Ichthyosis is a term used to describe continual scaling of the skin. It comes from a Greek word "ichthys" which means fish although not all affected people have fish-like scales. It is quite rare and can be inherited (genetic or congenital) or develop later in life (acquired). The inherited forms of ichthyoses are usually evident at or soon after birth and they tend to persist throughout life although many types improve with age.
The scaling of ichthyosis affects most if not all areas of the skin and is fairly consistent over the years. This is in contrast to other skin disorders such as eczema and psoriasis where scaling affects limited areas of the skin and changes its pattern frequently. However the more common, milder forms of ichthyosis do generally improve in summertime. Several of the rarer types of inherited ichthyosis also cause red inflamed skin and one form produces blistering of the skin in babies.
Harlequin ichthyosis (HI) — the most severe form of keratinizing disorders, often lethal in the neonatal period — is characterized by a profound thickening of the keratin skin layer, a dense "armour"-like scale that covers the body, and contraction abnormalities of the eyes, ears, and mouth.
Harlequin ichthyosis (HI) is inherited, and affected newborn infants are encased in "armour"-like thick, yellow plates of scales with deep red fissuring. The skin is pulled tight such that the face loses its normal appearance and appears frog-like or scaly, stretching the eyelids and lips and flattening of the ears and nose. The extremities are swollen due to constriction by massive thickening of the skin. Liveborn infants usually die within the first days of life from respiratory, infectious, and/or dehydration-related complications.
http://www.ichthyosis.org.uk/condition.aspx |
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| There are a number of rare genetic diseases which affect several systems of the body and also cause ichthyosis, these are referred to as ichthyosiform syndromes. The main forms of inherited ichthyosis are as follows:- |
| Ichthyosis vulgaris |
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| The most common form and usually quite mild with fine scaling or roughness on the arms and legs. It is more common in people with atopic (allergic) eczema and improves in warm weather |
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| X-linked recessive ichthyosis |
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| Occurs in boys and men and causes brownish flat scales most obvious on the arms, legs and tummy. It varies in severity and some affected babies have a difficult delivery It is passed on through the female line. |
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| Non-bullous ichthyosiform erythroderma and lamellar ichthyosis (nbCIE). |
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| These are very rare, often severe with prominent scaling and redness of most of the skin. Affected babies are often born with a shiny waxy second skin (collodion membrane) which sheds in a few days. |
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| Bullous ichthyosis (bCIE). |
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| This is another rare red ichthyosis which causes blistering, fragile skin early in infancy and thick scaling, especially around the joints later on. |
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| Harlequin ichthyosis. |
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| This condition is fortunately an extremely rare but severe ichthyosis which causes thick plates of scale and severe complications at birth. Many affected babies do not survive. |
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| Netherton's syndrome. |
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| Is made up of red inflamed scaly skin from birth, fragile spiky hair and difficulty gaining weight in early childhood. |
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| Sjögren-Larsson syndrome. |
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| 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. |
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| Conradi-Hünermann syndrome, Neutral lipid storage disease and KID syndrome. |
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These are very rare and associated with other medical problems.
http://www.ichthyosis.org.uk/types.aspx |
Ichthyosis Study
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