“Fish scale disease” (Ichthyosis)

 Family of genetic disorder

Defined:-

                 a congenital skin condition which cause

                 the epidermis to become dry & horny

                 like fish scales

 

 Ichthyosis comes from the Greek ( ichthys,     literally "fish"), since dry, scaly skin is the defining

feature of all forms of ichthyosis. 

Causes:-

                1) commonly caused by genetic factor

                  2) taking certain types of drugs. 

Symptoms:-

                       1) itchy skin

                       2) flaky skin

                       3) thickened skin

                       4) eczema

                       5) painful skin cracks

"SYMPTOMS MAY APPEAR BY THE AGE OF 5 YEARS"

Types:- 

              Many types of ichthyoses exist, and an exact diagnosis may be difficult. Types of ichthyoses are

classified by their appearance, if they are syndromic or not, and by mode of inheritance. For

example, non-syndromic ichthyoses that are inherited recessively come under the umbrella term

autosomal recessive congenital ichthyosis. 

Ichthyosis caused by mutations in the same gene can vary considerably in severity and 

symptoms. Some ichthyoses do not appear to fit exactly into any one type while mutations in

different genes can produce ichthyoses with similar symptoms. Of note, X-linked ichthyosis is

associated with Kallmann syndrome (close to the KAL1 gene). The most common or well-known

types are:

1) Ichthyosis vulgaris:- autosomal semi-dominanat

2) Ichthyosis lamellar:- autosomal recessive

3) Congenital ichthyosis erythroderma:- autosomal recessive

4) X-linked ichthyosis:- x-linked recessive

5) Epidermolytic hyperkeratosis:- dominate genetic condition caused by mutation of gene

 (keratin 1&10) 

#)Non-genetic ichthyosis:- Ichthyosis acquisita

Diagnosis:-

 Ichthyosis is a genetically and phenotypically heterogeneous disease , 

 A physician often can diagnose ichthyosis by looking at the skin. A family history is also useful

in determining the mode of inheritance. In some cases, a skin biopsy is done to help to confirm

the diagnosis while in others genetic testing may be helpful in making a diagnosis.

Treatment:-

Treatment consists of skin care, 

1) Moisturizer

2) Hydrotherapy

3) Anti-inflamatory & vit. A derivative. 

Treatments for ichthyosis often take the form of topical application of creams and emollient oils,

in an attempt to hydrate the skin. Creams containing a high percentage of urea or lactic acid

have been shown to work exceptionally well in some cases. Application of propylene glycol is

another treatment method. Retinoids are used for some conditions.

Exposure to sunlight may improve or worsen the condition. In some cases, excess dead skin

sloughs off much better from wet tanned skin after bathing or a swim, although the dry skin

might be preferable to the damaging effects of sun exposure.

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 ,may worsen with isotretinoin therapy.