Their Families.

The psychologic stress on the child and family, which arise from hemangiomas, the most common neoplasm of childhood, cannot be overestimated. This study determined the preoperative and postoperative psychosocial status and variation among Oriental children with hemangiomas and their families by questionnaire. Thirty patients who underwent surgery for hemangiomas were assessed for preoperative and postoperative psychosocial status by questionnaire. The distribution of the total mean score and variation between the preoperative and postoperative status was estimated. Based on these results, the significance was statistically analyzed according to variable determinants. This study showed that hemangiomas have harmful effects on psychosocial status of patients and families. After corrective surgery, an improvement in psychosocial status was noted with respect to the self-esteem category or categories related to social activity, and in the following variables, women, face, and dissatisfaction with appearance. When the authors care for patients with hemangiomas and their families, the psychosocial health must be presumed to be at particular risk. Earlier surgical interventions with esthetic concerns have permitted the patient and family the opportunity to reduce the psychologic impact that the hemangioma may otherwise have.

Burden of Infantile Hemangioma on Family: An International Observational Cross-Sectional Study.

BACKGROUND/OBJECTIVES: Infantile hemangioma (IH) is the most frequent benign tumor of infancy resulting from vascular proliferation. Data regarding the burden on families of children with IHs are limited. This study aimed to characterize IHs and provide a comprehensive evaluation of the burden of IHs on parents of children requiring systemic treatment in the United States and Europe. METHODS: This noninterventional cross-sectional study included infants with newly diagnosed IH requiring systemic treatment. A parent or family member completed two questionnaires (Family Member questionnaire; Hemangioma Family Burden [HFB] questionnaire). RESULTS: A total of 693 individuals were evaluable in five countries. IHs were observed in more girls than boys (66%-83% female) and the mean age at inclusion was 0.44 to 1.4 years. Approximately half of patients had superficial IHs, approximately 70% of cases affected the head, and approximately 80% of cases were moderate or severe. Most patients received propranolol treatment. Their child's IH affected more than 70% of parents in each country, but fewer than 10% were offered psychological support. Approximately half of all parents reported that their child's IH affected their professional life. The global HFB score was significantly (p < 0.001) greater with greater IH severity. More than 90% of parents in each country were satisfied with the care of their child's disease. CONCLUSIONS: This international study using the validated HFB questionnaire provides further insight into the burden of IH and highlights potential areas for future focus in assisting families with affected children.

The Tissue Expander Effect in Early Surgical Management of Select Focal Infantile Hemangiomas.

IMPORTANCE: The current standard of treatment for infantile hemangiomas (IHs) involves initial observation for regression throughout infancy and childhood, with or without medical management with ß-blocker medications. Approximately 50% of the lesions respond almost completely to this regimen. However, the remaining 50% of the lesions, especially established focal IHs of the lip, nose, eyelids, forehead, cheek, and scalp, do not regress completely with this regimen or do so leaving a deformity; among these lesions, early surgical management may result in a superior aesthetic and functional outcome. OBJECTIVE: To identify select focal head and neck lesions of IH that will likely not completely involute with medical management and that are ideal for a 1-stage surgical excision. DESIGN, SETTING, AND PARTICIPANTS: In this case series, records of infants and children presenting to a tertiary care vascular anomalies center for management of IHs by the senior author were reviewed. Representative examples of focal IHs of the lips, nose, eyelids, cheek, and glabella demonstrating the tissue expansion effect were selected for presentation. Expert opinion based on more than 20 years of experience of the senior surgeon treating more than 2000 patients with focal IH and long-term clinical follow-up is also provided. MAIN OUTCOMES AND MEASURES: Eradication of the IH while restoring aesthetic form and function to the face. RESULTS: Five examples of patients with focal IHs of the lip, nose, eyelid, cheek, and glabella demonstrating the tissue expander effect who were successfully treated with surgery are presented. The 5 patients with these lesions ranged in age from 3 months to 5 years old, and all of them were female. One of these patients was treated with ß-blockers, and another with steroids, with incomplete response to treatment prior to undergoing surgery. The tissue expander effect of a focal IH on adjacent, unaffected tissue facilitated excision of the lesion and primary closure without distortion of anatomical subunits in all 5 of these cases. Improved cosmesis with either improved or unaffected function was demonstrated. CONCLUSIONS AND RELEVANCE: Clinicians should consider early surgical intervention in infants with select focal infantile hemangiomas in lieu of prolonged observation or medical management. The psychological benefit of early removal of these disfiguring lesions has not been quantified, but is subjectively apparent to clinicians and the families of patients. Furthermore, the costs and unknown long-term sequelae of ß-blocker medication, which is the current standard of treatment for IHs along with observation for regression, have not yet been quantified but will gain increasing salience in the current medical climate. LEVEL OF EVIDENCE: 5.

Psychological comorbidities and compliance to interventional treatment of patients with cutaneous vascular malformations.

PURPOSE: The aim of this study was to assess qualitatively the psychological stressors affecting patients with cutaneous vascular malformations and hemangiomas (CVM-H) and their impact on compliance to interventional treatment. METHODS: A retrospective chart review was conducted of all patients with CVM-H treated by interventional neuroradiology at a single academic institution during a five-year period (2009-2014). Psychological complaints were documented during each clinic visit by a neuroradiologist. Compliance to interventional treatment was defined by adherence to the scheduled treatment sessions. Fisher's exact test was used to assess for associations between psychological complaints and compliance. RESULTS: Seventy-five patients were assessed, of whom 49 (65.3%) were female, with an age range of 2-78 years (mean age 30.2 years). All except one patient older than seven years of age (n = 71; 94.6%) had a psychological complaint, including fear of negative appearance (n = 53; 70.6%), dissatisfaction with appearance (n = 46; 61.3%), low self-esteem (n = 35; 46.6%), anxiety (n = 16; 21.3%), stress (n = 13; 17.3%), bullying (n = 5; 6.6%), and low mood (n = 4; 5.3%). Twenty-three (31%) patients were non-compliant. Low self-esteem was significantly associated with non-compliance (p = 0.0381). CONCLUSION: There is a high prevalence of psychological comorbidities among patients treated for CVM-H. This has potential implications for interventional treatment, as it was found that low self-esteem is significantly associated with non-compliance. These results suggest the need for early psychological support in these patients in order to maximize compliance to interventional treatment.

Social Impact of Facial Infantile Hemangiomas in Preteen Children.

IMPORTANCE: Involuted infantile facial hemangiomas (IHs) may adversely affect the social skills of children. OBJECTIVE: To assess the social impact of involuted facial IHs, with or without prior treatment, in preteen children. DESIGN, SETTING, AND PARTICIPANTS: An observational, cross-sectional study of social anxiety and skills in preteen children with facial IHs diagnosed during infancy. The study took place in an academic institution and a community dermatology practice between January 1, 2013, and July 30, 2014. Records on 236 children with IHs located in a cosmetically sensitive area were identified; of those, 144 potential participants (parents) were reached by telephone and mailed study packets. Thirty completed questionnaires were returned. Data analysis was performed from August 1, 2014, to September 7, 2015. INTERVENTIONS: The questionnaires included the following psychiatric scales: (1) Social Anxiety Scale for Children-Revised (SASC-R), completed by parents and children, including the domains of Fear of Negative Evaluation and Social Avoidance/Distress in New Situations (SAD-New) (higher scores indicate greater social anxiety), and (2) Social Competency Inventory (SCI), completed by parents, including the domains of Prosocial Behavior and Social Initiative (lower scores indicate poorer social competency). MAIN OUTCOMES AND MEASURES: Demographics, clinical details, and survey responses were collected. Analysis was conducted using t tests to compare scores for each survey domain with established normative data and between sex as well as between treatment vs nontreatment groups. RESULTS: Of the 144 potential participants, 30 (21%) responded. The mean age of the preteen subjects was 10.0 years (range, 5.4-12.9 years) with a 2:1 female to male ratio. Twenty-five children (83%) had a single IH, and the remaining 5 participants (17%) had multiple IHs, with at least 1 IH in a cosmetically sensitive area. The periocular region was the most common site of the IH (10 [33%]), followed by the nose (6 [20%]), cheek (5 [17%]), forehead (4 [13%]), lip or perioral region (4 [13%]), and ear (1 [3%]). Eighteen children (60%) had received treatment for their IH. With results reported as mean (SD), the SASC-R test showed that social anxiety of the children was not increased over normative data; however, those who did not receive IH treatment had significantly greater anxiety for new situations compared with those who received treatment (SAD-New: 15.5 [5.1] vs 11.5 [3.8]; P = .02). Results of the SCI scale indicated that the Prosocial Orientation domain score for the children was similar to normative data (3.96 [0.48] vs 3.89 [0.55], P = .50). Social Initiative domain scores were significantly poorer in children who did not receive treatment vs those who received treatment (3.45 [0.43] vs 4.03 [0.55]; P = .006). CONCLUSIONS AND RELEVANCE: Preteen children with involuted, untreated facial IHs have higher Social Anxiety domain scores in new situations and decreased Social Initiative domain scores compared with children who receive treatment for facial IH. Although this study is limited by a small sample size, it raises important considerations for whether early treatment of facial IHs in cosmetically sensitive areas has a beneficial effect on social skills in preteens.

Quality of life can be improved by surgical management of giant hepatic haemangioma with enucleation as the preferred option.

OBJECTIVES: Surgical resection represents the main curative treatment for giant hepatic haemangioma (GHH). The aim of this study was to compare the respective outcomes of hepatic enucleation (HE) and hepatic resection (HR) for GHH. METHODS: Giant hepatic haemangioma was defined as haemangioma of 5-15 cm in size. A prospectively maintained database consisting of a series of consecutive patients who underwent HE or HR of GHH from January 2004 to December 2013 was analysed. RESULTS: Hepatic enucleation was performed in 386 (52.9%) patients and HR in 344 (47.1%) of a final cohort of 730 patients. The median size of GHH was similar in the HR and HE groups (9.8 and 10.6 cm, respectively; P = 0.752). The HE group had a shorter median operative time (150 min versus 240 min; P = 0.034), shorter median hospital stay (5.7 days versus 8.6 days; P < 0.001), lower median blood loss (400 ml versus 860 ml; P < 0.001), and fewer complications (17.6% versus 28.2%; P < 0.001) than the HR group. Quality of life scores in both the HR and HE groups significantly improved compared with preoperative levels and were similar to those found in healthy Chinese individuals following surgery, confirming the efficacy of both treatments. CONCLUSIONS: Hepatic enucleation was associated with favourable operative outcomes compared with HR and is a safe and effective alternative to partial hepatectomy for GHH.

Haemangioma family burden: creation of a specific questionnaire.

To develop and validate a specific questionnaire to assess burden on families of children with infantile haemangioma (IH): the Haemangioma Family Burden questionnaire (HFB). Items were generated from a literature review and a verbatim report from parents. Subsequently, a study was implemented at the Necker Hospital and the Pellegrin Children's Hospital for psychometric analysis. The HFB was refined via item reduction according to inter-question correlations, consensus among experts and exploratory factor analysis. A 20-item questionnaire, grouped into 5 dimensions, was obtained. Construct validity was demonstrated and HFB showed good internal coherence (Cronbach's α: 0.93). The HFB was significantly correlated with the mental dimension of the Short-Form-12 (r = -0.75), and the Psychological General Well-Being Index (r = -0.61). HFB scores differed significantly according to the size and localization of the IH. A validated tool for assessing the burden on families of children with IH is now available.

Social isolation in parents of children with hemangiomas: effects of coping styles and emotional distress.

This study investigated factors associated with social isolation in parents of children with hemangiomas. Eighty-one parents completed questionnaires assessing their emotional distress, social isolation, and coping styles. To explore the relationships between these variables, a path analysis was used to test a model in which clinical characteristics of hemangiomas and parents' coping strategies do not have direct effects on their social isolation but indirect effects via their emotional distress. Bootstrapping was used to assess indirect effects. Time since onset and lesional complications had positive direct effects on parents' social isolation. Lesional visibility and emotion-focused coping had negative indirect effects on parents' social isolation via their emotional distress, while problem-focused coping showed a positive indirect effect. These findings may have implications for clinicians managing parents of children with hemangiomas.

The psychosocial impact of an infantile haemangioma on children and their parents.

Infantile haemangiomas (IHs) are common, benign vascular tumours in children that appear soon after birth and regress before the age of 12 years. Physicians have always been concerned about the considerable psychosocial impact these lesions might have on children and their parents. This is the first critical review of studies on the psychosocial impact of IHs on children and their families. Future directions for research are suggested. As propranolol is becoming the most common first choice treatment for IHs, this article discusses its use in the light of this review.

Hamartoma angiomatoso ecrino: presentación de dos casos / Eccrine Angiomatous Hamartoma: A Report of 2 Cases

El hamartoma angiomatoso ecrino es un tumor benigno e infrecuente que se caracteriza por la proliferación de elementos ecrinos y vasculares. Presentamos dos casos, el de una mujer de 33 años que consultó por una lesión marronácea en la espalda, con hiperhidrosis asociada, y el de un varón de 25 años con una lesión eritematosa, asintomática, en la palma izquierda. Tras el estudio histológico ambos pacientes se diagnosticaron de hamartoma angiomatoso ecrino. El hamartoma angiomatoso ecrino generalmente se presenta al nacimiento o en la infancia. Se manifiesta habitualmente como una lesión única con presentación clínica variable, por lo que su diagnóstico es fundamentalmente histológico. La localización más frecuente es la región distal de las extremidades. Suele ser asintomático, pero puede haber dolor e hiperhidrosis asociados. No requiere tratamiento salvo en aquellos casos asociados a sintomatología recalcitrante, sudor excesivo o por motivos estéticos (AU)

Eccrine angiomatous hamartoma is a rare, benign tumor characterized by a proliferation of eccrine and vascular structures. We present 2 cases and review the characteristics of this disorder. The first patient was a 33-year-old woman who consulted for a brownish lesion on her back associated with local hyperhidrosis. The second patient was a 25-year-old man withan asymptomatic erythematous lesion on his left palm. In both patients a diagnosis of eccrine angiomatous hamartoma was made based on the histological findings. Eccrine angiomatous hamartoma is usually present at birth or develops during childhood. It is typically a solitary lesion and signs and symptoms can vary; diagnosis is therefore based on histological study. The most common site is on the distal parts of limbs. The lesions tend to be asymptomatic, but there may be associated pain and hyperhidrosis. Treatment is not usually necessary except in cases with persistent symptoms, excessive sweating, or cosmetic concern (AU)

Psychosocial adjustment in children and adolescents with a craniofacial anomaly: diagnosis-specific patterns.

OBJECTIVE: To obtain descriptive information about diagnosis-specific patterns of psychosocial adjustment for children and adolescents with craniofacial anomalies. DESIGN: Chart review of medical records was used to obtain psychosocial checklists. Scores were compared with published norms to evaluate elevated risk of problems, separately for six diagnostic groups. SETTING: Plastic surgery department in urban university medical center. PARTICIPANTS: As part of routine ongoing care, 408 caregivers completed checklists assessing psychosocial adjustment of children aged 2 to 18 years with diagnosis of cleft lip and palate, cleft lip only, cleft palate only, craniosynostosis, hemifacial microsomia, and hemangioma. MAIN OUTCOME MEASURE: Child Behavior Checklist ( Achenbach, 1991 , 1992 ). RESULTS: The craniosynostosis group showed elevated rates (beyond norms) of social, academic, and attention problems. The cleft lip and palate group showed elevations in social problems and deficits in social, academic, and activities competencies. The other diagnostic groups had few areas of elevated problems and had reduced risk in some areas. CONCLUSION: Specific patterns of strengths and weaknesses in psychosocial adjustment may vary by craniofacial anomaly diagnosis. Replication of these findings, with extension to other craniofacial anomaly diagnoses, is warranted.