Skin lesions in children admitted to the paediatric intensive care unit: an observational study.

We analysed, by a prospective observational study over a 3-year period, the frequency and character of dermatological symptoms and diseases in children admitted to a tertiary general paediatric intensive care unit (PICU) of a university hospital. Skin problems were observed in 42 of 1,800 children admitted. There was a large variability in dermatological diseases in the PICU. In the majority (23/42), the skin problem was a dermatological manifestation of an underlying illness that caused admission to the PICU, e.g. infection, vasculitis or drug reaction. In four of the seven children who died, a direct relation between the dermatological disease and the cause of death could be established. Although the number of patients with relevant dermatological problems was limited, we believe that a skilled paediatric dermatologist is able to contribute to the effectiveness of diagnostic and therapeutic processes in paediatric intensive care patients.

A proposal for classification of entities combining vascular malformations and deregulated growth.

BACKGROUND: Agreement on terminology and nomenclature is fundamental and essential for effective exchange of information between clinicians and researchers. An adequate terminology to describe all patients showing vascular malformations combined with deregulated growth is at present not available. OBJECTIVES: To propose a classification of patients with vascular malformations, not restricted to the face, and growth disturbances based on simple, clinically visible characteristics, on which clinicians and researchers can comment and which should eventually lead to an internationally accepted classification. METHODS: Rooted in our joint experience we established a classification of vascular malformation not limited to the face, with growth disturbances. It is based on the nature and localization of the vascular malformations; the nature, localization and timing of growth disturbances; the nature of co-localization of the vascular malformations and growth disturbances; the presence or absence of other features. Subsequently a mixed (experienced and non-experienced) group of observers evaluated 146 patients (106 from the Netherlands; 40 from the UK) with vascular malformations and disturbed growth, using the classification. Inter-observer variability was assessed by estimating the Intra-Class Correlation (ICC) coefficient and its 95% confidence interval. RESULTS: We defined 6 subgroups within the group of entities with vascular malformation-deregulated growth. Scoring the patients using the proposed classification yielded a high inter-observer reproducibility (ICC varying between 0.747 and 0.895 for all levels of flow). CONCLUSIONS: The presently proposed classification was found to be reliable and easy to use for patients with vascular malformations with growth disturbances. We invite both clinicians and researchers to comment on the classification, in order to improve it further. This way we may obtain our final aim of an internationally accepted classification of patients, which should facilitate both clinical treatment and care of, as well as research into the molecular background of entities combining vascular malformation and deregulated growth.

Baseline Quality of Life in patients with Klippel-Trenaunay syndrome.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a congenital group of disorders characterised by vascular malformations (capillary malformation (CM), venous malformation (VM), and lymphatic malformation (LM)) and disturbed growth regulation. The burden caused by KTS symptoms can be evaluated using Quality of Life (QoL)-measuring questionnaires. This study aimed to assess the QoL in KTS patients using the Short Form Health Survey Questionnaire (SF-36) and Skindex-29 questionnaires, and to determine three grades of severity (mild, moderate and severe) according to the scores obtained. In addition, we compared the SF-36 results to those of a general Dutch population sample and a selected group of other chronic conditions. METHODS: KTS patients of the Dutch KTS foundation and of two medical centres answered SF-36 and Skindex-29 questionnaires. Control data of validated Dutch population SF-36 scores and literature-acquired scores for other diseases were available. RESULTS: A total of 78 patients were enrolled, of whom 34 (43.6%) were male; the mean age was 39.3 years (SD: 17.1; range: 12-78 years). The Dutch KTS group scored significantly lower than the general Dutch population on all SF-36 scales except Mental Health and Role Emotional. Furthermore, they scored significantly lower than other medical conditions on the Physical Functioning and Bodily Pain scales. According to the Skindex-29 results, KTS patients fall in the categories - symptoms: severe to very severe; emotions: diminutive to mild and functions: mild. The total score is lower than 40, indicating a negligible negative impact on QoL; however, new cut-off values are being calculated. CONCLUSIONS: Classification according to severity is important to educate patients accordingly, predict prognosis and set treatments. Especially in cases of severe KTS, physicians should not only be attentive to the physical aspects but also to the psychological and social aspects of KTS.

The course of life of patients with childhood atopic dermatitis.

Atopic dermatitis mainly covers the period of infancy to adulthood, an important period in the development of an individual. The impairment of quality of life and the psychological wellbeing of children with atopic dermatitis have been well documented but so far no data exist about the impact of atopic dermatitis in childhood on fulfilling age-specific developmental tasks and achieving developmental milestones during this period, referred to as the course of life. The aims of this study were to: (i) assess the course of life and define the disease-related consequences in young adult patients with childhood atopic dermatitis and (ii) determine whether the severity of atopic dermatitis is predictive for the course of life, the disease-related consequences and quality of life later in life. Adult patients who grew up with atopic dermatitis were asked to complete a medical history questionnaire, the Skindex-29, the "course of life" questionnaire and a subjective disease-specific questionnaire. Patients with severe atopic dermatitis in childhood showed a significant delayed social development in their course of life. The results of the disease-specific questionnaire demonstrated remarkable high percentages of psycho-social consequences and physical discomfort caused by atopic dermatitis in childhood. Patients showed a severely negative impact of atopic dermatitis on their current quality of life. This is the first study that applied the "course of life" questionnaire in atopic dermatitis. More insight in the course of life, disease-specific consequences and quality of life of atopic dermatitis is of high importance, especially in case of severe atopic dermatitis.

The skin in primary immunodeficiency disorders.

Primary immunodeficiency disorders comprise serious and rare diseases, predominantly in children. The skin may be involved in a primary immunodeficiency and the cutaneous alterations such as infections, eczematous dermatitis, erythroderma, autoimmune dermatoses and vasculitis may be the basis for the ultimate diagnosis. Dermatologists may realize something is wrong with the immunity of their patient and prompt diagnosis of the defect can lead to lifesaving treatment. Since atypical presentations of well known dermatoses are common in immunodeficiency disorders, histopathology and bacterial, fungal or viral tests of the skin lesions are often indispensable. The knowledge of skin findings in primary immunodeficiency diseases may help dermatologists to play a role in the early detection of these diseases. Key findings constitute specific skin symptoms combined with unusual or severe infections.