Ichthyosis

Icht1
Icht2

What is it?
This is a long term condition that results in persistently thick, dry, ‘fish scale’ skin. It comes from the Greek word ‘ichthys’ which means fish.  It is a rare condition and can be inherited (genetic or congenital) or it may develop later in life (acquired). The inherited forms are usually evident at or soon after birth and they tend to persist throughout life although some types will improve with age.

What causes it?
Most people with ichthyosis would have inherited a particular faulty gene from their parents. This faulty gene affects the rate at which their skin regenerates – either the shedding of old skin is too slow or the skin cells reproduce at a much faster rate than they shed old skin. Either way this causes a build up of rough dry scaly skin.

Symptoms appear within the first year of life.

Forms of inherited ichthyoses are listed below:

Ichthyosis vulgaris
A common condition that affects around 1 in 300 people. The dryness is usually mild and symptoms are few. The scales are small and fine being most obvious in the major flexures. The skin creases in the palm may be accentuated. Keratosis pilaris is often present on the limbs. It is more common in people with atopic eczema and psoriasis and improves with warm weather.

Bullous ichthyosis
This is rare, red and blistering. The skin is fragile in early infancy with thick scaling especially around joint.

Lamella ichthyosis
This is a condition where the baby is covered in a thick membrane at birth which is then shed. Scaling affects the body and the child may also have drooping eyelids.

Epidermolytic  ichthyosis
Skin is moist, red and tender at birth with blistering. Thick scaling occurs as the baby gets older and the thickened dead skin can produce a foul smelling odour.

X-linked ichthyosis scaling is present at or shortly after birth and only affects males.  It maybe associated with testicular acquired ichthyosis which tends to develop in adulthood and is normally linked to an underlying disease such as :

  • an underactive throid
  • kidney disease
  • sarcoidosis  (a rare disease that causes clumps of cells that form in the organs)
  • lymphoma (type of cancer)
  • HIV infection

Complications
Overheating can occur due to a reduced ability to sweat. There maybe limited movement as dry skin makes it too painful to move certain parts of the body. Skin infection can also occur after cracking and splitting. The patient may also have impaired hearing or eyesight as the skin may build up over the ears or eyes.

What is the treatment?
There is no cure for ichthyosis so treatment is focused on managing the disease. This involves moisturising and exfoliating the skin.

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