Congenital ichthyosis

Information about Congenital ichthyosis


Classification & external resources
ICD-10Q80.
ICD-9757.1
DiseasesDB6646
MeSHD007057


Ichthyosis is a family of genetic dermatological conditions seen in humans and domestic animals. The word comes from Greek ιχθύωσις lit. "forming fish", as people or animals with ichthyosis have scaly skin which can vaguely resemble the scales of a fish.

The term ichthyosis is sometimes used to refer to the specific condition ichthyosis vulgaris. Ichthyosis was formerly referred to as "pseudo-leprosy," as it can produce an appearance superficially similar to that of leprosy.

Types

While ichthyosis acquisita is acquired (as its name indicates), most forms of ichthyosis are considered congenital. These types include:

Some types of ichthyosis include:

Ichthyosis in domestic dogs

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.

Diagnosis

A physician (or veterinarian) 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 rare 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. Ectropion, if it occurs, can be treated surgically after skin hydration.

See also

External links

 •  • [ e]
Congenital malformations and deformations of integument (Q80-Q84, )
Congenital ichthyosisIchthyosis vulgaris - X-linked ichthyosis - Ichthyosis lamellaris - Epidermolytic hyperkeratosis - Harlequin type ichthyosis - Netherton's syndrome
Epidermolysis bullosaEpidermolysis bullosa simplex - Epidermolysis bullosa dystrophica
Other, of skinHereditary lymphedema - Xeroderma pigmentosum - Mastocytosis - Urticaria pigmentosa - Incontinentia pigmenti - Ectodermal dysplasia - Port-wine stain - Cutis laxa - Darier's disease - Bloom syndrome - Pseudoxanthoma elasticum
Of breastAccessory breast - Supernumerary nipple
Other,
of integument		Monilethrix
see also (, )
For other uses of "ICD", see ICD (disambiguation).


The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD
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List of ICD-10 codes. The version for 2007 is available online at [1]

Chapter Blocks Title
I Certain infectious and parasitic diseases
II Neoplasms
III Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
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For other uses of "ICD", see ICD (disambiguation).


The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD
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The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. These codes are in the public domain.

See also


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The Diseases Database is a free website that provides information about the relationships between medical conditions, symptoms, and medications.

It directly integrates the Unified Medical Language System.

External links

  • Diseases Database

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Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. Created and updated by the United States National Library of Medicine (NLM), it is used by the MEDLINE/PubMed
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A genetic disorder is a condition caused by abnormalities in genes or chromosomes. While some diseases, such as cancer, are due to genetic abnormalities acquired in a few cells during life, the term "genetic disease" most commonly refers to diseases present in all cells of the body
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Dermatology (from Greek δερμα, "skin") is a branch of medicine dealing with the skin and its appendages (hair, sweat glands, etc).

Scope of the field

Dermatologists
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Greek}}} 
Writing system: Greek alphabet 
Official status
Official language of:  Greece
 Cyprus
 European Union
recognised as minority language in parts of:
 European Union
 Italy
 Turkey
Regulated by:
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scale (Greek lepid, Latin squama) is a small rigid plate that grows out of an animal's skin to provide protection. In lepidopteran species, scales are plates on the surface of the insect wing, and provide coloration.
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MeSH D016112 Ichthyosis vulgaris is a skin disorder causing dry, scaly skin.

It is the most common form of ichthyosis, affecting around 1 in 250 people [1]. For this reason it is known as common ichthyosis.
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Leprosy
Classification & external resources

A 24-year-old man infected with leprosy.
ICD-10 A 30.
ICD-9 030

OMIM 246300
DiseasesDB 8478
MedlinePlus 001347
eMedicine med/1281   derm/223 neuro/187

MeSH C01.252.410.040.
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Ichthyosis acquisita
Classification & external resources

ICD-10 L 85.0
ICD-9 701.1

Ichthyosis acquisita (or "acquired ichthyosis") is a disorder clinically and histologically similar to ichthyosis vulgaris.
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An acquired disorder is a medical condition which develops post-fetally; in contrast with a congenital disorder, which is present at birth. A congenital disorder may be antecedent to an acquired disorder (such as Eisenmenger's syndrome).
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A congenital disorder is any medical condition that is present at birth. However, a congenital disorder can be recognized before birth (prenatally), at birth, years later, or never. The term congenital does not imply or exclude a genetic cause.
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MeSH D016112 Ichthyosis vulgaris is a skin disorder causing dry, scaly skin.

It is the most common form of ichthyosis, affecting around 1 in 250 people [1]. For this reason it is known as common ichthyosis.
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X-linked ichthyosis
Classification & external resources

ICD-10 Q 80.1
ICD-9 757.1

OMIM 308100
DiseasesDB 29136

eMedicine derm/191  
MeSH D016114 X-linked ichthyosis
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MeSH D017490 Ichthyosis lammellaris, also known as (recessive) lamellar ichthyosis and nonbullous congenital ichthyosis, is a rare inherited skin disorder, affecting less than 1 person in 300,000.
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Epidermolytic hyperkeratosis
Classification & external resources

ICD-10 Q 80.3

OMIM 113800
DiseasesDB 33392

eMedicine derm/590  
MeSH D017488 Epidermolytic hyperkeratosis, also known as
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MeSH D017490 Harlequin-type ichthyosis (also harlequin ichthyosis, ichthyosis congenita, or keratosis diffusa fetalis), a skin disease, is the most severe form of congenital ichthyosis, characterized by a thickening of the keratin
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Netherton's syndrome is a form of ichthyosis associated with SPINK5 .

It is named for E.W. Netherton.[1]

References

1. ^ Netherton, E. W. A unique case of trichorrhexis nodosa: 'bamboo hairs.'. Arch. Derm.

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Sjögren-Larsson syndrome
Classification & external resources

ICD-10 Q 87.1 (ILDS Q87.136)

OMIM 270200
DiseasesDB 30051

eMedicine derm/706  
MeSH D016111 Sjogren-Larsson syndrome (or Rud's syndrome
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dog breed is a group of dogs that have very similar or nearly identical characteristics of appearance or behavior or, usually, both, primarily because they come from a select set of ancestors who had the same characteristics.
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C. l. familiaris

Trinomial name
Canis lupus familiaris
(Linnaeus, 1758)

The dog (Canis lupus familiaris) is a domestic subspecies of the wolf, a mammal of the Canidae family of the order Carnivora.
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Golden Retriever is the basic name given to a breed of dog, originally developed to retrieve shot game during hunting. It is one of the most common family dogs as it is naturally very friendly and amenable to training.
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American Bulldog is a breed of working dog developed for catching livestock and for protecting property. Though larger in size, they are the closest surviving relative of the Old English Bulldog because they were not altered to as great an extent while in Colonial America as their
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Jack Russell Terrier is a type or landrace of small, principally white-bodied, smooth or rough-coated terrier that has its origins in fox hunting. The name "Jack Russell" has been used to describe a wide array of small white terriers, but is now most commonly used to describe a
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Cairn Terrier is a breed of dog of the terrier category. It is one of the oldest terriers, originating in the Scottish Highlands and recognised as one of Scotland's earliest working dogs, used for hunting burrowing prey among the cairns.
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Emollients are substances which soften and soothe the skin. They are used to correct dryness and scaling of the skin. They are a key component in the manufacture of lipstick, lotions and other cosmetic products.
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The retinoids are a class of chemical compounds that are related chemically to vitamin A. Retinoids are used in medicine, primarily due to the way they regulate epithelial cell growth.
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