Sturge-Weber syndrome: an update for the pediatrician.

Background: Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder characterized by the simultaneous presence of both cutaneous and extracutaneous capillary malformations. SWS usually presents as a facial port-wine birthmark, with a varying presence of leptomeningeal capillary malformations and ocular vascular abnormalities. The latter may lead to significant neurological and ocular morbidity such as epilepsy and glaucoma.

Incontinentia pigmenti Stage 1 is not simply vesiculo-bullous but vesiculo-pustular.

Incontinentia pigmenti (IP) is a rare X-linked dominant, male-lethal disorder characterized by pathognomic skin lesions. As described in the literature the typical cutaneous changes follow the pattern of Blaschko's lines and develop in four stages that usually start at birth. Stage 1 is called vesicular, bullous or inflammatory.

Capillary malformations in a child caused by a novel HRAS mutation.

A 6-year-old boy with multiple capillary malformations of the port-wine birthmark (PWB) type on the right leg since birth presented with a varicose vein and segmental overgrowth of the affected leg. Genetic testing on affected skin confirmed the presence of a somatic novel pathogenic HRAS 30 bp in-frame duplication/insertion in the switch II domain. This case illustrates the phenotypic overlap of different genotypes and shows that somatic HRAS pathogenic variants, especially in-frame duplications/insertions, must be added to the list of the underlying causes in capillary malformations.

Transient abdominal telangiectasia of the newborn: Four new cases with abdominal distension.

Transient abdominal telangiectasia of the newborn (TATN) is a recently described entity. The majority of cases had no specific clinical context or trigger identified, but a minority occurred in the clinical context of transient increased intraabdominal pressure. We report four additional cases of TATN, all in the presence of transient abdominal distension, further supporting the causative relationship of abdominal distension to TATN.

Acral manifestations associated with infection.

Acral lesions are well-known physical findings in various infectious disorders. Although they are often overlooked, they can be the key to the diagnosis of the underlying disease. Considering this, we present an overview of various infectious causes of acral lesions in childhood.

Care for children with severe chronic skin diseases.

In this study, the care for children with a severe chronic skin disease in our national expert center of pediatric dermatology was evaluated. Patients and their parents were questioned by using existing questionnaires: 50 pediatric patients completed the modified "my positive health" questionnaire of Huber and 51 parents completed Pelentsov parental needs scale. Nineteen involved professionals answered a questionnaire with open boxes.

Pitfalls in the diagnosis and management of Kawasaki disease: An update for the pediatric dermatologist.

Kawasaki disease is easily diagnosed when it presents in its complete form, but because not all characteristic symptoms are always present at the same time, and the diagnosis of incomplete and atypical Kawasaki disease is often challenging, a delay in diagnosis or misdiagnosis often occurs. We present the diagnostic approach to Kawasaki disease with common pitfalls and explain how to avoid them. We also describe current practice and new trends in treatment.

Infections and skin diseases mimicking diaper dermatitis.

Diaper dermatitis is a common condition that often prompts parents to seek medical attention. Irritant diaper dermatitis is by far the most common cause, but numerous potentially serious diseases can present with changes of the skin in the diaper area. The differential diagnosis can include psoriasis, metabolic disorders, rare immune diseases and infection.

Mastocytosis: a comprehensive insight.

Mastocytosis refers to a heterogeneous group of clinical disorders characterized by an abnormal accumulation of mast cells (MCs) in various tissues. The skin is the organ most frequently involved, but all organs may be affected. The clinical signs and symptoms are produced by the functional effects of mast cell-derived mediators and the anatomical distribution of the mast cells.

The proactive wet-wrap method with diluted corticosteroids versus emollients in children with atopic dermatitis: a prospective, randomized, double-blind, placebo-controlled trial.

Background: Wet-wrap treatment (WWT) has been advocated as a relatively effective treatment in children with severe atopic dermatitis (AD). WWT often serves as crisis intervention for AD.

Objectives: We sought to evaluate the use of WWT with diluted corticosteroids in comparison with emollient in children with severe AD during 4 weeks in a proactive schedule during which the frequency of corticosteroid applications was tapered.

Clinical aspects of diffuse cutaneous mastocytosis in children: two variants.

Objective: This paper describes two different clinical presentations of diffuse cutaneous mastocytosis (DCM), based on the largest series published to date. As far as we are aware, these two variants of clinical presentations have not yet been reported.

Design: We undertook a case controlled analysis of 8 children with DCM.

Messages from the Aalst Allergy Study.

Background: The prevalence of sensitization and allergic disease has increased significantly worldwide. The aim of the "Aalst Allergy Study" was to document prevalences of sensitization and allergic symptoms, and to evaluate the effect of personal and environmental influences on these prevalences in an unbiased Belgian pediatric population.

Methods: A cross-sectional study was performed in an unbiased population of 2021 Belgian schoolchildren (3.

The prevalence, characteristics of and risk factors for eczema in Belgian schoolchildren.

Childhood eczema is common in infants, but its nature and extent during later childhood remains unclear. In this cross-sectional study we examined the prevalence and characteristics of eczema in an unbiased community population of 2,021 Belgian schoolchildren, aged 3.4 to 14.