Ichthyosis
vulgaris
Inherited
as an autosomal dominant disorder, this condition is common and affects about 1
person in The relevant gene may be concerned with the production of
profilaggrin, a precursor of filaggrin, itself a component of keratohyalin
granules.
The
dryness is usually mild and symptoms are few. The scales are small and branny,
being most obvious on the limbs and least obvious in the major flexures. The
skin creases of the palm may be accentuated. Keratosis pilaris is often present on the limbs.
The
skin changes are not usually present at birth but develop over the first few
years of life. Some patients improve in adult life, particularly during warm
weather, but the condition seldom clears completely.
The
already dry skin chaps in the winter and is easily irritated by degreasing
agents. This should be taken into account in the choice of a career. Ichthyosis
of this type is apt to appear in a stubborn combination with atopic eczema.
It
can usually be distinguished from less common types of ichthyosis on the basis
of the pattern of inheritance and of the type and distribution of the scaling.
None
are usually needed.
This
is palliative. The dryness can be helped by the regular use of emollients,
which are best applied after a shower or bath. Emulsifying ointment, soft white
paraffin, E45 and unguentum merck are all quite suit-able (Formulary 1) and the
selection depends on the patient’s preference. Many find proprietary bath oils
and creams containing urea or lactic acid helpful also (Formulary 1).
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