Skin Conditions

Skin Conditions

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Aneurocutaneous syndrome of streaky, patchy, whorl-like, or linear macular hypopigmentation of the skin, often associated with seizures, developmental and intellectual retardation, and other anomalies. Dr Ito first wrote in a Japanese medical journal in 1952 about some patients he had seen who had a pattern of skin markings in a V shape over the spine and in lines down the arms and legs. Over the years other doctors have written in medical journals describing one or two patients with the same skin markings. Some of the patients have had problems with development. Until recently no one knew the cause of Ito's disease but the results of research carried out in Manchester have been confirmed by other studies in the USA.

Differential Diagnosis
Ectodermal Dysplasias, especially Naegeli Type; Incontinentia Pigmenti (Bloch-Sulzberger Syndrome); Systematized Nevus Depigmentosus; Tuberous Sclerosis; carbohydrate-deficient glycoprotein deficiency Type III.

The body is made up of millions of cells usually each one has the same genetic instructions as each other (chromosomes and genes). In Ito's disease there are two groups of cells, one with one set of genetic instructions and another with a slightly different set of instructions. It is the upset in “balance” between the two sets, which causes the problem.

In the type with the extra chromosomes, usually the egg and the sperm are normal when they join together. The fertilised egg then divides and it is at this division or one of the following divisions that the chromosomes do not divide equally giving rise to a set of cells with an extra chromosome.

In the type with the gene variation the egg and sperm contain normal gene instructions and when the fertilised egg divides a gene fault occurs in one of the cells and thereafter all the cells resulting from that cell contain the faulty gene.

The above problems just happen by chance and are not caused by anything the parents have done and are not usually inherited.

These include asymmetry of the body, patchy pigmentation which can occur on any part of the body, but not normally the palms, scalp or soles of the feet. Gross motor and psychomotor retardation, epilepsy, scoliosis, dental and bone anomalies, unusual ophthalmic features and kidney problems, amongst others. How a child is affected depends partly on the particular chromosome or gene fault and partly on how many cells contain the fault. The sort of problems children with Ito's disease have can be divided into two types: Physical and Developmental. Not all children have all of the problems.

Physical problems
These include problems obvious at birth such as unusual shape of joints, extra fingers, etc. Many children with Ito's disease have more growth on one side of the body than the other making them a little asymmetrical. The skin patterns are usually first noted when a child is a few months old.

Developmental problems
Some children with Ito's disease make very good progress and others are slow with their development. There is no way other than careful follow-up to predict how a particular child will develop. Even those children with problems with development do continue to grow and make progress although slower than other children. Extra help in nursery and school may be necessary.

Since there is no cure for Ito's disease, treatment has to be symptomatic. Epilepsy is treated with drugs (or occasionally surgery); Behavioural difficulties (including hyperactivity and autistic features) are often the most troublesome for families and frequently require appropriate intervention. Scoliosis (curvature of the spine) can be helped by the wearing of a brace and in some cases surgery. There is no treatment for the depigmentation markings of the skin

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