The skin may be pale and transparent with veins clearly visible and there is often increased pigmentation around the eyes or on the elbows, palms and soles of the feet. The heavily pigmented skin around the eyes may be wrinkled and that on the palms and soles thick. Very dry skin is usually a problem. There is a wide range of products available to help prevent and treat dry skin, but it may be best to avoid very greasy products in hot weather. Neither soap nor any perfumed bath additives, including baby bath products, should be used as these all have a drying effect and may cause irritation to sensitive skin. Always pat the skin dry rather than rub and apply cream immediately. In a large number of cases eczema is a problem and for some it is extreme; this must be treated by your Doctor or you may be referred to a Dermatologist. If persistent problems occur an allergy test may be offered. You should never try alternative remedies yourself without getting good professional advice.

During the summer months a high factor sun block should be used at all times, preferably one for sensitive skin. However, a heavy suncream may make the individual hot, so a lighter cream may be more suitable. Cotton clothes next to the skin and cotton bedding are advisable.

In the Ectodermal Syndrome known as Hay-Wells (AEC) the skin is more severely affected, such that infants may present with chronic wounds complicated by life-threatening infections requiring the care of a Doctor to relieve discomfort, prevent secondary infection and minimise scarring. Many families in this situation have tried various products but have found that persisting with salt water bathing is the best treatment.

“Working Together at last for Rare Diseases: ERN Skin”

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