Ichthyosis Classification and external resources
ICD-10	Q80.
ICD-9	757.1
DiseasesDB	6646
MeSH	D007057

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Ichthyosis is a heterogeneous family of more than 30, generalized, mostly genetic skin disorders. The word comes from [[]] ιχθύωση and Ancient Greek ιχθύς, „fish“. The most common type of ichthyosis is ichthyosis vulgaris accounting for >95% of the cases¹.

Types

There are more than 30 types of ichthyosis but an exact diagnosis may be difficult. Types of ichthyosis are classified based on their appearance and their genetic cause. Ichthyoses caused by the same gene can vary considerably in severity and symptoms. Some ichthyoses don't appear to fit exactly into any one type. Also different genes can produce ichthyosis with similar symptoms. The most common or well-known types are: ²

Non-congenital ichthyoses (vulgar ichthyosis, not visible at birth, develops within the first months after birth)

• Ichthyosis vulgaris
• X-linked ichthyosis

Congenital ichthyoses (present at the day of birth)

• Congenital ichthyosiform erythrodema (nbCIE)
• Epidermolytic hyperkeratosis (bullous ichthyosis, bCIE)
• Harlequin ichthyosis
• Ichthyosis bullosa of Siemens
• Ichthyosis hystrix
• Ichthyosis lamellaris (lamellar ichthyosis)

Ichthyoses with additional characteristics

• CHILD Syndrome
• Conradi-Hünermann syndrome
• Darier's disease
• Erythrokeratoderma variabilis
• IFAP syndrome (ichthyosis follicularis)
• KID syndrome (Keratitis-ichthyosis-deafness syndrome)
• Netherton syndrome
• Neutral lipid storage disease ((Dorfman-Chanarin syndrome)
• Refsum's disease
• Rud syndrome
• Sjögren-Larsson syndrome
• Tay syndrome (IBIDS syndrome, Trichothiodystrophy)

Non-genetic ichthyosis

• Ichthyosis acquisita is acquired not inherited. It's a manifestation of other diseases.

Diagnosis

A physician often can diagnose ichthyosis by looking at the skin. A family history is also very useful. In some cases, a skin biopsy will be done to help to confirm the diagnosis. In a biopsy, a small piece of skin is removed and examined under a microscope. In some instances, genetic testing may be helpful in making a diagnosis. Ichthyosis is not more or less common in any ethnic group. As of now, there is no way to prevent ichthyosis.

Treatments

Treatments for ichthyosis often take the form of topical application of creams and emollient oils, in an attempt to hydrate the skin. Retinoids are also used for some conditions. Exposure to sunlight may improve or worsen the condition.

There can be ocular manifestations of ichthyosis, such as corneal and ocular surface diseases. Vascularizing keratitis, which is more commonly found in congenital keratitis-ichythosis-deafness (KID), may worsen with isotretinoin therapy.

Ichthyosis in animals

Ichthyosis or ichthyosis-like diseases exist for several types of animals, including cattle, chickens, llamas, mice and dogs. ³ Ichthyosis of varying severity is well-documented in some popular breeds of domestic dogs. The most common breeds in which this condition manifests itself are Golden retrievers, American bulldogs, Jack Russell terriers, and Cairn terriers. ⁴

See also

• emollient
• Skin disease

References

1. ^ Okulicz JF, Schwartz RA (2003). "Hereditary and acquired ichthyosis vulgaris". International Journal of Dermatology 42 (2): 95-8. PMID 12708996. 
2. ^ Types of Ichthyosis, F.I.R.S.T
3. ^ Sundberg, John P., Handbook of Mouse Mutations with Skin and Hair Abnormalities, Page 333, Published by CRC Press, 1994, ISBN 0849383722
4. ^ Gross, Thelma Lee, Veterinary Dermatopathology, Page 174-179, Published by Blackwell Publishing, 2004, ISBN 0632064528

External links

• Foundation for Ichthyosis and Related Skin Types USA
• British Ichthyosis Support Group
• European Network for Ichthyosis
• DermAtlas 1896838546