Bullous Ichthyosis


Bullous Ichthyosis, also called bullous ichthyosiform erythroderma (BIE) or epidermolytic hyperkeratosis (EHK), is a rare genetic skin disorder affecting less than 1 in 100,000. It is characterised by blisters, skin fragility and ichthyosis (continual scaling of the skin).


From birth the skin is noted to be fragile with blisters and peeling. Often there is no evidence of ichthyosis at birth and the skin appears red with superficial erosions. From early childhood the skin becomes more scaly and the redness and blistering less noticeable. The skin thickening can affect any pad of the body but is most prominent on the scalp, around the neck and in the skin creases of the armpits, elbows and knees. Many patients with this condition develop thickening of the skin of the palms and soles. Older children and adults suffer from repeated skin infections especially in the skin folds.

Source: Ichthyosis Support Group


Bullous ichthyosis is caused by an abnormality of one of the many proteins in the skin, keratin. There are a number of different types of keratin and this condition is related to an abnormality in keratins 1 and 10. The specific gene site has been identified.

Source: Ichthyosis Support Group


Epidermolytic hyperkeratosis tends to improve with increasing age.

Source: Medscape


No reported cure or specific therapy is available for Bullous Ichthyosis; however, reports of improvement have been noted with high-dose beta-carotene, systemic retinoids, topical retinoids, 10% glycerin, lactic acid, alpha-hydroxy acid, calcipotriol, antibacterial soap, and urea.

Source: Medscape


Moisturising creams and a bath oil. It may be helpful to use a bath oil with an antiseptic from time to time.

Antibiotics when necessary.

Metronidazole gel is often used for weeping skin in the flexures to treat ”anaerobic” infections.

Acitretin or neotigason is a retinoid drug taken by mouth which is related to vitamin A and can be very helpful for this condition. It is best given at a low dose. It helps by lessening the thickened skin, but care needs to be taken in getting the right dose for a particular patient as it can sometimes worsen the blistering.

Source: Ichthyosis Support Group