Congenital ichthyosis of the neonate

Severe congenital ichthyosis of the neonate

This picture was taken in:Ain shams university O&G hospital on 22/5/08 at

12:30 am

during my night shift in NICU

The ichthyoses are a group of geneticskin diseases caused by an abnormality in skin growth that results in drying and scaling.

There are at least 20 types of ichthyosis. Ichthyosis can be more or less severe,

sometimes accumulating thick scales and cracks that are painful and bleed.

Ichthyosis is not contagious because it is inherited.They are born with dense plate-like scales forming a massive horny shell around their body. These scales or plaques measure up to 4-5 cm on a side and are usually diamond- or triangular-shaped. 'Splits' between these scales reveal erhythematous moist fissures, whose bright red colour is in stark contrast to the lighter-coloured scales. This shell impedes movement, and the fissures cause the protective skin barrier to be compromised, thus leaving the foetus susceptible to infection

The facial features of the afflicted are very severely deformed as a result of the skin disorder.

The eyes, which are most prominent, are red and bulbous, the eyelids turned inside out. Because the foetus's eyes are highly susceptible to trauma, they often bleed upon birth, thus giving them the bulbous red-eye appearance.

The lips, like the eyelids, are turned inside out and pulled by the dry skin into the visage of a clown's smile.

The ears are often very poorly developed with rudimentary auricles (the outer projecting portion of the ear), or they may be absent alltogether.

Nasal hypoplasia4 is also apparent.

Because of the scaling of the skin, the limbs are often deformed to the extent

that movement is restricted, and may be undersized and incompletely formed.

Polydactyly (the condition where one has more than the usual number of digits) has also been observed. The scales may sometimes also constrict blood-flow to the limbs, which results in swelling or in severe cases, gangrene.

Ichthyosis foetalis is very rare, with an incidence of one in 300,000 births. There is no racial or sex predilectionIn general, harlequin foetuses do not survive for very long.

In the past, the life expectancy for a harlequin foetus was about two weeks; however, intensive supportive care and the use of drugs has prolonged the lifespan of these babies and some have made it into adulthood.

However, the average lifespan is much lower, and the babies usually suffer from physical developmental delay, although intellectual development has been reported to be normal.

Death is often due to dehydration, systemic infection or impaired respiration.

Treatment of harlequin babies with a battery of medication ranging from oral retinoids (drugs used in the treatment of psoriasis) to antiseptics and topical paraffin ointments to soften the skin have been reported as successful in

improving the condition of harlequin babies

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This picture was taken by Riham Eldosouki **.