NEUROCUTANEOUS DISORDERS IN PEDIATRIC DERMATOLOGY

Jun
2004
Vol. 23. No. 2

Introduction

AS DERMATOLOGISTS, one of the most important things we may do is to detect subtle cutaneous clues that aid in the diagnosis or recognition of significant underlying disease. Pediatric patients are often referred
to dermatologists for evaluations of unusual  “birthmarks” or atypical rashes. In most cases, these are reflections of benign processes that require simple reassurance or straightforward treatment. However, in certain cases, subtle cutaneous signs can herald the presence or onset of significant underlying disease. Dermatologists should be aware of these cutaneous markers, the clinical characteristics that predict higher risk, and the appropriate diagnostic tests and  monitoring required to care for these patients.

Sturge-Weber Syndrome

Eulalia Baselga, MD

Sturge-Weber syndrome is a sporadic neurocutaneous disease characterized by facial port-wine
stain, ocular abnormalities (glaucoma and choroidal hemangioma) and leptomeningeal angioma.

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Incontinentia Pigmenti: A Window to the Role of NF-KB Function

Anna L. Bruckner, MD

Incontinentia pigmenti is an uncommon X-linked dominant genodermatosis primarily affecting females. Its hallmark is a unique skin eruption that presents in infancy along the lines of Blaschko and evolves through four stages: inflammatory, verrucous, hyperpigmented, and atrophic.

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