Materiały źródłowe

• #1 All about ichthyosis: causes, symptoms and treatments

  https://www.dexeryl.com/en/your-skin/ichthyosis

  Ichthyosis is a rare genetic skin disease characterised by very dry skin and a fish-scale appearance. […] Ichthyosis is a dermatological condition characterised by very dry and rough skin, significant shedding, i.e. removal of the surface layers of the skin in the form of small flakes, and hyperkeratosis, which is a thickening of the skin. […] It is a chronic disease, meaning that the symptoms are permanent. […] To date, there is no curative treatment for the various forms of ichthyosis. Management is mainly based on symptomatic and local treatment of skin dryness and hyperkeratosis. […] Emollients are the first line of treatment. Whatever the form of ichthyosis, there is an alteration of the skin’s barrier function and an emollient must be applied daily. […] Appropriate daily hydration and hygiene will work in synergy to treat the symptoms of ichthyosis.

• #2 Ichthyosis | Laserderm Dermatology & Cosmetic Laser Surgery

  https://www.laserderm.com/general-dermatology/skin-conditions/ichthyosis/

  Ichthyosis is a skin condition characterized by scaly skin that collects on the outer layer of the epidermis. […] Ichthyosis affects the body’s natural ability to shed dead skin cells and instead creates a chronic buildup of cells that are not able to flake away and shed normally. […] Signs and symptoms depend on the category of ichthyosis present, but typical signs include dry or scaly skin, excessive skin peeling, and blistering. […] There is no cure for ichthyosis, which means that the treatment options available are symptomatic in nature. […] By controlling the symptoms of the condition, you should be able to carry out everyday activities without feeling the discomfort associated with the disorder. […] The physicians at Laserderm Dermatology & Cosmetic Laser Surgery may also suggest preventative solutions that help alleviate some of the symptoms associated with ichthyosis.

• #3 Harlequin Ichthyosis (HI): Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/harlequin-ichthyosis

  Your baby will go to the neonatal intensive care unit (NICU) as soon as theyre born. There, your baby will stay in a high-humidity incubator to help regulate their body temperature. Nurses will care for your baby by: […] If your baby has a severe case of harlequin ichthyosis, they may receive treatment with an oral retinoid called etretinate. This drug can help remove the thick, platelike scales covering their skin. It can also help reverse issues such as constricting fingers, compromised blood flow, tightened chest making it difficult to breathe and tightened face making it impossible to feed. Your babys healthcare provider will only use oral retinoids if your baby has severe symptoms because long-term use can cause serious adverse side effects. […] Once the thick, platelike skin begins to split and peel off, your baby may be able to go home. But theyll require continued medical care. Harlequin ichthyosis treatment involves a team of healthcare providers. This team may include:

• #4 Ichthyosis

  https://www.rch.org.au/kidsinfo/fact_sheets/Ichthyosis/

  Ichthyosis means 'fish scale’, and is the name of a group of genetic skin diseases that cause dry, scaly, thickened skin. […] There is no known cure for ichthyosis, but there are treatments available that can help to improve the skin’s condition. […] Most children with ichthyosis will have a mild form of the disease, and there will a minimal effect on their general health and wellbeing. […] In rare cases, it can be very severe and affect the entire skin surface or cause blisters. […] If your child has ichthyosis, you may need to spend time each day caring for their skin. […] Moisturisers help improve the condition of skin dryness, replacing the lost moisture and holding it in the skin. […] Treatment may involve medications and always taking good care of the skin. […] If your child is prescribed retinoid medication they will need regular blood tests and check-ups.

• #5 Ichthyosis Vulgaris: Causes, Symptoms, and Diagnosis

  https://www.healthline.com/health/ichthyosis-vulgaris

  Ichthyosis vulgaris is an inherited or acquired skin condition that occurs when the skin doesnt shed its dead skin cells. […] The majority of cases are mild and confined to specific areas of the body. However, some cases are severe and cover large areas of the body, including the abdomen, back, arms, and legs. […] Symptoms of ichthyosis vulgaris include flaky scalp, itchy skin, polygon-shaped scales on the skin, scales that are brown, gray, or white, severely dry skin, and thickened skin. […] Symptoms of ichthyosis vulgaris are typically worse in winter, when the air is colder and drier. […] In severe cases, ichthyosis vulgaris may also cause deep, painful cracks to develop on the soles of the feet or palms of the hands. […] Theres currently no cure for ichthyosis vulgaris. However, treatment can help you manage your symptoms.

• #6 Ichthyosis

  https://www.nhs.uk/conditions/ichthyosis/

  Most types of ichthyosis cannot be cured, but there are treatments to help the symptoms. […] Treatments may include: moisturising treatments (emollients), such as lotions, creams, ointments and bath oils. […] A skin specialist will talk to you about the different options. […] For more severe ichthyosis symptoms treatment can also include: antibiotics to treat or prevent skin infections, retinoid tablets or creams to help improve the appearance of scaly skin. […] If the ichthyosis symptoms are caused by another health condition or medicine, treating the condition or changing the medicine can improve the symptoms. […] There are things you can do to help ease ichthyosis symptoms at home. […] use emollients on affected skin at least once a day ideally when your skin is still wet after having a bath or shower. […] try different types of emollient to find 1 that works best. […] talk to your skin specialist if any treatments cause irritation. […] do not use soap on affected skin use an emollient instead of soap.

• #7 Ichthyosis: Diagnosis, Treatment, and Steps to Take

  https://www.niams.nih.gov/health-topics/ichthyosis/diagnosis-treatment-and-steps-to-take

  Health care providers usually diagnose ichthyosis by: […] In addition, your health care provider may be able to diagnose ichthyosis with a genetic test that detects the mutated gene usually from a blood sample or a swab from the mouth. A genetic counselor or specialist can help you understand the test results. […] There is currently no cure for ichthyosis. The goals of treatment include reducing the redness of the skin, thickness of the scales, and itching. Treatments can include: […] Depending on the type and severity of the disease, doctors may recommend additional treatment with keratolytic topical agents, which can help to loosen scales. However, these can be irritating for some people and have potential side effects if used in large amounts. Talk to your doctor before using any treatment option.

• #8 Ichthyosis vulgaris: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/ichthyosis-vulgaris-treatment

  A dermatologist can often diagnose ichthyosis vulgaris by looking at a patients skin. […] Treatment focuses on reducing the scale and dry skin. […] Soaking helps hydrate your skin and soften the scale. […] Moisturizer can seal water from a bath or shower into your skin. […] If you develop a skin infection, your dermatologist will treat it with medication that you either take or apply to your skin. […] Soaking, moisturizing, and removing the scale can also get rid of the rough bumps on the skin, known as keratosis pilaris. […] Most people, however, need to continue treating their skin for life.

• #9 Ichthyosis – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/ichthyosis/

  Ichthyosis refers to a group of skin conditions that cause a widespread persistent dry, scaly skin. This can range from mild to severe and is sometimes associated with skin inflammation (red or darker skin). […] For the milder types of ichthyosis, the main treatments are regular bathing and application of moisturisers. […] When bathing, it is important to use a moisturiser as a soap substitute or dedicated products such as bath oils and shower lotions. […] Moisturising should be done at least twice a day. A person affected by ichthyosis should try several different types of moisturisers before deciding which suits them best. […] Infants with rarer and more severe types of ichthyoses require intensive medical and nursing care. […] Antibiotics or antiseptics may be prescribed for skin infections.

• #10 Ichthyosis Vulgaris: Causes, Symptoms, and Diagnosis

  https://www.healthline.com/health/ichthyosis-vulgaris

  Ichthyosis vulgaris is an inherited or acquired skin condition that occurs when the skin doesnt shed its dead skin cells. […] The majority of cases are mild and confined to specific areas of the body. However, some cases are severe and cover large areas of the body, including the abdomen, back, arms, and legs. […] Symptoms of ichthyosis vulgaris include flaky scalp, itchy skin, polygon-shaped scales on the skin, scales that are brown, gray, or white, severely dry skin, and thickened skin. […] Symptoms of ichthyosis vulgaris are typically worse in winter, when the air is colder and drier. […] In severe cases, ichthyosis vulgaris may also cause deep, painful cracks to develop on the soles of the feet or palms of the hands. […] Theres currently no cure for ichthyosis vulgaris. However, treatment can help you manage your symptoms.

• #11

• #12 Ichthyosis Vulgaris: Causes, Symptoms, and Diagnosis

  https://www.healthline.com/health/ichthyosis-vulgaris

  Ichthyosis vulgaris is an inherited or acquired skin condition that occurs when the skin doesnt shed its dead skin cells. […] The majority of cases are mild and confined to specific areas of the body. However, some cases are severe and cover large areas of the body, including the abdomen, back, arms, and legs. […] Symptoms of ichthyosis vulgaris include flaky scalp, itchy skin, polygon-shaped scales on the skin, scales that are brown, gray, or white, severely dry skin, and thickened skin. […] Symptoms of ichthyosis vulgaris are typically worse in winter, when the air is colder and drier. […] In severe cases, ichthyosis vulgaris may also cause deep, painful cracks to develop on the soles of the feet or palms of the hands. […] Theres currently no cure for ichthyosis vulgaris. However, treatment can help you manage your symptoms.

• #13

  https://journals.lww.com/ijpd/fulltext/2024/25020/clinical_spectrum_of_congenital_ichthyosis_in.4.aspx

  Ichthyosis severity was assessed according to the visual ichthyosis index severity (VIIS) score which measures the degree of erythema, scaling, and hyperkeratosis. […] A history of consanguinity was present in 43.75% of the children in our study, all belonging to Muslim population. […] Winter aggravation of ichthyosis was noted in 84.3% of the patients, out of which the majority were suffering from IV. […] Syndromic ichthyoses are disorders of keratinization, which, in addition to mostly generalized scaling and hyperkeratosis of the skin, also show additional symptoms in other organ systems. […] This study was undertaken to do a clinical-epidemiological evaluation of the children with CI. There is a paucity of Indian studies regarding this. Since inheritance plays a very important role in this condition, detailed genetic counseling is required for affected families. An early diagnosis is equally important for patients, their families, and the treating physician so that necessary interventional and therapeutic measures can be taken for better prognosis and prevention of complications.

• #14 Ichthyosis: Diagnosis, Treatment, and Steps to Take

  https://www.niams.nih.gov/health-topics/ichthyosis/diagnosis-treatment-and-steps-to-take

  Health care providers usually diagnose ichthyosis by: […] In addition, your health care provider may be able to diagnose ichthyosis with a genetic test that detects the mutated gene usually from a blood sample or a swab from the mouth. A genetic counselor or specialist can help you understand the test results. […] There is currently no cure for ichthyosis. The goals of treatment include reducing the redness of the skin, thickness of the scales, and itching. Treatments can include: […] Depending on the type and severity of the disease, doctors may recommend additional treatment with keratolytic topical agents, which can help to loosen scales. However, these can be irritating for some people and have potential side effects if used in large amounts. Talk to your doctor before using any treatment option.

• #15 Ichthyosis

  https://www.nhs.uk/conditions/ichthyosis/

  Most types of ichthyosis cannot be cured, but there are treatments to help the symptoms. […] Treatments may include: moisturising treatments (emollients), such as lotions, creams, ointments and bath oils. […] A skin specialist will talk to you about the different options. […] For more severe ichthyosis symptoms treatment can also include: antibiotics to treat or prevent skin infections, retinoid tablets or creams to help improve the appearance of scaly skin. […] If the ichthyosis symptoms are caused by another health condition or medicine, treating the condition or changing the medicine can improve the symptoms. […] There are things you can do to help ease ichthyosis symptoms at home. […] use emollients on affected skin at least once a day ideally when your skin is still wet after having a bath or shower. […] try different types of emollient to find 1 that works best. […] talk to your skin specialist if any treatments cause irritation. […] do not use soap on affected skin use an emollient instead of soap.

• #16 Inherited ichthyosis: Overview of management – UpToDate

  https://www.uptodate.com/contents/inherited-ichthyosis-overview-of-management

  Inherited ichthyosis: Overview of management […] There are no specific therapies for ichthyosis known to be useful in all patients. Treatment is individualized according to ichthyosis type and personal preference. Skin care measures and use of topical or systemic medications aimed at reducing hyperkeratosis and scaling and hydrating and softening the skin can provide symptomatic relief in most patients. However, more recent understanding of the pathogenesis of ichthyosis subtypes is leading to more targeted treatment with anti-inflammatory biologics, small molecule inhibitors, and gene replacement therapy. […] This topic will discuss the approach to the management of patients with ichthyosis. The pathogenesis, clinical presentation, and diagnosis of specific types of ichthyoses are discussed separately.

• #17 Ichthyosis vulgaris Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/ichthyosis-vulgaris

  Your provider may ask you to use heavy-duty moisturizers. Creams and ointments work better than lotions. Apply these to moist skin immediately after bathing. You should use mild, non-drying soaps. […] Your provider may tell you to use hydrating-moisturizing creams that contain keratolytic chemicals such as lactic acid, salicylic acid, and urea. These chemicals help skin shed normally while retaining moisture. […] Call for an appointment with your provider if: Symptoms continue despite treatment, Symptoms get worse, Skin lesions spread, New symptoms develop.

• #18 Ichthyosis Vulgaris: Causes, Symptoms, Treatment & Recovery

  https://my.clevelandclinic.org/health/diseases/21069-ichthyosis-vulgaris

  Ichthyosis vulgaris is a skin condition that causes excessively dry, thick, scaly skin. […] Treatment usually involves skin care at home to minimize symptoms and prevent infections. Your healthcare provider will instruct you to soak, exfoliate and moisturize your skin frequently. […] The following skin care strategies can help you manage ichthyosis vulgaris: Soak: Take a bath at least once a day, using mild cleansers. Consider soaking in salt water, which will help loosen scales. Pat your skin dry after bathing. Your healthcare provider may suggest adding a tiny amount of bleach to the water to prevent skin infections. Exfoliate: Gently exfoliate your skin to loosen and remove scales and rough patches. Exfoliating will also help moisturizers penetrate the skin. Try using a pumice stone or a slightly abrasive sponge after your skin has soaked. You can also apply products containing exfoliants, like salicylic acid, glycolic acid, lactic acid, alpha hydroxyl acid or urea. Moisturize: Apply lotions or creams to your skin immediately after bathing. Consider thick emollients, such as petroleum jelly. If you have open sores, your dermatologist may recommend applying an emollient before baths. Consider using a humidifier to make the air around you less dry. Avoid irritants: Don’t use products that may irritate the skin, like those with dyes and fragrances. Choose clothing made of soft, moisture-wicking materials.

• #19 Ichthyosis vulgaris Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/ichthyosis-vulgaris

  Your provider may ask you to use heavy-duty moisturizers. Creams and ointments work better than lotions. Apply these to moist skin immediately after bathing. You should use mild, non-drying soaps. […] Your provider may tell you to use hydrating-moisturizing creams that contain keratolytic chemicals such as lactic acid, salicylic acid, and urea. These chemicals help skin shed normally while retaining moisture. […] Call for an appointment with your provider if: Symptoms continue despite treatment, Symptoms get worse, Skin lesions spread, New symptoms develop.

• #20 Ichthyosis – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/ichthyosis/

  Ichthyosis refers to a group of skin conditions that cause a widespread persistent dry, scaly skin. This can range from mild to severe and is sometimes associated with skin inflammation (red or darker skin). […] For the milder types of ichthyosis, the main treatments are regular bathing and application of moisturisers. […] When bathing, it is important to use a moisturiser as a soap substitute or dedicated products such as bath oils and shower lotions. […] Moisturising should be done at least twice a day. A person affected by ichthyosis should try several different types of moisturisers before deciding which suits them best. […] Infants with rarer and more severe types of ichthyoses require intensive medical and nursing care. […] Antibiotics or antiseptics may be prescribed for skin infections.

• #21 Scaly Skin | Ichthyosis Vulgaris – Skinsight

  https://skinsight.com/skin-conditions/ichthyosis-vulgaris/

  Ichthyosis vulgaris should improve by restoring moisture to the skin. Thick creams (eg, CeraVe Moisturizing Cream) and ointments (eg, Vaseline) are better moisturizers than lotions, and they should be applied just after bathing, while the skin is still moist, and at least one other time every day. The following over-the-counter products may be helpful: […] Any cracks in the skin should be treated immediately with an over-the-counter topical antibiotic ointment (eg, Neosporin) to help prevent infection. […] To treat the dry, scaly skin of ichthyosis vulgaris, the medical professional may recommend a topical cream or lotion containing: […] If you develop dry, scaly skin that is not improved by twice daily application of an over-the-counter moisturizer, see a medical professional for evaluation.

• #22 Ichthyosis Vulgaris: Causes, Symptoms, Treatment & Recovery

  https://my.clevelandclinic.org/health/diseases/21069-ichthyosis-vulgaris

  Ichthyosis vulgaris is a skin condition that causes excessively dry, thick, scaly skin. […] Treatment usually involves skin care at home to minimize symptoms and prevent infections. Your healthcare provider will instruct you to soak, exfoliate and moisturize your skin frequently. […] The following skin care strategies can help you manage ichthyosis vulgaris: Soak: Take a bath at least once a day, using mild cleansers. Consider soaking in salt water, which will help loosen scales. Pat your skin dry after bathing. Your healthcare provider may suggest adding a tiny amount of bleach to the water to prevent skin infections. Exfoliate: Gently exfoliate your skin to loosen and remove scales and rough patches. Exfoliating will also help moisturizers penetrate the skin. Try using a pumice stone or a slightly abrasive sponge after your skin has soaked. You can also apply products containing exfoliants, like salicylic acid, glycolic acid, lactic acid, alpha hydroxyl acid or urea. Moisturize: Apply lotions or creams to your skin immediately after bathing. Consider thick emollients, such as petroleum jelly. If you have open sores, your dermatologist may recommend applying an emollient before baths. Consider using a humidifier to make the air around you less dry. Avoid irritants: Don’t use products that may irritate the skin, like those with dyes and fragrances. Choose clothing made of soft, moisture-wicking materials.

• #23 Ichthyosis

  https://www.nhs.uk/conditions/ichthyosis/

  Most types of ichthyosis cannot be cured, but there are treatments to help the symptoms. […] Treatments may include: moisturising treatments (emollients), such as lotions, creams, ointments and bath oils. […] A skin specialist will talk to you about the different options. […] For more severe ichthyosis symptoms treatment can also include: antibiotics to treat or prevent skin infections, retinoid tablets or creams to help improve the appearance of scaly skin. […] If the ichthyosis symptoms are caused by another health condition or medicine, treating the condition or changing the medicine can improve the symptoms. […] There are things you can do to help ease ichthyosis symptoms at home. […] use emollients on affected skin at least once a day ideally when your skin is still wet after having a bath or shower. […] try different types of emollient to find 1 that works best. […] talk to your skin specialist if any treatments cause irritation. […] do not use soap on affected skin use an emollient instead of soap.

• #24 Current Research Reveals New Treatment Options for Ichthyosis

  https://www.dermatologytimes.com/view/current-research-reveals-new-treatment-options-for-ichthyosis

  Patients with ichthyosis will often require antiseptic and topical and/or systemic antibiotics to address the skin infections that can often arise. […] In those patients with a higher incidence of skin infections, a small amount of bleach can be added to the bath which can help reduce the microbial load on the skin and reduce the frequency of skin infections. […] One of the most recent developments in therapy is a topical vitamin A derivative that has shown significant efficacy in helping to quell symptoms. […] In our experience, vitamin A derivatives appear to be exceedingly effective in most cases of moderate to severe ichthyosis and emollients and keratolytics the most effective in ichthyosis vulgaris, the most common form seen in patients, Goldust said. […] Because there is no definitive cure for ichthyosis, the therapy is lifelong, with the best prognosis seen in the inherited ichthyosis vulgaris variant of the disease.

• #25 Ichthyosis Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1198130-treatment

  Oral retinoids display an impressive antikeratinizing action in ichthyosiform dermatoses. Etretinate (1 mg/kg/d) and isotretinoin (2 mg/kg/d) have been shown to reduce scaling, discomfort, and disfigurement. However, when these drugs are discontinued, the ichthyotic skin recurs, thereby necessitating long-term use. […] Patients with epidermolytic hyperkeratosis may develop chronic bacterial infections of the skin necessitating long-term antibiotic therapy. […] The primary aim of eye care in both adults and children with ichthyosis is to maintain moisture, integrity, and clarity of the cornea. When ichthyosis involves the eye or eyelids, there is a high risk of the cornea becoming dry and developing an epithelial defect that may lead to an infection of the cornea. […] In chronic ocular surface disorders associated with ichthyosis, nonpreserved artificial tears (carboxymethylcellulose sodium 0.5-1.0%) and ointment (white petrolatum 56.8%, mineral oil 41.5%) are preferred to prevent complications from dryness and exposure.

• #26 Ichthyosis Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1198130-treatment

  Topical cyclosporine A 2% given 3 times daily has been shown to be beneficial in the treatment of deep stromal keratitis associated with KID syndrome. […] When cicatricial ectropion develops in patients with lamellar ichthyosis despite room humidification and vigorous skin lubrication, the danger of corneal breakdown and perforation is noted. […] Because ichthyosis is primarily a skin disorder, periodic evaluation by a dermatologist is recommended. […] In patients with Refsum disease (ichthyosis and pigmentary retinopathy), chlorophyll in the diet should be excluded (ie, green vegetables [phytanic acid], animal fat [phytol]). […] Because skeletal hyperostosis and arthralgia may occur with long-term oral etretinate and isotretinoin use, this form of treatment is reserved only for those patients with very severe scaling and cosmetic deformity. […] The mainstay of ichthyosis therapy includes removal of surface scales, and application of a water barrier.

• #27 Ichthyosis

  https://www.nhs.uk/conditions/ichthyosis/

  Most types of ichthyosis cannot be cured, but there are treatments to help the symptoms. […] Treatments may include: moisturising treatments (emollients), such as lotions, creams, ointments and bath oils. […] A skin specialist will talk to you about the different options. […] For more severe ichthyosis symptoms treatment can also include: antibiotics to treat or prevent skin infections, retinoid tablets or creams to help improve the appearance of scaly skin. […] If the ichthyosis symptoms are caused by another health condition or medicine, treating the condition or changing the medicine can improve the symptoms. […] There are things you can do to help ease ichthyosis symptoms at home. […] use emollients on affected skin at least once a day ideally when your skin is still wet after having a bath or shower. […] try different types of emollient to find 1 that works best. […] talk to your skin specialist if any treatments cause irritation. […] do not use soap on affected skin use an emollient instead of soap.

• #28 Ichthyosis Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1198130-treatment

  Topical cyclosporine A 2% given 3 times daily has been shown to be beneficial in the treatment of deep stromal keratitis associated with KID syndrome. […] When cicatricial ectropion develops in patients with lamellar ichthyosis despite room humidification and vigorous skin lubrication, the danger of corneal breakdown and perforation is noted. […] Because ichthyosis is primarily a skin disorder, periodic evaluation by a dermatologist is recommended. […] In patients with Refsum disease (ichthyosis and pigmentary retinopathy), chlorophyll in the diet should be excluded (ie, green vegetables [phytanic acid], animal fat [phytol]). […] Because skeletal hyperostosis and arthralgia may occur with long-term oral etretinate and isotretinoin use, this form of treatment is reserved only for those patients with very severe scaling and cosmetic deformity. […] The mainstay of ichthyosis therapy includes removal of surface scales, and application of a water barrier.

• #29 Current Research Reveals New Treatment Options for Ichthyosis

  https://www.dermatologytimes.com/view/current-research-reveals-new-treatment-options-for-ichthyosis

  Research and ongoing clinical trials are giving some hope to those with ichthyosis but for now most people manage their disease symptomatically. […] However, for the time being, most patients can manage their disease only symptomatically. […] Currently, there are no FDA-approved treatments for ichthyosis; however, we are hopeful that this may change in the near future, Mohamad Goldust, MD, said. […] Traditional mainstay treatment approaches for ichthyosis are focused on hydrating the skin with emollients and moisturizers with the goal of trapping moisture within the skin, which can lead to an amelioration of the presenting dryness and scaling, in addition to using the keratolytic agents. […] Taking frequent and long baths is an important cornerstone of therapy that can help soften and release the excessive scaling typically seen in patients.

• #30 New developments in the molecular treatment of ichthyosis: review of the literature | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-022-02430-6

  Ichthyosis covers a wide spectrum of diseases affecting the cornification of the skin. Current treatment for ichthyosis is focused on symptom relief and includes emollients, keratolytics, and oral retinoids. The efficacy of these treatments is moderate and is usually not effective on inflammation of the skin. Promising developments have been made in pathogenesis-based therapies, such as enzyme replacement therapy and gene therapy, and recent findings concerning the immune profile of ichthyosis patients have given new ground to repurpose biologicals. The aim of this review is to provide an overview of the current status on pathogenesis-based therapy for ichthyosis. Biological therapy includes a wide range of products, such as monoclonal antibodies that aim on targeting specific marks, e.g., tumor necrosis factor alpha (TNF-), interleukin-13 (IL-13), interleukin-17 (IL-17) and interleukin-23 (IL-23). The use of biological therapies could be beneficial in the treatment of several ichthyosis subtypes. However, the response is mostly seen as reduction of the inflammatory component and pruritus. The effect on scaling seems to be more variable. A larger randomized controlled trial (RCT) and/or large open-label cohort is necessary to rule out a possible publication bias and result in more specific data and correlation with genetic mutations and immunological profile. Furthermore, there are case reports that describe a good response at first, but a decline after several weeks. Perhaps biological therapies in ichthyosis patients require an increased dosage and/or interval compared to the currently used regimen indicated for psoriasis and atopic dermatitis.

• #31 Congenital ichthyosis – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/congenital-ichthyosis/

  Innovative therapies, including biologic agents, small molecules, and gene therapy, are being explored as potential treatments for ichthyosis. Although these therapies are still in the experimental phase, they hold promise for more targeted and effective management of the condition. […] Patients with ichthyosis often face complications such as chronic skin infections and ocular issues. Long-term antibiotic therapy may be necessary for those with recurrent bacterial infections. For ocular complications, maintaining corneal moisture and integrity is crucial, often requiring the use of nonpreserved artificial tears and ointments. […] While there is no cure for congenital ichthyosis, advancements in treatment options provide hope for better management of the condition. From traditional emollients and keratolytics to advanced systemic therapies and emerging gene therapies, patients have more options than ever before.

• #32 Ichthyosis in the Newborn

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3758581/

  Management of neonates with ichthyosis necessitates a multidisciplinary approach, with family members, nursing staff, neonatologists, dermatologists and, in some cases other specialists, all serving as active participants. […] The majority of therapies aimed at improving barrier function are fairly simple and straightforward. […] The mainstays of management include providing a humidified, temperature-controlled environment via an isolette, daily bathing with only water or mild cleanser, and frequent, liberal applications of bland emollients such as petrolatum-based products. […] Because many infants with ichthyosis are born prematurely, the primary risks or complications are oftentimes those related to prematurity rather than skin; therefore, many of the medical concerns are similar to those that neonatologists manage on a routine basis.

• #33 Reddit – The heart of the internet

  https://www.reddit.com/r/ichthyosis/comments/rmde1i/my_ichthyosis_vulgaris_care_routine/

  If you have a bathroom heater, use it. I let the bathroom get nice and warm, and if it’s going to be a short shower I don’t turn on the vent until I get out. I want the air warm and full of moisture when I get out of the shower. […] In the shower I use Dove Deep Moisture Hydrating Body Wash. I keep the water body temp warm, hot or cold water will strip the moisture right out of your skin! With a scrubby lufa I scrub head to toe (but not too hard!), being careful to only exfoliate as much as I think I actually need. Over exfoliating will not help your skin retain moisture! Extra notes below if you’re starting new and need more intense exfoliation tips to get over the initial scale build up. […] I don’t linger in the shower, long showers are not our friend. I get out, towel off fast, and IMMEDIATELY put on Eucerin Advance Repair body lotion head to toe (do NOT wait, your skin is losing moisture by the second!). This lotion has the holy trifecta of IV moisturizers: ceremides, glycerine, and urea (don’t worry, it’s synthetic!). Specifically „Advanced Repair” formula is critical, other Eucerin formulas don’t have the same combo of ingredients that are so perfect for us. Then I wrap up in a fluffy robe for at least a few minutes or more if I can, to lock all that lotion in while it works.

• #34 Reddit – The heart of the internet

  https://www.reddit.com/r/ichthyosis/comments/rmde1i/my_ichthyosis_vulgaris_care_routine/

  Do not EVER skip the moisturizing step after exfoliating. When we scrub we take off what little moisture barrier we have, so if you don’t restore it after your shower with moisturizer, you’ll just dry out and crack even worse than before. […] For my face I use Cereve PM with ceremides (morning and night), plus also Eucerin Dry Skin Replenishing Face Cream during the winter (it has Urea and Lactate), but be careful not to over do it with urea on your face, it can be a little harsh if you use it too much. I use it every couple days and it makes a world of difference. At night I usually use a Olay Retinol cream, because of my age but also because retinol is supposedly helpful for our skin. Also often instead of a wash with water I use micellar water, I find it to be more gentle on the moisture barrier. Sometimes I don’t wash at all for 24 hours and just slather heavily with Cereve PM, I can just strangely tell when my skin as been over washed, it feels kinda raw and irritated. I only actually wash my face with cleanser every other day (when I wash my hair), I use CeraVe Hydrating Face Wash because it has ceremides, and another important IV ingredient, glycerine!

• #35 Reddit – The heart of the internet

  https://www.reddit.com/r/ichthyosis/comments/rmde1i/my_ichthyosis_vulgaris_care_routine/

  In the worst of winter I also do a full body moisturizer right before bed (on top of doing that after every shower) so it can really soak in over night under the sheets (better for some reason). I use the Eucerin A.R. full body plus Amlactin on any rough skin spots. […] Cold winter air is the enemy! Protect your calves with tall socks, protect your hands with gloves. Any inch of skin you can cover will help keep in that fragile moisture barrier. […] If your skin is burning that’s because your skin is so dry and built up that it’s cracked and the acidic lotion is getting into the micro cuts in your skin. If that happens and you can’t stand it, you can get ahead of the cracked skin with basic soak and scrub (tub soaks work better than long showers if that’s an option for you, tub soaks can also substitute the ammonium lactate lotion if you can’t find it). Make SURE you use the Eucerin A.R. after, or a comparable ingredient product, this will help your skin heal. If you improve your skin with the soak and scrub methods you should be able to eventually start using the Amlactin without burning.

• #36 Reddit – The heart of the internet

  https://www.reddit.com/r/ichthyosis/comments/rmde1i/my_ichthyosis_vulgaris_care_routine/

  Do not EVER skip the moisturizing step after exfoliating. When we scrub we take off what little moisture barrier we have, so if you don’t restore it after your shower with moisturizer, you’ll just dry out and crack even worse than before. […] For my face I use Cereve PM with ceremides (morning and night), plus also Eucerin Dry Skin Replenishing Face Cream during the winter (it has Urea and Lactate), but be careful not to over do it with urea on your face, it can be a little harsh if you use it too much. I use it every couple days and it makes a world of difference. At night I usually use a Olay Retinol cream, because of my age but also because retinol is supposedly helpful for our skin. Also often instead of a wash with water I use micellar water, I find it to be more gentle on the moisture barrier. Sometimes I don’t wash at all for 24 hours and just slather heavily with Cereve PM, I can just strangely tell when my skin as been over washed, it feels kinda raw and irritated. I only actually wash my face with cleanser every other day (when I wash my hair), I use CeraVe Hydrating Face Wash because it has ceremides, and another important IV ingredient, glycerine!

• #37 Reddit – The heart of the internet

  https://www.reddit.com/r/ichthyosis/comments/rmde1i/my_ichthyosis_vulgaris_care_routine/

  I didn’t used to have this problem, so it’s new to me and I’m still working on figuring it out. But since it became an issue, I’ve improved it greatly with Nizoral Anti Dandruff Shampoo a couple times a week, and Earth Science Ceremide shampoo and conditioner every other day. I wash my hair every other day for the sake of my long hair and my sanity, but I suspect my scalp would be happier if I was shampooing daily. When it gets bad I also literally put Eucerin Advanced Repair lotion straight on my scalp 5-10 minutes before I shower and that helps a ton. […] Throughout the day I use amlactin on my hands pretty much any time they get wet. They’re prone to this issue called Keratolysis exfoliativa that kind looks like dry and peeling blisters. Regular Amlactin is the only thing that’s ever prevented it. I also like Eucerin Advanced Repair Cream for my hands (it’s heavier, lasts longer).

• #38 Ichthyosis Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1198130-treatment

  Oral retinoids display an impressive antikeratinizing action in ichthyosiform dermatoses. Etretinate (1 mg/kg/d) and isotretinoin (2 mg/kg/d) have been shown to reduce scaling, discomfort, and disfigurement. However, when these drugs are discontinued, the ichthyotic skin recurs, thereby necessitating long-term use. […] Patients with epidermolytic hyperkeratosis may develop chronic bacterial infections of the skin necessitating long-term antibiotic therapy. […] The primary aim of eye care in both adults and children with ichthyosis is to maintain moisture, integrity, and clarity of the cornea. When ichthyosis involves the eye or eyelids, there is a high risk of the cornea becoming dry and developing an epithelial defect that may lead to an infection of the cornea. […] In chronic ocular surface disorders associated with ichthyosis, nonpreserved artificial tears (carboxymethylcellulose sodium 0.5-1.0%) and ointment (white petrolatum 56.8%, mineral oil 41.5%) are preferred to prevent complications from dryness and exposure.

• #39

  https://www.ichthyosis.org.uk/pages/faqs/category/treatments-and-caring-for-ichthyosis

  The primary aim of eye care for adults and children with ichthyosis is to maintain moisture, integrity and clarity of the cornea (transparent part of the eye) and surface of the eye. There is a high risk of the cornea becoming dry and developing an epithelial defect (a breach in its protective surface) that may result in an infection of the cornea. […] If incomplete blink or eyelid closure exists, then regular eye lubrication with artificial tears is essential. Preservatives are a legal requirement for eye drops in multi-dose containers and may be necessary for stabilization of eye drops. However, they often act in a non-specific manner as detergents or for unknown reasons occasionally cause side-effects on the surface of the eye such as redness, inflammation, irritation and discomfort. […] Conservative treatment with intensive ocular lubricants, eyelid emollients and massage can result in improvement of eyelid ectropion and to some degree retraction of the eyelid. Regular stretching of the eyelid skin and application of eyelid emollients can avoid the need for eyelid surgery in mild forms of congenital ichthyosis.

• #40 Ichthyosis Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1198130-treatment

  Oral retinoids display an impressive antikeratinizing action in ichthyosiform dermatoses. Etretinate (1 mg/kg/d) and isotretinoin (2 mg/kg/d) have been shown to reduce scaling, discomfort, and disfigurement. However, when these drugs are discontinued, the ichthyotic skin recurs, thereby necessitating long-term use. […] Patients with epidermolytic hyperkeratosis may develop chronic bacterial infections of the skin necessitating long-term antibiotic therapy. […] The primary aim of eye care in both adults and children with ichthyosis is to maintain moisture, integrity, and clarity of the cornea. When ichthyosis involves the eye or eyelids, there is a high risk of the cornea becoming dry and developing an epithelial defect that may lead to an infection of the cornea. […] In chronic ocular surface disorders associated with ichthyosis, nonpreserved artificial tears (carboxymethylcellulose sodium 0.5-1.0%) and ointment (white petrolatum 56.8%, mineral oil 41.5%) are preferred to prevent complications from dryness and exposure.

• #41

  https://www.ichthyosis.org.uk/pages/faqs/category/treatments-and-caring-for-ichthyosis

  The primary aim of eye care for adults and children with ichthyosis is to maintain moisture, integrity and clarity of the cornea (transparent part of the eye) and surface of the eye. There is a high risk of the cornea becoming dry and developing an epithelial defect (a breach in its protective surface) that may result in an infection of the cornea. […] If incomplete blink or eyelid closure exists, then regular eye lubrication with artificial tears is essential. Preservatives are a legal requirement for eye drops in multi-dose containers and may be necessary for stabilization of eye drops. However, they often act in a non-specific manner as detergents or for unknown reasons occasionally cause side-effects on the surface of the eye such as redness, inflammation, irritation and discomfort. […] Conservative treatment with intensive ocular lubricants, eyelid emollients and massage can result in improvement of eyelid ectropion and to some degree retraction of the eyelid. Regular stretching of the eyelid skin and application of eyelid emollients can avoid the need for eyelid surgery in mild forms of congenital ichthyosis.

• #42

  https://www.ichthyosis.org.uk/pages/faqs/category/treatments-and-caring-for-ichthyosis

  It is therefore rare nowadays to occur. In adults, a contrary response to evaporative-type dry eye is reflex tearing. This may be considered as the body’s response in attempting to lubricate the drying surface of the eye. […] Individuals who experience this may not have significant symptoms of ocular discomfort or redness of the eye. Reflex tearing may occur outdoors in cold and windy weather or in a dry, air-conditioned environment. […] Eyelid skin grafting is generally undertaken where symptomatic corneal exposure or watering persists despite adequate conservative skin treatments. It should also ideally be undertaken before scarring of the ocular surface occurs. […] I believe bandages are beneficial for treating adults and children with ichthyosis and would go as far as to say that they are essential for treatment of severe forms of ichthyosis. […] The cream with 10% Urea and 5% Lactic Acid is recommended for adults and children over 3 years old.

• #43 Harlequin Ichthyosis Treatment & Management: Approach Considerations, Medical Care, Consultations

  https://emedicine.medscape.com/article/1111503-treatment

  Maintain a sterile environment to avoid infection. […] Continue careful attention to skin care and use of emollients during retinoid therapy. […] Infants with harlequin ichthyosis can be successfully breastfed or bottle-fed as the eclabium improves. […] Early formation of a multidisciplinary team is recommended and may include the following: Neonatologist, Dermatologist, Medical geneticist, Ophthalmologist, Ear-nose-throat specialist, Plastic surgeon, Dietician, Social worker, Occupational therapist, Physical therapist. […] Hyperkeratosis causing constriction of limbs, digits, or nasal obstruction may need to be treated surgically. […] Infants are discharged from the hospital when their cutaneous symptoms are improving, feeding and weight gain are established, and they are free of infection. […] The primary care physician should closely monitor the infants for growth, development, social issues, and skin surveillance. […] Follow-up with an ophthalmologist is required. Recurrent exposure keratitis can be a problem as a result of persistent ectropion.

• #44

• #45 Ichthyosis: Diagnosis, Treatment, and Steps to Take

  https://www.niams.nih.gov/health-topics/ichthyosis/diagnosis-treatment-and-steps-to-take

  Nurse educators, who specialize in helping people to understand their overall condition and to set up their treatment plans. […] Depending on the type and severity of the disorder, you may find living with ichthyosis to be challenging. However, the following self-care tips may help you manage the disease, improve your health, and enjoy a better quality of life: […] Take baths to add more moisture to the skin and help remove the scales before applying topical agents. […] Find a supportive community or join an online support group focused on ichthyosis. Some people may find it helpful to speak to a mental health professional about coping with the disorder.

• #46 Fragomented Health Care Delivery in Ichthyosis | Actas Dermo-Sifiliográficas

  http://www.actasdermo.org/en-fragomented-health-care-delivery-in-articulo-S1578219015001560

  Congenital ichthyosis is a very rare genetic diseasean estimated 294 patients in Spain are thought to be affected. The disease usually has a large impact on patients quality of life because, regardless of the type of ichthyosis, patients may experience intractable pruritus, hyperhidrosis, failure to thrive during childhood, cicatricial alopecia, repeated keratitis, conductive hearing loss, and social problems in addition to cutaneous manifestations. As a result, in addition to dermatologists, most patients need to be attended by other specialists such as ophthalmologists, ear-nose-throat specialists, endocrinologists, nutrition experts, and psychologists. […] Our survey shows that very few physicians have experience with a significant number of patients with ichthyosis, and that it is likely that most patients are not attended regularly by other specialists who could help them with some of their possible comorbidities.

• #47 Harlequin Ichthyosis Treatment & Management: Approach Considerations, Medical Care, Consultations

  https://emedicine.medscape.com/article/1111503-treatment

  Newborns with harlequin ichthyosis require management in a level III neonatal intensive care unit. […] Patient’s airway, breathing, and circulation stability to be accessed after delivery. Early intubation may be required. […] Babies require intravenous access. Peripheral access may be difficult and umbilical cannulation may be necessary. […] Bathe infants twice daily and use frequent wet sodium chloride compresses followed by application of bland lubricants to soften hard skin. […] According to Rajpopat et al, early retinoid treatment (by day 7) may require prompt consideration, as these medications can take some days to obtain. […] Tazarotene, a topical retinoid, has been reported to be beneficial. […] Neonates with harlequin ichthyosis initially do not feed well and may require tube feeding.

• #48 Lamellar ichthyosis Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/lamellar-ichthyosis

  Lamellar ichthyosis (LI) is a rare skin condition. It appears at birth and continues throughout life. […] Collodion babies usually need to stay in the neonatal intensive care unit (NICU). They are placed in a high-humidity incubator. They will need extra feedings. Moisturizers need to be applied to the skin. After the collodion membrane is shed, babies can usually go home. […] Lifelong care of the skin involves keeping the skin moist to minimize the thickness of the scales. Measures include: Moisturizers applied to the skin, Medicines called retinoids that are taken by mouth in severe cases, High-humidity environment, Bathing to loosen scales.

• #49 Harlequin Ichthyosis Treatment & Management: Approach Considerations, Medical Care, Consultations

  https://emedicine.medscape.com/article/1111503-treatment

  Newborns with harlequin ichthyosis require management in a level III neonatal intensive care unit. […] Patient’s airway, breathing, and circulation stability to be accessed after delivery. Early intubation may be required. […] Babies require intravenous access. Peripheral access may be difficult and umbilical cannulation may be necessary. […] Bathe infants twice daily and use frequent wet sodium chloride compresses followed by application of bland lubricants to soften hard skin. […] According to Rajpopat et al, early retinoid treatment (by day 7) may require prompt consideration, as these medications can take some days to obtain. […] Tazarotene, a topical retinoid, has been reported to be beneficial. […] Neonates with harlequin ichthyosis initially do not feed well and may require tube feeding.

• #50 Ichthyosis in the Newborn

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3758581/

  Management of neonates with ichthyosis necessitates a multidisciplinary approach, with family members, nursing staff, neonatologists, dermatologists and, in some cases other specialists, all serving as active participants. […] The majority of therapies aimed at improving barrier function are fairly simple and straightforward. […] The mainstays of management include providing a humidified, temperature-controlled environment via an isolette, daily bathing with only water or mild cleanser, and frequent, liberal applications of bland emollients such as petrolatum-based products. […] Because many infants with ichthyosis are born prematurely, the primary risks or complications are oftentimes those related to prematurity rather than skin; therefore, many of the medical concerns are similar to those that neonatologists manage on a routine basis.

• #51 Care of infants with ichthyosis requires ‘all hands on deck’ | MDedge

  https://blogs.the-hospitalist.org/content/care-infants-ichthyosis-requires-all-hands-deck

  CHICAGO The neonatal period and early infancy are especially critical for patients with ichthyosis, because compromised barrier function increases risk for morbidity and mortality. […] Youre going to want to get all hands on deck for the care of these patients. And dont forget about the family involve them in the care as much as possible. Reassure them; normalize their feelings, acknowledge them. […] Babies with moderate to severe congenital ichthyosis are typically cared for in the neonatal ICU of a tertiary care center by a multidisciplinary team consisting of neonatology, dermatology, nursing, nutrition, and genetics, as well as ophthalmology, otolaryngology, orthopedics, plastic surgery, and spiritual/religious services in many cases. […] They need to be in an isolette, generally with humidity somewhere between 50% and 70% you dont want it too high, because they can overheat. Its also important to get them out of the isolette and into an open crib when theyre ready. That can help with bonding and has been shown to decrease hospital stay. […] Pain control is an imperative aspect of pain management. […] Look at heart rate, blood pressure, crying, level of arousal, and have a low threshold to treat for pain, especially prior to changing dressings. […] Families want to know that pain is being adequately controlled.

• #52 Newborn with Harlequin Ichthyosis and the Nursing Care – Journal of Pediatric Emergency and Intensive Care Medicine

  https://caybdergi.com/articles/newborn-with-harlequin-ichthyosis-and-the-nursing-care/doi/cayd.78941

  Harlequin ichthyosis is the severest form of non-bullous ichthyosis, which is quite uncommon in newborns, and is usually characterized by fatal extreme keratinization of the skin. […] The present case report concerning a newborn with harlequin ichthyosis, which is an uncommon condition requiring intensive care, aimed to guide the care process by discussing the experiences over the nursing care period. […] Within the scope of nursing process, the data were collected, the diagnoses were identified, interventions were planned and implemented, and the outcomes of these implementations were evaluated. […] Life expectancy of the patient, who was expected to die within a few days after birth, was extended by efficient and preventive nursing care, but these implementations have not been sufficient to prolong survival.

• #53 Newborn with Harlequin Ichthyosis and the Nursing Care – Journal of Pediatric Emergency and Intensive Care Medicine

  https://caybdergi.com/articles/newborn-with-harlequin-ichthyosis-and-the-nursing-care/doi/cayd.78941

  Specific to this case, we can say that death could be postponed by nursing care. Palliative and preventive care is recommended when harlequin ichthyosis is encountered. […] Nursing care process for Baby Y, who has been monitored in the intensive care unit, lasted 51 days. […] Within nursing care process consisting of palliative and preventive care, data were collected from the patient, nursing diagnoses were made, interventions were planned and implemented, and the outcomes were evaluated. […] The major problem encountered in the first days in Baby Y was impaired tissue integrity. […] Given that such patients generally die in the first days of life due to sepsis, dehydration and impaired thermoregulation, Baby Y was kept in a humidified incubator. […] Adequate fluid replacement was performed to hydrate the patient.

• #54 Newborn with Harlequin Ichthyosis and the Nursing Care – Journal of Pediatric Emergency and Intensive Care Medicine

  https://caybdergi.com/articles/newborn-with-harlequin-ichthyosis-and-the-nursing-care/doi/cayd.78941

  Nursing interventions were performed to provide the infant with food necessary/adequate for his age and requirements. […] Within the scope of nursing care, harmful plaques were removed, oral cavity was controlled frequently, and oral care was performed regularly to prevent secondary infections. […] Depending on the diagnosis of the risk of ineffective breathing pattern, which resulted from adequate expansion of the chest due to impaired skin elasticity, breathing was monitored frequently, SPO2 was also monitored, and aspiration was performed when necessary in order to maintain effective breathing pattern. […] Since the hyper keratinized skin of the newborn, who was at risk for imbalanced body temperature, hindered normal heat loss, it was aimed to keep the body temperature within the normal limits (36-37.2 C).

• #55 Newborn with Harlequin Ichthyosis and the Nursing Care – Journal of Pediatric Emergency and Intensive Care Medicine

  https://caybdergi.com/articles/newborn-with-harlequin-ichthyosis-and-the-nursing-care/doi/cayd.78941

  In order to prevent risk for bleeding from deep fissures found on the skin, the skin was moistened every day at 3-hour intervals and bleeding profile was monitored. […] Despite the precise treatment-care process, hyperthermia was encountered more frequently in the next days. […] The present case includes significant ethical issues concerning the beginning and end of life. […] Patients with harlequin ichthyosis need to be followed in full-equipped intensive care units and by multidisciplinary approach. […] Life expectancy for Baby Y, who received efficient preventive and nursing care, was prolonged and the interventions allowed him to survive for 51 days.

• #56 Ichthyosis – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/ichthyosis/

  Ichthyosis can have a significant psychological and emotional impact on those affected by it. It is important that you speak to the dermatology team so they can direct you or your child to other teams who can support your needs. […] Parents will be taught how to cope with the day-to-day management.

• #57 The Burden of Autosomal Recessive Congenital Ichthyoses on Patients and their Families: An Italian Multicentre Study | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3822

  Autosomal recessive congenital ichthyoses (ARCI) are characterized by generalized skin scaling, hyperkeratosis, erythroderma, and disabling features affecting the skin (palmoplantar keratoderma, fissures, pain, itch), eyes, ears, and joints. […] Disease management requires a multidisciplinary team with periodic consultations. No curative therapy exists: available treatments are merely symptomatic and need lifelong administration. […] Overall, skin and extracutaneous manifestations profoundly alter patients physical appearance and affect their self-perception. […] The results can be used to improve care plans, including psychological support and therapeutic patient education. […] The current findings point to the need for improved management in this often neglected, but relatively common complication of ichthyosis.

• #58 The Burden of Autosomal Recessive Congenital Ichthyoses on Patients and their Families: An Italian Multicentre Study | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3822

  These results emphasize the need to offer psychological support to both patients and family members aimed at reducing disease burden and improving QoL. […] A primary role is obviously played by the psychologist, but other multidisciplinary team members, in particular dermatologists, dedicated nurses, and social workers, should also actively participate. […] Finally, these findings could be relevant in guiding the choice of outcome measures in future observational clinical studies and randomized controlled trials.

• #59 Ichthyosis – British Skin Foundation

  https://knowyourskin.britishskinfoundation.org.uk/condition/ichthyosis/

  For the milder types of ichthyosis, the main treatments are regular bathing and application of moisturisers. […] When bathing, it is important to use a moisturiser as a soap substitute or dedicated products such as bath oils and shower lotions. […] Moisturising should be done at least twice a day. A person affected by ichthyosis should try several different types of moisturisers before deciding which suits them best. […] Infants with rarer and more severe types of ichthyoses require intensive medical and nursing care. […] Children and adults affected by these severe types may be given a trial of retinoid (synthetic vitamin A) treatment. […] Antibiotics or antiseptics may be prescribed for skin infections. […] Ichthyosis can have a significant psychological and emotional impact on those affected by it. It is important that you speak to the dermatology team so they can direct you or your child to other teams who can support your needs.

• #60 Ichthyosis: Diagnosis, Treatment, and Steps to Take

  https://www.niams.nih.gov/health-topics/ichthyosis/diagnosis-treatment-and-steps-to-take

  Nurse educators, who specialize in helping people to understand their overall condition and to set up their treatment plans. […] Depending on the type and severity of the disorder, you may find living with ichthyosis to be challenging. However, the following self-care tips may help you manage the disease, improve your health, and enjoy a better quality of life: […] Take baths to add more moisture to the skin and help remove the scales before applying topical agents. […] Find a supportive community or join an online support group focused on ichthyosis. Some people may find it helpful to speak to a mental health professional about coping with the disorder.

• #61 Ichthyosis in the Newborn

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3758581/

  Parents and caregivers must be educated about skin care and provided with written instructions regarding bathing and emollient use. […] Finally, as mentioned above, we encourage referral to the Foundation for Ichthyosis and Related Skin Types (FIRST), which can serve as a means of continued education and support for patients and families.

• #62

• #63 Ichthyosis – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/ichthyosis/

  Ichthyosis can have a significant psychological and emotional impact on those affected by it. It is important that you speak to the dermatology team so they can direct you or your child to other teams who can support your needs. […] Parents will be taught how to cope with the day-to-day management.

• #64 Care of infants with ichthyosis requires ‘all hands on deck’ | MDedge

  https://blogs.the-hospitalist.org/content/care-infants-ichthyosis-requires-all-hands-deck

  CHICAGO The neonatal period and early infancy are especially critical for patients with ichthyosis, because compromised barrier function increases risk for morbidity and mortality. […] Youre going to want to get all hands on deck for the care of these patients. And dont forget about the family involve them in the care as much as possible. Reassure them; normalize their feelings, acknowledge them. […] Babies with moderate to severe congenital ichthyosis are typically cared for in the neonatal ICU of a tertiary care center by a multidisciplinary team consisting of neonatology, dermatology, nursing, nutrition, and genetics, as well as ophthalmology, otolaryngology, orthopedics, plastic surgery, and spiritual/religious services in many cases. […] They need to be in an isolette, generally with humidity somewhere between 50% and 70% you dont want it too high, because they can overheat. Its also important to get them out of the isolette and into an open crib when theyre ready. That can help with bonding and has been shown to decrease hospital stay. […] Pain control is an imperative aspect of pain management. […] Look at heart rate, blood pressure, crying, level of arousal, and have a low threshold to treat for pain, especially prior to changing dressings. […] Families want to know that pain is being adequately controlled.

• #65 Harlequin Ichthyosis: Definition, Symptoms, Treatment, and Prognosis

  https://www.healthline.com/health/harlequin-ichthyosis

  Harlequin ichthyosis is a serious condition, but medical advances have greatly improved the outlook for babies born with it. […] A newborn with Harlequin ichthyosis requires neonatal intensive care, which may include spending time in a heated incubator with high humidity. […] But early, intensive treatment is vital. […] Theres no cure for Harlequin ichthyosis, so management becomes a crucial part of the equation after initial treatment. And its all about the skin. […] For maximized benefit, apply ointments and moisturizers right after a bath or shower, while the skin is still moist. […] If you have a school-aged child, make sure to let the school nurse know about their condition and any treatment they may need throughout the school day. […] Living with Harlequin ichthyosis or raising a child with the condition can feel overwhelming at times. The Foundation for Ichthyosis and Related Skin Types offers support group listings, virtual and in-person meetings with others in the community, treatment tips, and more. […] Harlequin ichthyosis is a chronic disease that will always require careful monitoring, skin protection, and topical treatments. But children with a Harlequin ichthyosis diagnosis in recent years have a much better outlook than those born in previous decades.

• #66 Ichthyosis Vulgaris: Causes, Symptoms, and Diagnosis

  https://www.healthline.com/health/ichthyosis-vulgaris

  Regularly apply moisturizers that have urea or propylene glycol in them. These chemicals will help your skin stay moist. […] Using a humidifier in your home will add moisture into the air and keep your skin from drying out. […] Your doctor may also prescribe specialized creams or ointments to help moisturize the skin, get rid of dead skin, and control inflammation and itching. […] Living with ichthyosis vulgaris and similar skin conditions is difficult at times, especially for children. […] The key to living with this condition is learning to make management of this disease part of your daily routine.

• #67 Harlequin Ichthyosis: Symptoms, Causes, and Treatment Options – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/harlequin-ichthyosis-symptoms-causes-and-treatment-options/

  Managing Harlequin ichthyosis requires a combination of medical treatments and home care strategies. Regular moisturizing is essential to keep the skin hydrated and reduce scaling. Using gentle cleansers and avoiding hot water can prevent further irritation. Applying emollients after bathing helps lock in moisture. Wearing breathable fabrics minimizes skin irritation, while staying hydrated and using humidifiers supports skin health from the inside out. Avoiding harsh chemicals and practicing sun protection are also crucial to prevent skin damage. […] Telemedicine offers a convenient way to seek medical help for Harlequin ichthyosis. Patients can consult healthcare providers from home, ensuring timely symptom management and treatment adjustments. Early diagnosis and consistent management are key to improving the quality of life for those living with this condition.

• #68 Harlequin Ichthyosis: Symptoms, Causes, and Treatment Options – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/harlequin-ichthyosis-symptoms-causes-and-treatment-options/

  Living with Harlequin ichthyosis requires ongoing care and attention to skin health. Establishing a daily skincare routine that includes moisturizing and gentle cleansing is essential. Staying informed about new treatments and maintaining regular communication with healthcare providers helps manage symptoms effectively. Support groups and online communities offer emotional support and practical advice from others living with the condition. Prioritizing self-care and seeking professional guidance when needed can significantly enhance quality of life.

• #69 Ichthyosis vulgaris Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/ichthyosis-vulgaris

  Your provider may ask you to use heavy-duty moisturizers. Creams and ointments work better than lotions. Apply these to moist skin immediately after bathing. You should use mild, non-drying soaps. […] Your provider may tell you to use hydrating-moisturizing creams that contain keratolytic chemicals such as lactic acid, salicylic acid, and urea. These chemicals help skin shed normally while retaining moisture. […] Call for an appointment with your provider if: Symptoms continue despite treatment, Symptoms get worse, Skin lesions spread, New symptoms develop.

• #70 Harlequin Ichthyosis Treatment & Management: Approach Considerations, Medical Care, Consultations

  https://emedicine.medscape.com/article/1111503-treatment

  Maintain a sterile environment to avoid infection. […] Continue careful attention to skin care and use of emollients during retinoid therapy. […] Infants with harlequin ichthyosis can be successfully breastfed or bottle-fed as the eclabium improves. […] Early formation of a multidisciplinary team is recommended and may include the following: Neonatologist, Dermatologist, Medical geneticist, Ophthalmologist, Ear-nose-throat specialist, Plastic surgeon, Dietician, Social worker, Occupational therapist, Physical therapist. […] Hyperkeratosis causing constriction of limbs, digits, or nasal obstruction may need to be treated surgically. […] Infants are discharged from the hospital when their cutaneous symptoms are improving, feeding and weight gain are established, and they are free of infection. […] The primary care physician should closely monitor the infants for growth, development, social issues, and skin surveillance. […] Follow-up with an ophthalmologist is required. Recurrent exposure keratitis can be a problem as a result of persistent ectropion.

• #71 Evaluation of Patients with Ichthyosis Followed in a Neonatal Intensive Care Unit: A Single Center Experience Journal of Clinical Practice and Research

  https://jcpres.com/article/14

  Ichthyosis is a keratinization disorder that is characterized by a defective skin barrier and inability to retain water in the skin. Ichthyosis is extremely rare and mostly hereditary, and its manifestations typically involve dryness, scaling, and hyperkeratosis. […] Patients with ichthyosis should be protected from infection and hypernatremic dehydration during the neonatal period. After diagnosis, patients with ichthyosis should be screened for concomitant genetic disorders and their families should be referred to genetic counseling. […] In managing this disorder, genetic analysis and counseling are as important as proper skin care, hydration, and infection prevention and should not be overlooked.

• #72 Ichtyoses – Therapeutics in Dermatology

  https://www.therapeutique-dermatologique.org/spip.php?article1683

  Ichthyosis is a disorder of cornification, as it results from a disturbance of the formation of the outermost skin barrier (stratum corneum). […] Dermatologists can often diagnose this disorder clinically, but a skin biopsy is usually performed to confirm the diagnosis and the type of ichthyosis. […] There is no cure for inherited ichthyoses. Severe disease forms, like collodion baby and harlequin baby, are life-threatening Dermatological emergencies requiring management in a neonatal intensive care unit, which includes placement in a high-humidity incubator, continuous clinical monitoring, nutritional and ventilatory support, careful skin care and prevention of infection. […] General skin care measures recommended for patients with ichthyosis include: Use of non-soap cleansers, Regular application of moisturising creams containing keratolytics (e.g.: urea, salicylic acid, glycolic acid, lactic acid).

• #73 Ichtyoses – Therapeutics in Dermatology

  https://www.therapeutique-dermatologique.org/spip.php?article1683

  Every patient with suspected ichthyosis should see a Dermatologist for adequate diagnosis and treatment. […] Ichthyosis patients therefore require daily skin care, including regular application of moisturising creams containing keratolytics. […] Secondary skin and/or systemic infections must be prevented and promptly treated.

• #74

  https://www.nursingcenter.com/journalarticle?Article_ID=3158437&Journal_ID=675992&Issue_ID=3158220

  Background: There exists a group of rare, inherited scaly skin disorders, generally termed ichthyosis, that can be evident in the infant at the time of birth. […] Purpose: This article explores the 3 most common nonsyndromic forms of ichthyosis seen in neonates as follows: X-linked recessive, lamellar, and bullous congenital ichthyosiform erythroderma. Moreover, harlequin ichthyosis, a lamellar subtype, is highlighted for being the most severe, clinically problematic, and often lethal form of the disorder. […] Implications for Practice: Considerations for the neonatal nurse practitioner charged with caring for these patients, including specific recommendations for care in the clinical setting, are discussed. Additionally, genetic counseling and the risks of reoccurrence are explored. […] Findings/Results: A description of each of these types and their incidence is included, followed by an explanation of the genetic mutations causing them. The phenotypes and natural history are reviewed, as is expected management of the disorder throughout the patient’s lifetime.

• #75 Alan Mendelsohn, MD, Shares Insights Into Congenital Ichthyosis Patient Care and the Development of TMB-001

  https://www.dermatologytimes.com/view/alan-mendelsohn-md-shares-insights-into-congenital-ichthyosis-patient-care-and-the-development-of-tmb-001

  We’ve had some children who’ve said this is the first time in their life that they can put on sneakers and run outside. […] I never have even thought about sweating as something that’s important, said Alan Mendelsohn, MD, while discussing the impact of congenital ichthyosis on patients. […] But remember, people with ichthyosis, lack the, in many cases lack the ability to sweat. They’re at a higher risk of heatstroke. […] It’s one of the most important projects I personally have ever been involved with in terms of the impact it has on the patient. […] I think we will move forward into lots more investment in thinking about how we can better affect patients with rare dermatological diseases. […] There’s never a good time to have a rare disease, but I think now is the time for people with rare diseases to have some more hope.

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