Part 1: Ichthyosis refers to a group of skin disorders that lead to dry, itchy skin that appears scaly, rough, and red. The symptoms can range from mild to severe.
There are more than 30 distinct types of ichthyosis, including those that occur as part of another syndrome or condition.
Doctors may determine the type of ichthyosis by identifying the:
* Gene mutation.
* Inheritance pattern through analyzing family trees.
* Symptoms, including their severity and which organs they affect.
* Age when symptoms first appeared.
Some types of the disease, which are inherited and are not part of a syndrome, include the following:
Ichthyosis vulgaris is the most common type. It is usually mild and appears in the first year of life with dry, flaky skin.
Harlequin ichthyosis is usually seen at birth and causes thick scaly plates of skin that cover the entire body. This form of the disorder can affect the shape of facial features and may limit joint movement.
Epidermolytic ichthyosis is present at birth. Most infants are born with fragile skin and blisters covering their body. Over time, the blisters disappear, and scaling of the skin develops. This can have a ridged appearance over areas of the body that bend.
Lamellar ichthyosis is present at birth. The infant is born with a tight clear covering the entire body, called a collodion membrane. Over several weeks, the membrane peels away, and large, dark, plate-like scales develop over most of the body.
Congenital ichthyosiform erythroderma is present at birth. Infants also often present with a collodion membrane.
X-linked ichthyosis usually develops in males and begins at about 3 to 6 months of life. Scaling is usually present on the neck, lower face, trunk, and legs, and symptoms can worsen over time.
Erythrokeratodermia variabilis usually develops a few months after birth and progresses during childhood. The skin can develop rough, thick or reddened areas of skin, usually on the face, buttocks, or limbs. The affected areas can spread on the skin over time.
Progressive symmetric erythrokeratoderma usually appears in childhood with dry, red, scaly skin primarily on the limbs, buttocks, face, ankles, and wrists.
The symptoms of ichthyosis can range from mild to severe. The most common symptoms include:
* Dry skin.
* Itching.
* Redness of the skin.
* Cracking of the skin.
* Scales on the skin that are white, gray, or brown and have the following appearance:
* Small and flaky.
* Large, dark, plate-like scales.
* Hard, armor-like scales.
Depending on the type of ichthyosis, other symptoms may include:
* Blisters that can break, leading to wounds.
* Hair loss or fragile hair.
* Dry eyes and difficulty closing eyelids. Inability to perspire (sweat) because skin scales clog the sweat glands.
* Difficulty hearing.
* Thickening of the skin on the palms of the hands and soles of the feet.
* Tightening of the skin.
* Difficulty flexing some joints.
Cause of Ichthyosis
Gene mutations (changes) cause all of the inherited types of ichthyosis.
Many gene mutations have been identified and the inheritance pattern depends on the type of ichthyosis.
People continually grow new skin and shed old skin throughout their lives. For people with ichthyosis, the mutated genes change the normal skin growth and shedding cycle, causing skin cells to do one of the following:
* Grow faster than they are shed.
* Grow at a normal rate, but shed at a slow rate.
* Shed faster than they grow.
There are different types of inheritance patterns of ichthyosis, including:
Dominant, which means you inherit one normal copy and one mutated copy of the gene that causes ichthyosis.
The abnormal copy of the gene is stronger or “dominant” over the normal copy of the gene, causing the disease. A person with a dominant mutation has a 50% chance (1 in 2) of passing the disorder to each of his or her children.
Recessive, which means that your parents do not have signs of ichthyosis, but both parents carry only one abnormal gene, which is not enough to cause the disease.
When both parents carry one recessive gene, there is a 25% chance (1 out of 4) per pregnancy of having a child who inherits both of these mutated genes and develops the disorder.
There is a 50% chance (2 out of 4) per pregnancy of having a child who inherits only one mutated recessive gene, making them a carrier of the disease gene without noticeable signs.
If one parent has a recessive form of ichthyosis with two mutated genes, all their children will carry one abnormal gene, but will not usually have noticeable signs of ichthyosis.
X-linked, which means the gene mutations are located on the X sex chromosome.
Each person has two sex chromosomes:
Females generally have two X chromosomes (XX), and males generally have one X chromosome and one Y chromosome (XY).
The mother always passes on an X chromosome, but the father can pass on either an X or Y chromosome.
The inheritance pattern for X-linked ichthyosis is usually recessive; this means that males, who only have one X chromosome to begin with, pass on the mutated X chromosome.
Because of this pattern, females are affected more often, and typically they have one mutated and one normal X chromosome.
Spontaneous, which means the gene mutation occurs randomly without a family history of the disorder. This is most common in dominant and X-linked ichthyosis.
References for above info:
Here
What causes ichthyosis vulgaris part 2:
Inherited ichthyosis vulgaris is caused by genes. The person either inherits the genes for this disease from one or both parents or genes changes while the baby is forming inside the womb.
As a result, the skin has less filaggrin than it needs. The body needs filaggrin to create a healthy outermost layer of skin.
Without enough filaggrin, the body cannot shed skin cells as it should. The older skin cells build up on the skin’s surface, causing the scale and thickened skin.
In very rare cases, a medicine or vitamin can trigger acquired ichthyosis vulgaris. Ones that can do this, include:
*Cimetidine (an antacid and antihistamine used to treat ulcers and acid reflux)
* Clofazimine (a drug used to treat leprosy)
* Nicotinic acid, a B vitamin
References for above info:
Here
Filaggrin:
Filaggrin is a filament-associated protein that binds to keratin fibers in epithelial cells.
Ten to twelve filaggrin units are post-translationally hydrolyzed from a large profilaggrin precursor protein during terminal differentiation of epidermal cells.
Filaggrin is a protein originated from pro-filaggrin, produced by keratinocytes. It is the main component of keratohyalin granules, visualized by light microscopy within the granular layer.
Filaggrin is a protein that occurs naturally in the skin.
It’s vital for the skin’s ability to remain hydrated because it breaks down into water-binding amino acids or “natural moisturizing factors” (NMFs) that help keep the skin properly hydrated.
Without NMFs, the skin barrier dries out and cracks. This can lead to itching and leave the skin more prone to infection.
Filaggrin is a histidine-rich protein. And histidine is metabolized to trans-urocanic acid (trans-UCA); pyrrolidone-5-carboxylic acid (PCA) is the other main breakdown product of filaggrin and together these organic acids help to maintain the pH gradient of the epidermis as evidenced by a higher surface pH in FLG null mutation carriers.
What causes lack of filaggrin?
Genetic filaggrin deficiency—a chronic condition affecting up to 9% of the population.
Skin inflammation (e.g., from atopic dermatitis or psoriasis), which can cause a decrease in the natural production of filaggrin and the enzyme that breaks down filaggrin into water-binding amino acids.
References for above info:
Histidine:
L-histidine (also known as histidine) is an essential amino acid.
Deficiencies have been linked to eczema in both children and adults. Since it is an essential amino acid – which is the building block of protein – our bodies cannot produce L-histidine on its own and it must be consumed through the diet.
A recent study showed that supplementing with 4g of L-histidine per day improved clinical signs and symptoms of atopic dermatitis by about 32%.
But further studies are necessary to determine whether or not l-histidine is an effective tool for reducing eczema.
Histidine can be found in pork, beef, lamb, chicken, turkey, fish, soy, beans, milk, cheese, nuts, seeds, whole grains and eggs, buckwheat, corn, cauliflower, mushrooms, potatoes, bamboo shoots, bananas, cantaloupe and citrus fruits.
Fruits High in Histidine (per 100 g edible portion)
Banana (raw) 80 mg.
Avocado (raw) 61 mg.
Kiwifruit (raw) 23 mg.
Strawberry (raw) 15 mg.
Melon ( raw) 14 mg.
Japanese apricot (raw) 12 mg.
Pineapple (raw) 11 mg.
A recent study showed that supplementing with 4g of histidine per day improved clinical signs and symptoms of atopic dermatitis by about 32%.
References for above info:
Disruption of the skin's barrier function also leads to excessive water loss. This is why the skin becomes so dry. In the extreme, this may lead to dehydration.
Children with ichthyosis need to drink plenty of water or juice, and provisions must be made at school to allow this.
References for above info:
Keratinocytes
Keratinocytes are the primary type of cell found in the epidermis, the outermost layer of the skin.
In humans, they constitute 90% of epidermal skin cells. Basal cells in the basal layer of the skin are sometimes referred to as basal keratinocytes.
Keratinocytes form a barrier against environmental damage by heat, UV radiation, water loss, pathogenic bacteria, fungi, parasites, and viruses.
A number of structural proteins, enzymes, lipids, and antimicrobial peptides contribute to maintain the important barrier function of the skin.
Keratinocytes differentiate from epidermal stem cells in the lower part of the epidermis and migrate towards the surface, finally becoming corneocytes and eventually being shed, which happens every 40 to 56 days in humans.
Keratinocytes are cells that make up over 90% of the epidermis or the outer layer of the skin.
They produce and secrete a protein called keratin, which is also called an intermediate filament protein, which holds the skin cells and layers together.
The amount of keratin secreted by the keratinocytes in different parts of the body is responsible for how thick that area of skin becomes.
This is why the skin around the knees and soles of the feet are tougher than the thin, soft and sensitive skin around the neck.
Foods that naturally increase the production of keratin in our bodies:
Sunflower seeds
Almonds
Nuts
Garlic
Carrots
Chickpeas
Green leafy vegetables
Kale
Avocados
Spinach
Broccoli
Legumes
Sweet potatoes
Carrots
Salmon
Chicken
Eggs
Mangoes
Products containing urea, lactic acid or other alpha-hydroxy acids seem to help some individuals with ichthyosis.
References for the above info:
Additional references:
The above info was copied and pasted from online sources. The reference links will take you to the source per section.
To recap on the types of foods that can help alleviate symptoms and assist in bringing the body into more balance:
pork
beef
lamb
chicken
turkey
fish
salmon
collagen
gelatin
beans
legumes
milk
cheese
eggs
nuts
seeds; chia, pumpkin, sesame, sunflower seeds
whole grains
buckwheat
banana
kiwi
strawberry
cantaloupe
japanese apricot
pineapple
citrus fruits
mango
carrots
chickpeas
green leafy vegetables
kale
avocados
spinach
broccoli
corn
cauliflower
mushrooms
potatoes
sweet potatoes
bamboo shoots
An area that can trigger painful symptom episodes are food allergies. So be sure to keep that in mind as you're going through the list of foods and making any type of dietary changes.
Food allergies that are common in triggering symptoms are wheat, dairy and nuts.
As well as laundry detergents, soaps, shampoos, lotions, makeup, perfumes, cologne, aftershave, etc.
Research shows that keeping a food journal is very beneficial in pinpointing certain food and other environmental allergies that trigger symptoms.
When using skincare products in treating the symptoms, using a product that contains emollients, humectants and occlusives will give a well-rounded approach to your skin care.
