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单词 Ichthyosis
释义
Ichthyosis

A disorder in which the skin is permanently dry and scaly. It is usually genetically determined and several different forms are recognised:

Ichthyosis vulgaris

Common and inherited as a dominant trait. Beginning in early childhood, it is often associated with atopic eczema (see DERMATITIS). The limb flexures and face are spared.

X-linked ichthyosis

is much less common, more severe and appears earlier than ichthyosis vulgaris. The fish-like scales are larger and darker and do not spare the flexures and face.

Ichthyosiform erythroderma

Of two types and very rare: in the recessive form, the appearance at birth is of the so-called ‘collodion baby’; in the dominant form the baby is born with universally red, moist and eroded skin with an unpleasant smell. Gradually, over several months, thick scales replace the ERYTHEMA.

Treatment

Minor forms are helped by constant use of EMOLLIENTS and moisturising applications. Cream containing UREA can be valuable. The rare erythrodermic patterns in the neonate require skilled intensive care as thermoregulation is disturbed and massive fluid loss occurs through the skin. Later in childhood, oral RETINOIDS are useful.

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更新时间:2025/4/22 8:41:29