Epidermolysis bullosa in three Chinese neonates.

Epidermolysis bullosa (EB) is one of the rare blistering diseases that may present in the neonatal period. Diagnosis is based on clinical symptomatology, histopathology, electron microscopy and genetic studies. Age of onset, symptomatology and prognosis of the various subtypes are varied. We describe three cases of EB dystrophica (EBD) followed at the paediatric dermatology clinic. All of them were symptomatic and diagnosed in the neonatal period. One patient had very severe disease and was confirmed to have the Hallopeau-Siemens subtype with both copies of his entire chromosome three from his mother. This extremely rare mode of inheritance is known as uniparental isodisomy. The clinical course of the other two neonates was milder and suggested that they had the dominant form of EBD. Many issues associated with the disease may be encountered. Specifically, details in skin care, counselling and advice were provided and reinforced to the parents. A multidisciplinary approach with inputs from the neonatologists, paediatricians, dermatologists, plastic surgeons, dietitians, physiotherapists, occupational therapists, and special nurses are required to assure optimal outcome.

Efficacy and problems associated with using a wet-wrap garment for children with severe atopic dermatitis.

Use of a wet wrap for short-term relief of itch in children with severe atopic dermatitis has been advocated but objective determination of its efficacy has been difficult and many issues associated with its use are yet to be defined. We tested a new garment for the wet-wrap procedure in six patients with atopic dermatitis and objectively determined whether a 3-day usage could indeed relieve the distressing symptom of itch using a wrist motion monitor. The garments were effective in the short-term improvement of itching, severity of atopic dermatitis and quality of life in these children. Many issues associated with its use were identified. Clear instructions and individualized regimes (such as the choice of emollient, bathing ointment and topical corticosteroid) are essential for optimal outcome.

Steroid fears in children with eczema.

BACKGROUND: Topical glucocorticoids (GCs) are the mainstay of treatment for eczema, but GC phobia and fears are very common among the parents of paediatric patients. AIM: To survey the nature and extent of "fears" of GC use, and to evaluate if disease severity is associated with such fears. METHODS: Patients with eczema managed in the paediatric dermatology outpatient clinic of a university hospital were recruited in this survey. Disease severity and various aspects of belief and practices of GC use were assessed with the Nottingham Eczema Severity Score and a questionnaire. RESULTS: GC "fears" were present in two fifths of informants with non-eczematous skin disease and mild eczema, but three fifths in moderate-to-severe disease. Requests for steroid-sparing medications (such as tacrolimus or pimecrolimus) had been made in nearly 50% of cases with moderate-to-severe eczema, and many parents would wait until eczema had worsened or apply GC only as a last resort to avoid potential side effects. "Fears" were predominantly interpersonal and rarely iatrogenic in nature. Skin problems (in particular skin thinning) and adverse effects on growth were the side effects of GC of most concern. However, fewer than half of the informants had discussed their concerns with doctors. CONCLUSION: Our results suggest the importance of understanding the nature and extent to which GC fears, regardless of eczema severity, are prevalent so that sound advice can be offered in a timely manner to parents and patients.