Nonbullous congenital ichthyosiform erythroderma (NBCIE) is a specific type of ichthyosis mainly affecting the skin. Most infants with NBCIE are born with a tight, shiny covering on their skin, called a collodion membrane, which is typically shed within a few weeks.
What causes congenital ichthyosis?
Ichthyosis vulgaris is commonly caused by a genetic mutation that’s inherited from one or both parents. Children who inherit a defective gene from just one parent have a milder form of the disease.
How is Epidermolytic ichthyosis treated?
There is no cure for epidermolytic ichthyosis (EI). The primary goal of therapy is to reduce hyperkeratosis. This can be achieved with topical keratolytics such as lactic acid, alpha-hydroxy acid, or urea. Topical emollients such as those containing glycerin are also often useful.
Is epidermolytic hyperkeratosis a rare disease?
Epidermolytic ichthyosis (EI) is a rare, genetic skin disorder. It becomes apparent at birth, or shortly after birth, with reddening, scaling, and severe blistering of the skin. Hyperkeratosis (thickening of the skin) develops within months and worsens over time.
What is a collodion baby?
The term collodion baby (CB) refers to a newborn whose entire body is covered with an adherent, supple, parchment-like membrane. 1. The condition is usually associated with ectropion, eclabium, hypotrichosis, hypoplastic nasal and auricular cartilage, and pseudocontractures.
Is erythroderma genetic?
Congenital ichthyosiform erythroderma is genetically diverse and has been found to have mutations in the same genes as lamellar ichthyosis discussed above (TGM1, ABCA12, ALOXE3, ALOX12B, and NIPAL4) which explains the phenotypic overlap of these two diseases.
Why do my legs look like fish scales?
Ichthyosis is a group of about 20 skin conditions that cause skin dryness and scaling. The condition gets its name from the Greek word for fish, because the skin looks like fish scales. You might also hear it called fish scale or fish skin disease.
Can ichthyosis be cured?
There’s no cure for ichthyosis, but moisturising and exfoliating the skin daily can help prevent dryness, scaling and the build-up of skin cells.
How can ichthyosis be prevented?
There is no way to prevent ichthyosis. As with other genetic diseases, there is a risk that children of an affected parent will inherit the gene.
What does Epidermolytic hyperkeratosis look like?
Epidermolytic hyperkeratosis (EHK) presents as erythematous, scaly, crusted plaques . (Courtesy J. Conlon, MD.) EHK clinically shows erythematous, scaly plaques, which may present anywhere on the body but predominate around creases and folds (intertriginous areas) .
What are the symptoms of Epidermolytic ichthyosis?
What are the clinical features of epidermolytic ichthyosis?
- Widespread erythroderma, blisters, and superficial ulceration at birth.
- Peeling, erosions, and denuded skin after minor friction or trauma.
- Improvement with age; blistering is replaced by dark-brown, grey, or white dry or macerated scales and erythema.
How common is Epidermolytic hyperkeratosis?
Epidermolytic hyperkeratosis affects approximately 1 in 200,000 to 300,000 people worldwide.
Why does hyperkeratosis occur?
Causes and types Pressure-related hyperkeratosis occurs as a result of excessive pressure, inflammation or irritation to the skin. When this happens, the skin responds by producing extra layers of keratin to protect the damaged areas of skin. Non-pressure related keratosis occurs on skin that has not been irritated.
Does hyperkeratosis go away?
They do not disappear without treatment. Inherited forms of hyperkeratosis are lifelong conditions.
What does Epidermolytic mean?
: a state of detachment or loosening of the epidermis.
What is Harlequin baby syndrome?
Related Disorders Lamellar ichthyosis may also cause reddened skin (erythroderma), thickened skin on the palms and soles and decreased sweating with heat intolerance. (For more information on lamellar ichthyosis, choose Ichthyosis, Lamellar as your search term in the Rare Disease Database.)
What is a plastic baby?
Collodion babies, scientifically known as plastic babies, are often premature who are born encased in a skin that resembles a yellow, tight and shiny film or dried collodion (sausage skin). … The plastic newborns are scientifically known as collodion babies.
What causes erythema Toxicum?
The cause of erythema toxicum neonatorum is unknown. Multiple theories have been proposed to explain this common disorder. Neonates have an increased number of hair follicles compared with adults, and the occurrence of erythema toxicum neonatorum in nonhair-bearing areas such as palms and soles is rare.
What is congenital ichthyosis?
Congenital ichthyosis is a collective name for a group of monogenetic disorders of cornification, sometimes associated with systemic symptoms. There may be an abnormal quality or quantity of scale produced, abnormal thickness of stratum corneum or abnormal keratinocyte kinetics, often associated with skin inflammation.
Is Xerosis a disease?
Xerosis is the medical name for dry skin. It comes from Greek: ‘xero’ means ‘dry’ and ‘osis’ means ‘disease’ or ‘medical disorder’. Xerosis is caused by a lack of moisture in the skin, which may be the result of ageing (senile Xerosis) or due to underlying diseases such as Diabetes.
What causes Trichothiodystrophy?
What causes trichothiodystrophy? Trichothiodystrophy is caused by defective DNA repair and transcription and is inherited in an autosomal recessive pattern, meaning both parents must carry a copy of the mutated gene that causes trichothiodystrophy for a child to inherit the condition.
Does dead skin turn white?
Maceration occurs when skin has been exposed to moisture for too long. A telltale sign of maceration is skin that looks soggy, feels soft, or appears whiter than usual. There may be a white ring around the wound in wounds that are too moist or have exposure to too much drainage.
Why is the skin on my legs shiny?
Shiny legs syndrome is often caused by stretching of the skin, which might be related to pooling of fluid in the lower extremities. Because chronic congestive heart failure leads to such an accumulation, shiny legs may be a sign of a heart disorder.
Does dead skin itch?
Depending on the cause, itchy skin can be short-term and disappear quickly, such as when itching occurs due to a buildup of dead skin cells. For example, if you feel itchy all over and live in a dry climate, you may have winter itch, which is caused by the lack of moisture in your skin.
How long is the average lifespan of a person with ichthyosis?
In the past, it was rare for a baby born with Harlequin ichthyosis to survive beyond a few days. But things are changing, largely due to improved intensive care for newborns and the use of oral retinoids. Today, those who survive infancy have a life expectancy extending into the teens and 20s.
Is Vaseline good for ichthyosis?
Petroleum jelly is another good choice. Apply an over-the-counter product that contains urea, lactic acid or a low concentration of salicylic acid twice daily. Mild acidic compounds help skin shed its dead skin cells. Urea helps bind moisture to skin.
Does ichthyosis get worse with age?
The condition usually improves with age. For the most part, people who have ichthyosis vulgaris live a normal life, although they will probably always have to treat their skin. The disease rarely affects overall health.
How do you get rid of gator skin on arms?
7 Ways to Help Cure Your Winter Alligator Skin
- Drink more water. …
- Stop the steaming hot showers. …
- Use the right soap. …
- Ditch that scratchy mesh sponge (maybe) …
- Don’t completely dry off after a shower. …
- Moisturize at the right time. …
- Try a humidifier.
Is ichthyosis considered disability?
Ichthyosis is one of the impairments listed in the Social Security Administrations Blue Book. If your ichthyosis is severe enough (or includes flare-ups that are severe enough) to hinder your ability to work, you may be eligible for Social Security Disability benefits.
How do you get rid of ichthyosis?
How do dermatologists treat ichthyosis vulgaris?
- Take baths as often as directed. Soaking helps hydrate your skin and soften the scale. …
- Reduce the scale during your baths. …
- Apply moisturizer to damp skin within two minutes of bathing. …
- Apply petroleum jelly to the deep cracks. …
- Treat a skin infection.
Graduated from ENSAT (national agronomic school of Toulouse) in plant sciences in 2018, I pursued a CIFRE doctorate under contract with Sun’Agri and INRAE in Avignon between 2019 and 2022. My thesis aimed to study dynamic agrivoltaic systems, in my case in arboriculture. I love to write and share science related Stuff Here on my Website. I am currently continuing at Sun’Agri as an R&D engineer.