Ichthyosis is a term used to describe continual scaling of the skin. It comes from a Greek word ?reg;ichthys' which means fish. It is quite rare and can be inherited (genetic or congenital) or it can develop later in life (acquired). The inherited forms of ichthyosis are usually evident at, or soon after, birth and they tend to persist throughout life, although some types improve with age. Most, if not all, of the skin is affected. Ichthyosis can cause severe psychological problems. The public's reaction to its appearance is often hostile and unsympathetic. Staring and teasing are common.
Faulty copies of genes, which are passed on from one generation to the next, cause inherited ichthyosis. Each type of ichthyosis is due to a different genetic mutation and the pattern of inheritance varies. Recent research on bullous ichthyosis has led to the discovery of a particular mutation to the gene that controls the skin protein keratin. Other types of ichthyosis have been shown to be related to abnormalities in the lipid or fat chemicals in the skin such as in X-linked ichthyosis.
These can vary greatly between the different types of ichthyosis (see individual types). Darkened and rough skin can appear as scales separated by deep creases. In some cases skin blisters and peels. The main forms of inherited ichthyosis are as follows:
Ichthyosis vulgaris - The most common form and usually quite mild, with fine scaling or roughness on the arms and legs.
X-linked recessive ichthyosis - Occurs in men and boys and causes brownish flat scales mostly to the arms, legs, and tummy. Varies in severity and some affected babies have a difficult delivery. Genetically, it is passed on through the female line.
Non-bullous ichthyosiform erythroderma and lamellar ichthyosis - These are very rare, are often severe, with prominent scaling, and cause redness over most of the skin. Affected babies are often born with a shiny waxy second skin (collodian membrane), which sheds in a few days.
Bullous ichthyosis - This is another rare red ichthyosis, which causes blistering, fragile skin early in infancy and thick scaling especially around the joints later on.
Harlequin ichthyosis - This condition is an extremely rare but very severe form of ichthyosis, which causes thick plates of scale and severe complications at birth. Many affected babies do not survive.
Netherton's syndrome - The affected infant has red inflamed scaly skin from birth, fragile spiky hair and difficulty gaining weight in early childhood.
Sjogren?Larsson syndrome - Another condition that also produces scaling and thickening of the skin which may not be obvious until a few months of age. Affected babies also have a type of cerebral palsy.
Conradi-Hunermann syndrome, neutral lipid storage disease and KID syndrome - These are very rare and are associated with other medical problems.
The milder ichthyoses can be well controlled using regular moisturisers (emollients), including bath oils, soap substitutes and moisturising creams. The more severe forms may make affected babies quite unwell and treatment in special care baby units will give them a good start. Moisturisers are again important as an ongoing treatment and antibiotics may be needed from time to time. A group of drugs called retinoids may be tried as they lessen the scaling in many forms of ichthyosis. The complicated varieties of ichthyosis will need additional treatments according to their severity. As yet there is no cure for ichthyosis.
Joke and Humors
How 1 to 10