A Simple Guide to Ichthyosis

A Simple Guide to Ichthyosis
----------------------------------------------

What is Ichthyosis?
------------------------------------

Ichthyosis is a rare non contagious disease of the keratin of the skin where there is reduction of sweat and sebaceous secretions.

The word Ichthus means fish.

So Ichthyosis means fish like appearance of the skin.


Who is at risk of Ichthyosis?
----------------------------------------------


a family history of ichthyosis


What are the causes of Ichthyosis?
--------------------------------------------------


Most forms of ichthyosis are due to:

A.genetic conditions:
------------------------

1.Ichthyosis vulgaris 95% -autosomal dominant
2.X-linked ichthyosis -sex linked recessive
3.Ichthyosis lamellaris - autosomal recessive usually in newborns
4.Harlequins Ichthyosis -autosomal recessive
5.Bullous Ichthyosis - autosomal dominant

B.Acquired Ichthyosis
----------------------

6.Ichthyosis acquisita occur in :
leprosy,
hypothyroidism,
lymphoma,
sarcoidosis,
Hodgkin disease

7.Xeroderma - is a mild form of ichthyosis, occurs in middle aged and older patients.


What are Signs and symptoms of Ichthyosis?
---------------------------------------------


Symptoms:

1.extensor surface of limbs covered with dry scales(rarely the whole body is covered).

2.axilla and antecubal fossa (elbows,armpits,groins,back of knee)not affected

3.Usually symptoms present in childhood but improve during puberty to becoming worst again in later life.

4.Rashes can be extremely itchy in xeroderma

5.The condition is worse in colder weather.

6.Hair may be thin and dry.

7.Nails are brittle and dry.


How is the diagnosis of Ichthyosis made?
--------------------------------------------------------


1.Appearance of the skin

2.Family history of Ichthyosis

2.Skin biopsy for microscopic examination.


What are the complications of Ichthyosis?
-----------------------------------------------


1.keratisis with corneal and ocular surface disease

2.Ectropion of eyelids due to dehydration

3.Deafness in a Keratitis-Ichthyosis-Deafness (KID)Disease


What is the treatment of Ichthyosis?
------------------------------------


There is no cure for Ichthyosis.

1.Symptomatic treatment with emolient creams such as petroleum jelly my help to hydrate the skin.

2.Itch may be controlled with antihistamines

3.Hydration with propylene glycol solution will help

4.Urea in emulsion creams has also help the dry skin

5.Topical or oral retinoic acid has brought some improvement to the skin.

6.Exposure to the sun may improve or worsen the condition.


What is the prognosis of Ichthyosis ?
------------------------------------------


It is a lifelong condition and need continuous treatment.


What are the Preventive measures taken for Ichthyosis ?
--------------------------------------------------------


There is no known preventive measure for ichthyosis