What is Harlequin Ichthyosis?

Ichthyosis is an inherited disorder of the skin. If both parents carry the gene, it’s very bad for their baby. If only one parent carries the gene, science tells us it can be handled.

It begins before birth in many cases, and the baby is born with thick dry scales all over the body. There are holes where the ears, eyes, nose, and mouth should be. The scales prevent the infant’s body to sweat or even breathe properly causing death to occur after a few days.

What Makes It Harlequin Ichthyosis?

The harlequin is a character in the 1580 Italian Commedia dell’arte. His costume is covered in diamond shaped materials. What makes the disease Harlequin ichthyosis is the diamond shaped scales all over the body.

How Did The Disease Begin?

The Reverend Oliver Hart from Charleston, South Carolina found the first case of the disease on April 5, 1750. His parishioner, Mary Evans, had borne an infant covered in diamond shaped scales with holes for eyes, nose, mouth, and ears. The baby passed two days later.

Those with this disease are often dehydrated and suffer from hypothermia, because the skin’s scales fail to keep heat and water in the body. The scales also often prevent the infant from breathing properly due to the chest being unable to expand and contract. Thus, infants usually die after a couple days.

Is Harlequin Ichthyosis Curable?

While the disease isn’t curable, it is manageable. Skin care and hydration become vital to keeping the infant alive. Antibiotics will keep infection at bay.

Retinoids will help slough off scaly skin. Wrapping the infant in bandages is a further barrier to infection. Eye drops or protective masks help baby’s eyes function normally.

Our skin is what protects us from outside forces trying to make us ill. The skin must be kept viable and healthy for us to remain healthy. That’s why management of this skin disease is so vitally important.

Skin lotions rich in moisturizers like lanolin, ceramides, and glycerine should be massaged into baby’s skin daily. Oral retinoids help to shed thick skin. Consult a doctor or specialist for other management ideas.