Predictors of Health-related Quality of Life and Psychological Adjustment in Children and Adolescents With Congenital Melanocytic Nevi: Analysis of Parent Reports.

Objectives This cross-sectional study assessed health-related quality of life (HRQOL) and psychological adjustment in children and adolescents affected by congenital melanocytic nevi (CMN) and identified potential predictors of adjustment. Methods Participants were recruited worldwide with the help of patient organizations. Data were obtained from parents of 235 children affected by CMN, aged between 1 month and 18 years (M = 6.3 y; SD = 5.0 y), using a web-based survey. Measures included the Pediatric Quality of Life InventoryTM 4.0 and the Strengths and Difficulties Questionnaire. Sample scores were compared to normative data. Demographic characteristics as well as CMN-related variables were examined as possible predictors of outcome, using multivariate analyses. Results Parents of children and adolescents born with a CMN reported significantly lower HRQOL and somewhat higher emotional and behavioral problems compared to community norms. Impairments in HRQOL and psychological adjustment were predicted by lower socioeconomic status, neurological problems, skin-related discomfort (e.g., itch or pain), and perceived stigmatization. The size of the CMN and whether or not the CMN had been (partially) removed by surgery were no significant predictors. The relationship between visibility of the skin lesion and psychological adjustment and psychosocial health was found to be mediated by perceived stigmatization. Conclusions In children and adolescents affected by CMN, those experiencing neurological problems, skin-related discomfort or high levels of perceived stigmatization are particularly vulnerable for impaired HRQOL and psychological maladjustment and therefore might need special monitoring and support.

Patients with Hidradenitis Suppurativa Have a High Psychiatric Disease Burden: A Finnish Nationwide Registry Study.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease of hair follicles that is associated with various comorbidities. To further clarify the associations between HS and psychiatric disorders, we conducted a nationwide retrospective study that included 4,381 patients with HS, and 39,554 with psoriasis and 43,248 with melanocytic nevi as controls. Patient data were obtained from the statutory Finnish Care Register for Health Care. The incidence of HS was 3.0/100,000 persons/year. At least one psychiatric diagnosis was found in 24.1% of the patients with HS compared with 19.1% of the patients with psoriasis (odds ratio 1.34; 95% confidence interval 1.24-1.46) and 13.5% of those with melanocytic nevi (odds ratio 2.04; confidence interval 1.88-2.22). Every mental disorder examined was significantly more frequent in HS than in the two other groups. Mental disorders were more common in women than in men with HS and psoriasis. The main finding of our study is that patients with HS have a higher risk of mental disorders than patients with psoriasis. Additionally, this study reveals that HS is associated with both schizophrenia (odds ratio 1.57; confidence interval 1.24-1.98) and bipolar disorder (odds ratio 1.81; confidence interval 1.47-2.23), findings that to our knowledge have not been reported previously.

Clinical course, treatment modalities, and quality of life in patients with congenital melanocytic nevi - data from the German CMN registry.

BACKGROUND: Congenital melanocytic nevi (CMN) are associated with mental stress as well as medical risks for those affected. The German CMN registry was initiated in 2005. Herein, we present results from an interim analysis focusing on disease course, treatment modalities, and quality of life. PATIENTS AND METHODS: One hundred patients enrolled in the registry between 2005 and 2012 were included in this prospective cohort study, and asked to participate in a follow-up survey. In addition, standardized questionnaires were used to collect data on quality of life (dermatology life quality index, DLQI) and perceived stigmatization (perceived stigmatization questionnaire, PSQ; social comfort questionnaire, SCQ). RESULTS: Eighty-three percent of patients (or their parents) provided answers to the survey questions (mean patient age: 11.2 years, median: 6 years; mean follow-up: 4.4 years). Overall, four individuals were diagnosed with melanoma, including two pediatric cases with CNS melanoma, one adult with cutaneous melanoma, and one case which later turned out to be a proliferative nodule. Four children were diagnosed with neurocutaneous melanocytosis, three of whom exhibited neurological symptoms. Eighty-eight percent (73/83) of patients underwent surgery. Seventy-eight percent reported no or only minor impact of the CMN on quality of life. In general, perceived stigmatization and impairment of social well-being were also low. CONCLUSIONS: Our results provide an overview of the situation of CMN patients in Germany, Austria, and Switzerland. Three percent of patients developed melanoma; 4 % showed CNS involvement.

Higher psychological and psychovegetative strain in adolescents with atypical pigment naevi.

An observational, exploratory, cross-sectional study was performed to assess whether the presence of atypical naevi (AN) in adolescents is associated with psychological and psychovegetative stress parameters. Fifty-one students of a secondary school in Graz, Austria, completed a defined test procedure consisting of an initial period of rest, a standardised mental stress task, another period rest and a questionnaire, the change-sensitive symptom list (ASS-SYM). Electrocardiogram and blood pressure were recorded continuously. The study population was divided in two groups: probands without AN (NAN, n = 33), and probands with at least one AN (n = 18). We found higher values for the AN group in all scales of ASS-SYM, reaching statistical significance in the dimensions "nervousness and mental tension" (p = 0.025), "psychophysiological dysregulation" (p = 0.020), burden of pain" (p = 0.023) and "general symptoms and problems" (p = 0.031). Regarding physiological parameters, the AN group showed higher vegetative strain reflected in heart rate and heart rate varibility during the periods of rest as well as a reduced baroreceptor sensitivity. On the basis of our results, the presence of AN in adolescents seems to be associated with a higher vegetative arousal. Additionally, participants with AN complained significantly more often about stress-associated general psychological symptoms and problems.

The eleventh reported case of Mulvihill-Smith syndrome in the literature.

BACKGROUND: The Mulvihill-Smith Syndrome was first recognized in 1975. After the recognition of the Mulvihill-Smith Syndrome, ten cases have been described. CASE PRESENTATION: This article describes the eleventh case of this syndrome in a male patient, 24 years-old with short stature and microcephaly with mild cognitive impairment, deafness and allergic conjunctivitis. The patient was hospitalized several times for repeated infections, and the presence of multiple melanocytic nevi on his skin was noticed. CONCLUSIONS: Based on the entire set of signs and symptoms presented in our study, it was diagnosed the patient with Mulvihill-Smith Syndrome.

Stigmatization predicts psychological adjustment and quality of life in children and adolescents with a facial difference.

OBJECTIVES: This cross-sectional study assessed psychological adjustment and health-related quality of life (HRQOL) in children and adolescents with congenital or acquired facial differences and identified potential predictors of adjustment. METHODS: Data were obtained from 88 children, ages 9 months to 16 years, by means of parent questionnaires (n = 86) and standardized interviews with children ≥7 years old (n = 31). Evaluation measures included the Child Behavior Checklist (CBCL), KIDSCREEN-27, TNO-AZL Preschool Quality of Life Questionnaire (TAPQOL), and Perceived Stigmatization Questionnaire. RESULTS: Psychological adjustment, as measured by the CBCL, was within norms. Parent-reported HRQOL was good in preschool children. Parent- and self-reported HRQOL of participants 7-16 years old was impaired in several dimensions, including psychological well-being. Psychological adjustment (especially internalizing behavior problems) and HRQOL were predicted primarily by perceived stigmatization. CONCLUSIONS: Identification of stigma experiences and appropriate support may be crucial to enhancing psychological adjustment and quality of life in children with facial disfigurement.

Large congenital melanocytic nevi: associated risks and management considerations.

Large congenital melanocytic nevi (LCMN) in neonates can cause considerable concern for parents, family members, and physicians. A detailed understanding of the medical risks, including cutaneous melanoma (CM), extracutaneous melanoma (ECM), and neurocutaneous melanocytosis (NCM), as well as the psychological stress that these lesions can cause in patients, will guide informed management decisions as well as provide comfort to parents. Current data indicate that LCMN greater than 20 cm, and more likely greater than 40 to 60 cm, are the lesions at greatest risk for complications such as CM, ECM, and NCM. Additionally, lesions on the trunk are at greater risk for developing CM, and LCMN in association with numerous satellite nevi are at greatest risk for NCM. Individualized management plans, including clinical observation, magnetic resonance imaging (MRI), and possibly surgery should be based on the risk versus benefit ratio, taking into account the size of the LCMN, its location, the number of satellite nevi, symptoms, and numerous other factors which will be reviewed. This paper will provide a detailed analysis of the risks associated with LCMN, as well as a discussion regarding management and treatment options.

Knowledge and perception of melanocytic nevi and sunburn in young children.

BACKGROUND/OBJECTIVES: Understanding the public's perception of nevi and sunburn is crucial to melanoma prevention efforts. METHODS: We investigated the knowledge and perception of melanocytic nevi and sunburns in 77 children 6 to 10 years old (mean 8.2) in two elementary schools in Styria, Austria. The children were interviewed by specially trained psychologists about the number of their moles and how they felt having them. Additionally questions about sunburn history and sunburn perception were asked. The spontaneous answers of the children were recorded, there were no pregiven answers. Afterwards the children were examined by dermatologists clinically and with dermatoscopes. RESULTS: The 96% of the children could describe a nevus (the term "mole" was translated to "nevus") and 91% did not feel bothered about theirs. Only 26% had noted the appearance of new nevi within the last year. The 67% of all children had at least one sunburn and remembered the clinical features. The 20% of the children knew that sunburns could provoke skin cancer. All children felt comfortable during the clinical and dermatoscopic examination. CONCLUSION: Children aged from 6 to 10 years know exactly why they had suffered from sunburn, can describe the sunburn and how to avoid it. They do not feel bothered by their nevi and are alert to the appearance of new nevi.

Mental health in patients with systemic sclerosis: a controlled investigation.

BACKGROUND: Despite the undeniable impact of systemic sclerosis (SS) on quality of life, only a few studies so far have focused on its psychiatric or psychological aspects. We aimed at assessing psychiatric symptoms and self-image in inpatients with SS and comparing them with patients with either a very mild skin condition or a serious skin condition. METHODS: Three groups were recruited: (i) 38 consecutive female inpatients with SS; (ii) 38 age-matched female outpatients with melanocytic naevi; (iii) 35 age-matched female inpatients with melanoma. All participants completed the Zung Anxiety Scale, the Zung Depression Scale and a self-report questionnaire measuring self-perceived personal qualities. Patients with SS were also clinically interviewed by a psychologist. RESULTS: The clinical interview revealed the presence of a psychiatric disorder in most (81%) patients with SS. The Zung scales corroborated the presence of mild to moderate anxiety and depression among patients with SS, who scored significantly higher than patients with either naevi or melanoma on both scales. Scores on the questionnaire assessing self-perceived personal qualities were very similar in the three groups and indicated a fairly high level of self-esteem. CONCLUSIONS: This study suggested that psychosocial issues are quite relevant in patients with SS and underscored the need for a biopsychosocial approach to the clinical management of these patients. Timely detection of psychosocial difficulties and appropriate psychological or psychiatric intervention may represent important steps toward better adherence to medical treatment and improved psychological well-being and quality of life.

Congenital nevus: the Indiana University's approach to treatment.

The article will review the different treatment modalities for congenital nevi and the senior author's approach to these lesions. The management of congenital melanocytic nevus presents a diagnostic dilemma. Treatment must weigh the risk of malignancy, psychological distress to parents and child, and potential morbidities of excision. Excision of every nevus is neither applicable nor practical. Most plastic surgeons would agree that a large nevus mandates excision because of the risk of malignancy. However, debates occur regarding treatment of small and medium size nevi. Opinions differ regarding the types of reconstruction, malignant potential, and psychological impact of leaving a lesion untreated.

Psychosocial sequelae in 29 children with giant congenital melanocytic naevi.

Giant congenital melanocytic naevus (GCMN) may be expected to affect psychosocial functioning of children and their parents due to deviant appearance and painful treatment. To obtain insight into clinical aspects and psychosocial functioning of those suffering from GCMN, 29 children diagnosed with GCMN syndrome or single GCMN received a dermatological examination, were interviewed, and their mothers and teachers completed standardized questionnaires on the child's competence and behavioural/emotional problems and their own adjustment. Social problems were reported for 30% of the patients and behavioural/emotional problems for 25.9%. There was no correlation between visibility of the naevus, treatment or child age and psychological problems. Mothers reported considerable psychosocial burden. It is concluded that children with GCMN are at increased risk of social and behavioural/emotional problems, and mothers suffer considerable psychological impact of their child's condition.

'Naevophobia'.

In recent years there has been intensive media interest in pigmented lesions and several skin cancer prevention/early detection campaigns. Pigmented lesions may cause great anxiety, although patients are usually reassured once their moles have been examined by a dermatologist. We describe a case in which a young man with multiple pigmented naevi attempted to remove all the lesions himself. This self-mutilation occurred despite his having attended a dermatology clinic.

Congenital giant pigmented nevi.

A congenital giant pigmented nevus is a rare skin condition which can involve nearly the entire skin surface. The exact lifetime risk of melanoma developing in a person with a giant nevus is unknown, but is approximated at 6%-12%.

Public knowledge about characteristics of moles and melanomas.

In a study aiming to determine public knowledge about moles and melanomas 590 residents of Victoria aged 14 and over were interviewed in a face-to-face household survey. The majority believed that moles were primarily raised lesions. Recognition of the term melanoma was high (91 per cent), but many held misconceptions about key characteristics. Many thought an early melanoma was raised (20 per cent) or could be raised (42 per cent), compared with only 10 per cent who thought it was flat. Fifty per cent of respondents thought being ugly was a common characteristic. In response to photographs of skin lesions, a late melanoma, seborrhoeic keratosis and a squamous cell carcinoma were most likely to be identified as needing to be seen by a doctor, while a normal raised mole and two early melanomas were least likely. The data suggest that a large proportion of the community have misconceptions about the early signs of melanoma. Education programs are needed to inform the public that most early melanomas are flat. Strategies which might confuse this message, such as likening melanoma to moles, should be avoided.