What is the scaly skin disease ichthyosis?

Condition analysis:
Ichthyosis is a group of more than two dozen skin diseases that vary in severity. Most are mild inconveniences, and some are life-threatening.

They have in common that the skin is thickened with scaly patches, especially on the shins, the forearms and upper arms, and the palms of the hands and the bottoms of the feet. The skin of the fingers is dry and bleeds easily. Opposing skin in the armpits, the groin, the elbow and the knee is usually not effected.

There are huge psychological problems due to appearance. Patients have issues with self-image and may withdraw.

People with the severe forms of the disease are prone to skin infection. Some of these skin infections are actually treated in burn units of hospitals due to their seriousness.

Most cases of ichthyosis are genetic in that a parent with the disease can pass it on to their children. It is not uncommon for a parent to have mild ichthyosis and the child to have more severe disease. Most children with icthyosis are born without evidence of the disease and develop symptoms in later childhood.

Some forms of ichthyosis are also more severe in childhood, improve in young adulthood and worsen in old age. The disease can wax and wane throughout life.

It is also interesting that many, perhaps most, patients with this condition also have skin allergies, eczema and/or asthma. The patient with moderate to severe icthyosis often has problems sweating due to the buildup of scales on the skin. The scales essentially seal the sweat glands. This can cause a tremendous itch known as "prickly itch."

Patients can also have serious sensitivity to heat. The most effective treatments are exfoliating chemicals to remove the scales and allow the skin underneath to function normally.

These exfoliating drugs often must be applied several times a day, and treatment may continue for years. The itch can be treated with Benadryl creams and topical steroids.

Instructions:
Surgical Care: When cicatricial ectropion develops in patients with lamellar ichthyosis despite room humidification and vigorous skin lubrication, the danger of corneal breakdown and perforation is noted. Full-thickness skin grafts from the forearm, postauricular, and groin areas may be used to successfully repair the abnormalities. [7] In addition, Apligraf (Organogenesis Inc, Canton, Mass), a human skin equivalent, may facilitate the repair of cicatricial ectropion in severe cases when autologous donor graft tissue is not available. A concomitant medial and/or lateral lid tarsorrhaphy is recommended in severe cases. The incidence of ectropion recurrence may be decreased if surgery can be postponed until suitable nonscaly patches of skin can be clearly identified to serve as graft donor sites.

For a persistent corneal epithelial defect, an amniotic membrane transplantation may be necessary to promote epithelial wound healing.