Materiały źródłowe

• #1 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.

• #2 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. […] This knowledge, combined with constant development of pathogenesis-based therapies, such as protein replacement therapy and gene therapy, are rather promising for patients with inherited skin diseases. […] Current treatment for ichthyosis is focused on symptom relief and includes emollients, keratolytics, and oral retinoids. […] 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).

• #3 Management of Ichthyosis: A Brief Review

  https://www.skintherapyletter.com/ichthyoses/management-ichthyosis-review/

  Ichthyosis management depends on disease severity and includes topical agents and lifestyle modifications with or without oral retinoids. […] Management should incorporate hydration and lubrication with the addition of keratolytics and modulators of keratinocyte differentiation depending on scale severity. […] Although generally not necessary in the management of the common ichthyoses (ichthyosis vulgaris and X-linked recessive ichthyosis), oral retinoids are a mainstay in the systemic management of severe disease. […] The majority of ichthyosis therapies aim to improve the barrier function of the skin. […] Ichthyosis management requires a multimodal approach, including topical and oral agents in addition to lifestyle modifications.

• #4 Congenital Ichthyosis: An Overview of Current and Emerging Therapies | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-0415

  Three important mechanisms are involved in the action of topical agents used in the treatment of ichthyosis: hydration, lubrication and keratolysis. The latter effect can also be achieved with systemic retinoids. For ichthyosis with an increased tendency towards skin infections, antimicrobials are another group of widely used agents. […] Therapy of ichthyosis remains mainly symptomatic and is empirically based on the use of topical emollients, various keratolytic agents and, in more severe cases, oral retinoids (vitamin A analogues). It is the purpose of this review to discuss the current therapy of ichthyosis and to highlight the need for new and more specific treatments. […] A primary objective in ichthyosis therapy is to remove scales and to reduce uncomfortable dryness of the skin (xerosis) without causing too much irritation. To accomplish this, the following aspects have to be taken into consideration before prescribing a treatment: (i) the age and sex of the patient, (ii) the type and severity of the disease, (iii) the extent and location of the skin lesions.

• #5 Ichthyosis vulgaris: Diagnosis and treatment

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

  There is no cure for inherited ichthyosis vulgaris. Treatment focuses on reducing the scale and dry skin. To do this, a treatment plan may require you to: […] Soaking helps hydrate your skin and soften the scale. If you have open sores, your dermatologist may recommend placing petroleum jelly or another product on these before getting into the water. This can reduce the burning and stinging caused by the water. Some patients say that adding sea salt (or table salt) to the water reduces the burning and stinging. Adding salt may also reduce the itch. […] Your dermatologist may recommend that you reduce the scale while its softer by gently rubbing the scale with an abrasive sponge, buff puff, or pumice stone. […] Moisturizer can seal water from a bath or shower into your skin. Your dermatologist may recommend a moisturizer that contains an active ingredient like urea, alpha hydroxyl acid, or lactic acid. These and other active ingredients can also help reduce scale.

• #6 Ichthyosis vulgaris

  https://dermnetnz.org/topics/ichthyosis-vulgaris

  Treatment aims to reduce dryness, scaling, splitting and thickening of the skin. This is achieved with exfoliation and moisturising on a regular, daily basis. […] Apply emollients with high lipid content, such as lanolin cream (a sebum-like substance derived from wool-bearing animals). […] Bathe in salt water. […] Apply creams or lotions containing salicylic acid, glycolic acid, lactic acid or urea to exfoliate and moisturise skin. These may irritate active eczema. […] Oral retinoids such as acitretin or isotretinoin can be prescribed in severe cases. […] Apply lotions and creams to damp skin to trap in the moisture (within 3 minutes of showering/bathing). […] Lotion and creams can be kept under occlusion for 1 or 2 hours with a cling-film wrap to enhance skin hydration. […] Gently rub a pumice stone on wet skin to help remove thickened crusty skin. […] Brush washed hair to remove scales from scalp.

• #7 Ichthyosis, therapy – Altmeyers Encyclopedia – Department Dermatology

  https://www.altmeyers.org/en/dermatology/ichthyosis-therapy-156446

  Experience in the external and internal treatment of ichthyoses is generally based on the knowledge and experience of experts. A wide range of treatment options is available for the external therapy of ichthyosis. […] Regular, even multiple, daily bathing is also recommended in daily care. This cleanses the skin, loosens scales and removes ointment residues. At the same time, the skin is hydrated. […] Cooking or sea salt baths promote hydration and keratolysis. […] To support desquamation, the following bath additives have proved helpful: sodium hydrogen carbonate, wheat starch, rice starch and corn starch. […] After a softening bath, horny deposits can also be removed mechanically. […] The balneotherapeutic measures should be followed by a refatting local therapy to maintain the hydration achieved by the bath.

• #8 Ichthyosis vulgaris: Diagnosis and treatment

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

  This can help get rid of the deep cracks. […] If you develop a skin infection, your dermatologist will treat it with medication that you either take or apply to your skin. If you get frequent skin infections, your dermatologist may recommend adding a small amount of bleach to your bath. Called bleach bath therapy, this can help reduce the bacteria on your skin. You should only use bleach bath therapy if your dermatologist recommends it. […] If the ichthyosis is severe and fails to improve with baths, moisturizer, and scale remover, your dermatologist can prescribe medicine. […] Soaking, moisturizing, and removing the scale can also get rid of the rough bumps on the skin, known as keratosis pilaris. […] If the ichthyosis is mild, you may need to treat it only during the winter. […] To improve acquired ichthyosis vulgaris, you must also treat the disease that triggered the ichthyosis. If the disease can be cured, the ichthyosis may go away. If a medicine triggered the ichthyosis, reducing the dose often gets rid of the ichthyosis.

• #9 Ichthyosis keeps surprising us | Actas Dermo-Sifiliográficas

  http://www.actasdermo.org/en-ichthyosis-keeps-surprising-us-articulo-S1578219013000899

  In clinical practice, the pediatric dermatologist from time to time encounters patients who have ichthyosis. Patients with this rare disease need a precise diagnosis, good advice on how to manage their skin condition, and adequate genetic counseling. […] Treatments such as topical urea ointments often have very little effect on this symptom. In contrast, oral retinoids, such as acitretin, can be a very effective remedy resulting in normalization of sweat gland function, as demonstrated by gravimetric measurement of sweat rates before and after treatment. […] Today we know that ichthyosis patients should take a cleansing bath daily and rub their skin with special gloves to mechanically remove some of their scales. In many, although not all patients, this mechanical scale removal is greatly facilitated by adding two handfuls of sodium bicarbonate (baking powder) to the bath water.

• #10 Ichthyosis – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/cornification-disorders/ichthyosis

  Treatment involves emollients and sometimes oral retinoids. […] Other treatments of ichthyosis include emollients, keratolytics, and retinoids. […] Emollients are the mainstay of therapy for all patients with ichthyosis. […] Topical keratolytics are used to thin excess scale and to promote peeling of the stratum corneum. […] Retinoids are also effective in treating inherited ichthyoses. […] Research is ongoing in the development of molecular treatments for ichthyoses and related diseases, including protein and enzyme replacement, repurposed biologic agents, small-molecule agents, and gene replacement and editing. […] Emollients that speed the shedding of skin (keratolytics) are effective in treating ichthyosis.

• #11 Ichthyosis: Types, Symptoms, & Treatment

  https://www.webmd.com/skin-problems-and-treatments/what-is-ichthyosis

  Ichthyosis can’t be cured, but treatments can relieve the scaling and make you feel more comfortable. […] It can’t be cured, but treatments can help you manage dry and scaly skin. […] Rub cream, lotion, or ointment onto your skin every day to add moisture. Look for rich creams that have any of these ingredients: lanolin, alpha hydroxy acids, urea, or propylene glycol. Products with ceramides or cholesterol also keep skin moist. […] Apply the lotion right after you step out of the shower or bath, while your skin is still damp. This will help hold in moisture. […] If the dryness and scaling are severe, your doctor might prescribe an oral retinoid medicine such as acitretin (Soriatane) or isotretinoin (Absorica, Claravis, Sotret, and others). Retinoids can cause side effects such as weakened bones, dry mouth, and upset stomach. You might need antibiotics for skin infections. If your skin condition is caused by another condition or medicine you’re taking, treating the disease or changing the dose may help. […] Your dermatologist can recommend treatments to help your skin look and feel better.

• #12 Ichthyosis Vulgaris Treatment | Water’s Edge Dermatology | FL Dermatologists

  https://www.wederm.com/patient-library/ichthyosis-vulgaris/

  Ichthyosis is a group of skin diseases that causes extremely dry, thick, and scaly skin. […] Treatment for Ichthyosis […] Since there is no cure for inherited ichthyosis vulgaris, treatment focuses on managing the disease and lessening the symptoms. Your Water’s Edge Dermatology practitioner will recommend ways to reduce the scale and dry skin. […] Soaking in a warm bath can help hydrate your skin and soften the scale. You can then slough off the scale by gently rubbing it with an abrasive sponge, buff puff, or pumice stone. […] You should apply moisturizer to damp skin within 2 minutes of bathing to seal in water from a bath or shower. Moisturizers that contain an active ingredient like urea, alpha hydroxyl acid, or lactic acid can also help reduce scale. You should also apply petroleum jelly to deep cracks in thickened skin.

• #13 Therapies in Practice: New Options for Ichthyosis Vulgaris

  https://practicaldermatology.com/topics/general-topics/therapies-in-practice-new-options-for-ichthyosis-vulgaris/21933/

  Ichthyosis vulgaris is the most common type of ichthyosis accounting for about 95 percent of all cases of hereditary ichthyosis. […] For IV, moisturizing creams are the cornerstone of management, which is largely symptomatic. Traditionally, clinicians have emphasized moisturizing ingredients that also confer keratolytic effects, such as urea or lactic acid. […] Given the emerging evidence implicating fillagrin deficiency in the pathogenesis of ichthyosis, treatment aimed at restoration of the barrier function has been advocated in ichthyosis and related barrier defect diseases. […] Several barrier repair creams are currently available on the market. The newest, EpiCeram (Promius) is formulated with a 3:1:1 ratio of ceramides, cholesterol, and free fatty acids intended to optimize the repair of barrier of function. […] Both patients had notable improvement in symptoms of ichthyosis at two weeks; continued use of barrier repair cream led to continued improvement. Patients were counseled to continue use for maintenance of benefit.

• #14 Overcoming the Barrier Treatment of Ichthyosis: A Combination-therapy Approach | JCAD – The Journal of Clinical and Aesthetic Dermatology

  https://jcadonline.com/overcoming-the-barrier-treatment-of-ichthyosis-a-combination-therapy-approach/

  Ichthyosis vulgaris is an inherited disorder of keratinization that results in asteatotic scales on extensor surfaces of the arm, legs, and trunk. A combination-therapy approach with a physiological lipid-based barrier repair topical emulsion and ammonium lactate 12% lotion applied topically was shown to be effective at four-week follow up without any untoward side effects. This combination therapy addresses the importance of caring for both the corneocytes (bricks) and the intercellular lipid bilayer (mortar) for optimal benefit. […] The patient was treated with a combination regimen including ammonium lactate (AL) 12% lotion, followed by a physiological lipid-based barrier repair cream containing ceramides, cholesterol, and free fatty acids in a 3:1:1 ratio designed to simulate the normal intercellular lipid bilayer. […] The combination therapy of Ec and AL 12% lotion addresses both elements in order to optimize treatment for the IV patient.

• #15 Ichthyosis vulgaris: Pictures, diagnosis, and treatment

  https://www.medicalnewstoday.com/articles/318242

  Currently, there is no known cure for the condition, but the consistent and regular use of moisturizers and exfoliants are often enough to resolve symptoms. […] Unfortunately, there is no known cure for ichthyosis vulgaris. Treatment options revolve around lessening symptoms by removing scaling and reducing skin dryness. […] Regular, consistent exfoliation aims to slowly dull scales. Once scaling subsides, the skin is better able to take in and retain moisture. […] Commonly recommended ways to reduce scaling include: soaking the affected area in salt water or bathe in salt water; soaking the affected area in lukewarm water and then gently rubbing in a circular motion using a pumice stone; using moisturizers that contain exfoliating chemicals, such as glycolic acid, alpha hydroxy acid, lactic acid, salicylic acid, or urea; carefully brushing washed hair to remove scaling on the scalp; for severe cases, using oral vitamin A-based medications like acitretin or isotretinoin to slow down skin cell production.

• #16 X-Linked Ichthyosis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/23337

  Older children and adults may incorporate topical keratolytics into their treatment regimen. However, their use is challenging in young children due to the potential for irritation and discomfort. Caution should be exercised when applying topical keratolytics over large body surfaces in children, considering the risk of systemic absorption and associated toxicities. Commonly available keratolytics typically contain 5% to 12% lactic acid, 2% to 10% urea, 5% to 15% glycolic acid, 10% to 25% propylene glycol, and 3% to 6% salicylic acid. […] In severe forms of X-linked ichthyosis, patients may benefit from the intermittent use of topical or even systemic retinoids. Limited evidence exists regarding the efficacy of keratolytics, topical, and systemic retinoids for treating mild-to-severe X-linked ichthyosis. Tazarotene, 0.05% gel, has been shown to cause marked clinical improvement in X-linked ichthyosis, whereas oral acitretin has significantly improved scaling and erythema.

• #17 Ichthyosis vulgaris: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001451.htm

  Ichthyosis vulgaris is a skin disorder passed down through families that leads to dry, scaly skin. […] 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.

• #18 Ichthyosis, therapy – Altmeyers Encyclopedia – Department Dermatology

  https://www.altmeyers.org/en/dermatology/ichthyosis-therapy-156446

  Urea is generally used successfully in a 10% application form. Urea reduces epidermal proliferation, has barrier-regenerating, antimicrobial and keratolytic effects, smoothes the skin and promotes the penetration of other active ingredients in a base-dependent manner. […] Local retinoids have a very good antikeratotic effect, but are usually only suitable for localized cornification disorders or stubborn areas such as hands, feet, lower legs and over joints due to the irritative potential. […] Acitretin is the only approved agent for systemic treatment of ichthyosis. It inhibits keratinization and regulates cellular differentiation.

• #19 What Is Acquired Ichthyosis?

  https://www.verywellhealth.com/acquired-ichthyosis-vulgaris-2860934

  Acquired ichthyosis has also been associated with the use of certain medications, such as niacinamide, Tagamet (cimetidine) and Lamprene (clofazimine). […] The severity of acquired ichthyosis usually depends on the underlying condition present. As the systemic condition is treated, the ichthyosis generally improves. The skin affected by ichthyosis is treated by hydration with alpha-hydroxy acid lotions, such as Lac-Hydrin (ammonium lactate). Topical retinoid cream like Retin-A (tretinoin), may also be used. Scales and skin buildup can be reduced with salicylic acid. […] Whichever treatment you use, it’s important to keep the skin hydrated with a lotion that won’t evaporate. If you have acquired ichthyosis, make sure to consistently follow-up with your dermatologist and any other specialists monitoring any underlying conditions.

• #20 Lamellar Ichthyosis Treatment & Management: Approach Considerations, Consultations, Prevention

  https://emedicine.medscape.com/article/1111300-treatment

  Topical N-acetylcysteine for the treatment of lamellar ichthyosis: an improved formula. […] Successful treatment with topical N-acetylcysteine in urea in five children with congenital lamellar ichthyosis. […] Topical application of tazarotene in the treatment of nonerythrodermic lamellar ichthyosis. […] Oral liarozole in the treatment of patients with moderate/severe lamellar ichthyosis: results of a randomized, double-blind, multinational, placebo-controlled phase II/III trial.

• #21 Congenital ichthyosis: a practical clinical guide | CCID

  https://www.dovepress.com/congenital-ichthyosis-a-practical-clinical-guide-on-current-treatments-peer-reviewed-fulltext-article-CCID

  Both the erosions and hyperkeratosis of epidermolytic ichthyoses are often exacerbated by friction, making reasonable minimization of friction helpful. […] As understanding of the genetic causes and pathophysiology of congenital ichthyoses has expanded, pathogenesis-based therapies have been attempted with mixed success. […] The lure of these pathogenesis-based treatments is great, but by definition, each subtype of ichthyosis requires a different treatment. […] Generalized hyperkeratosis, scaling, and dryness experienced by congenital ichthyosis patients are commonly treated using topical and/or oral retinoids; however, this use is currently off-label. […] TMB-001 is a new topical isotretinoin ointment that uses a proprietary polyethylene glycol delivery technology to treat congenital ichthyosis by providing hydration, lubrication and scale reduction.

• #22 Congenital ichthyosis: a practical clinical guide | CCID

  https://www.dovepress.com/congenital-ichthyosis-a-practical-clinical-guide-on-current-treatments-peer-reviewed-fulltext-article-CCID

  A Phase 3 trial with TMB-001 0.05% ointment is ongoing, and this medication represents a promising new therapy that could provide congenital ichthyosis patients an effective on-label topical retinoid option. […] Treatments span the spectrum of traditional dermatology (eg, baking soda and bleach baths, emollients, etc.) to cutting edge (eg, pathophysiologic-based gene therapy).

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

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

  Ichthyosis vulgaris is a common condition that causes dry, scaly skin. […] There isnt a cure, but you can minimize symptoms and prevent infections by soaking, exfoliating and moisturizing your skin frequently. […] 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. […] For severe cases that dont improve with skin care strategies, your healthcare provider may prescribe oral retinoids such as acitretin or isotretinoin. […] Although theres no cure, there are treatments available to help you manage the condition. If you think you have ichthyosis vulgaris, talk to a healthcare provider like a dermatologist. They can diagnose the issue and prescribe medication to help minimize severe symptoms and prevent infections.

• #24 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. Similarly, liarozole (150 mg bid), an imidazole derivative, inhibits the cytochrome P450-dependent 4-hydroxylation of retinoic acid, resulting in increased tissue levels of retinoic acid and a reduction in epidermal proliferation and scaling. […] 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. Corneal infections may result in a scar that reduces the clarity of the eye or, at worst, perforation of the cornea.

• #25

  https://www.ichthyosis.org.uk/faqs/retinoids-for-ichthyosis

  Retinoids are drugs derived from Vitamin A which are used to treat a number of skin conditions. The most common are acetretin (Neotigason) for the treatment of ichthyosis and isotretinoin (Roaccutane) for the treatment of severe forms of acne. […] For ichthyosis the acetretin will help the skin cells grow and differentiate more normally. This process of skin maturation is known as keratinization. […] Individuals with the most severe forms of ichthyosis. It can only be prescribed and supervised by a hospital based specialist (usually a Consultant Dermatologist). […] The manufacturers recommend that continuous treatment should not last longer than 6 months; however, in clinical practice and reported studies there are now a significant number of patients who have been on retinoids for over 5 years, some for greater than 10 years. If there is a good response to treatment, acetretin can be continued safely long-term with appropriate monitoring.

• #26 Old drugs shed new light on a rare skin disease | Drug Discovery News

  https://www.drugdiscoverynews.com/old-drugs-shed-new-light-on-a-rare-skin-disease-16138

  Every day Simmons spends as many as four hours scrubbing his skin to get rid of the dry, scaly layer accumulating on the surface. […] What Simmons wants more than anything is a cure; something that strikes right at the cause of ichthyosis. But research to develop ichthyosis treatment has been limited and slow. […] There are no approved treatments for ichthyosis, so when Joyce Teng, a dermatologist at Stanford University, sees patients, she tries to identify ways to at least alleviate the discomfort that comes with the disease. This includes moisturizing to reduce dryness and exfoliating to remove the scales. […] One of the biggest breakthroughs occurred in the 1980s, when doctors realized that retinoids could decrease scaling. […] Simmons takes acitretin, an oral retinoid, which has helped him manage his skin but is by no means a cure.

• #27 Congenital Ichthyosis: An Overview of Current and Emerging Therapies | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-0415

  The therapeutic armature for treating ichthyoses extends from simple balneo-therapy and mechanical removal of scales to highly effective topical formulations and systemic therapy requiring strict medical attention. For most types of ichthyoses, a first-line therapy includes hydration and lubrication, in order to improve the barrier function and facilitate desquamation. […] By combining two or more keratolytic agents and moisturizers in the same lipophilic cream base it is often possible to achieve additive or even synergistic effects in LI without the need to use irritating concentrations of either agent alone. […] In all types of ichthyosis retinoid therapy induces a decrease in skin thickness and scaling, which begins about 12 weeks after the initiation of therapy. Thickening recurs after the retinoid is discontinued. […] An alternative to synthetic retinoid treatment is to manipulate the endogenous level of all-trans retinoic acid (tretinoin) by blocking its cellular catabolism in the skin with retinoic acid metabolism blocking agents (RAMBAs).

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

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

  Theres currently no cure for ichthyosis vulgaris. However, treatment can help you manage your symptoms. […] Regularly apply moisturizers that have urea or propylene glycol in them. These chemicals will help your skin stay moist. Using products with urea, lactic, or salicylic acid can also help your skin shed dead cells. […] Your doctor may also prescribe specialized creams or ointments to help moisturize the skin, get rid of dead skin, and control inflammation and itching. These may include topical treatments containing the following ingredients: […] Retinoids may be used in difficult cases to slow your bodys production of skin cells. These substances are derived from vitamin A, so they may have some adverse side effects. Side effects may include lip swelling or hair loss. Birth defects may occur if taken during pregnancy.

• #29

  https://mohsdermhouston.com/ichthyosis-vulgaris-dry-skin-treatment/

  Ichthyosis vulgaris is usually diagnosed by dermatologists by looking at the patients skin. […] Inherited ichthyosis vulgaris doesnt have any cure. The treatment basically focuses on reducing dry and scaly skin. Your dermatologist may ask you to do the following: Take regular and frequent baths as directed. Soaking your skin will help in softening scales and hydrating the skin. […] You should apply moisturizer while your skin is still damp from bathing. Dont wait for more than two minutes to get this done. Moisturizer helps in sealing water from the shower or bath. You may be recommended moisturizers containing lactic acid, urea and alpha hydroxyl acid. These ingredients are helpful in reducing scales. […] Your dermatologist will prescribe medications for treating skin infections. These medications may need to be taken orally or applied topically. […] Your dermatologist may ask you to add a tiny amount of bleach to your bath if you are prone to skin infections. This is called bleach bath therapy and can help in reducing bacteria.

• #30 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. […] To prevent cicatricial ectropion in lamellar ichthyosis, a humidified atmosphere combined with the use of topical moisturizing agents is beneficial. Petrolatum ointment and 10% urea cream applied to the eyelid skin several times daily helps to prevent skin contracture. […] Pimecrolimus 1% (Elidel) an immunomodulating agent used in the treatment of atopic dermatitis, has been shown to be effective in patients with Netherton 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. Full-thickness skin grafts from the forearm, postauricular, and groin areas may be used to successfully repair the abnormalities.

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

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

  For a persistent corneal epithelial defect, an amniotic membrane transplantation may be necessary to promote epithelial wound healing. […] Because ichthyosis is primarily a skin disorder, periodic evaluation by a dermatologist is recommended. […] The mainstay of ichthyosis therapy includes removal of surface scales, and application of a water barrier.

• #32 New developments in the molecular treatment of ichthyosis: review of the literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9287901/

  Ichthyosis covers a wide spectrum of diseases affecting the cornification of the skin. In recent years, new advances in understanding the pathophysiology of ichthyosis have been made. This knowledge, combined with constant development of pathogenesis-based therapies, such as protein replacement therapy and gene therapy, are rather promising for patients with inherited skin diseases. Several ongoing trials are investigating the potency of these new approaches and various studies have already been published. Furthermore, a lot of case series report that biological therapeutics are effective treatment options, mainly for Netherton syndrome and autosomal recessive congenital ichthyosis. It is expected that some of these new therapies will prove their efficacy and will be incorporated in the treatment of ichthyosis.

• #33 New developments in the molecular treatment of ichthyosis: review of the literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9287901/

  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).

• #34 New developments in the molecular treatment of ichthyosis: review of the literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9287901/

  Secukinumab is a recombinant human monoclonal antibody that targets the IL-17A cytokine. […] Dupilumab is used in the treatment of AD. It blocks the interleukin-4 receptor and therefore inhibits IL-4 and interleukin-13 (IL-13). […] Gene therapy is aiming to restore the wild type gene function. […] Enzyme replacement therapy (ERT) entails the replacement of a deficient structural protein or enzyme. […] Lipid replacement could be effective for CHILD syndrome, which is caused by a deficiency in bulk cholesterol. […] In conclusion, enzyme and lipid replacement are very interesting strategies to improve ichthyosis symptoms, by directly substituting the deficient substance. More studies should be encouraged to look at the clinical effect and consider the possible toxicity arising from accumulation of metabolic intermediates.

• #35 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

  The rationale of repurposing biologics for ichthyosis is based on studies of Paller et al. […] Secukinumab is a recombinant human monoclonal antibody that targets the IL-17A cytokine. […] Dupilumab is used in the treatment of AD. […] A limited number of case reports describe usage of biological therapy in the other subtypes of ichthyosis. […] In conclusion, the use of biological therapies could be beneficial in the treatment of several ichthyosis subtypes. […] Enzyme replacement therapy (ERT) entails the replacement of a deficient structural protein or enzyme. […] For ichthyosis, it could be applicable for recessive forms, where a deficiency of a specific protein leads to the phenotype. […] Gene therapy is aiming to restore the wild type gene function. […] Gene therapy has not yet been explored as widely as biologicals have.

• #36 Successful treatment of an adult with lamellar ichthyosis | BTT

  https://www.dovepress.com/successful-treatment-of-an-adult-with-atopic-dermatitis-and-lamellar-i-peer-reviewed-fulltext-article-BTT

  In our patient, although acitretin provided a good therapeutic effect for LI, it was discontinued due to worsening atopic dermatitis. Dupilumab was prescribed for three months to treat the worsening atopic dermatitis and markedly improved the LI, atopic dermatitis, and asthma. […] Dupilumab is useful for the treatment of atopic dermatitis mainly due to its role in helper (Th)2-mediated immunity. […] Three-month treatment with dupilumab improved a case of lamellar ichthyosis associated with atopic dermatitis and asthma.

• #37 STELARA – Treatment of Ichthyosis

  https://www.jnjmedicalconnect.com/products/stelara/medical-content/stelara-treatment-of-ichthyosis

  Case reports describing the use of STELARA in patients with ichthyosis are detailed below.1-6 […] The patient was initiated on STELARA 1.2 mg/kg every 8 weeks. […] With the addition of STELARA therapy, the patient achieved symptom control. […] At 22 months, STELARA 0.75 mg/kg was initiated at weeks 0, 4, and every 12 weeks thereafter. […] STELARA was initiated at 0.75 mg/kg at weeks 0, 4, and every 12 weeks with dramatic skin improvement. […] STELARA therapy was initiated, resulting in itch improvement after 4 weeks. […] At 12 weeks, there was a 59% reduction in composite IASI (from 8.2 to 3.3), 40% reduction in IASI-Scaling (from 3.5 to 2.1), and 74% reduction in IASI-Erythema (from 4.7 to 1.2).

• #38 New developments in the molecular treatment of ichthyosis: review of the literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9287901/

  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. […] Several therapies, such as the use of siRNA and CRISPR/Cas9, are already in the pre-clinical testing for EB, where they show promising results. These therapies have not yet been explored for ichthyosis, but could lead to new perspectives in the search for a cure.

• #39 Ichthyosis: A Road Model for Skin Research | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3433

  Substitution and replacement therapy are other interesting approaches. […] Since various types of ceramides can now be synthetized in large amounts, they are obvious candidates for testing topically in ARCI. […] In this context, enzyme replacement therapy (ERT) with topically applied recombinant transglutaminase may become an attractive (but expensive) future option, especially for patients with TGM1-associated ARCI. […] Perhaps a combination of ERT and supplementation with synthetic ceramides would prove most versatile, although this approach remains to be studied. […] In the long term, the search for new therapies in ichthyosis should also focus on alternative ways to restore the skin barrier and to dampen excessive intrinsic responses, which often cause more harm than relief to the patient. […] Whether this goal is attainable through gene technology and new biologics, or by specifically tailored molecules and substitution therapies, remains to be determined.

• #40 Ichthyosis: A Road Model for Skin Research | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3433

  Careful medical attention is frequently required, including oral retinoid therapy. […] The vast majority of patients with ichthyosis have only mild to moderate skin symptoms, which are readily controlled by daily applications of cream. […] Encouragingly, many new ideas for ichthyosis treatment are in the pipeline, targeting not only the causative mechanisms, but also secondary events, such as inflammation and hyperproliferation. […] Although gene therapy for skin diseases has not yet proved as successful as initially hoped, topical antisense therapy blocking the translation of mutated mRNA has shown promising results, at least in pachyonychia congenita, a keratinopathic disorder mechanistically similar to epidermolytic ichthyosis. […] Moreover, disruption of mutated KRT10 in EI keratinocytes using a transcription activator-like effector nuclease (TALEN) technology reverts the intermediate filament fragility in vitro. […] These and other approaches, such as CRISPR/Cas9 gene editing, aimed at correcting the underlying mutation in situ, holds promise for a more specific gene therapy for ichthyosis in the future.

• #41 Hydrotherapy for Ichthyosis | Avene Center

  https://www.centrethermalavene.com/en/our-treatments/our-intensive-dermatology-hydrotherapies/hydrotherapy-ichthyosis

  Our hydrotherapy center welcomes growing numbers of patients suffering from ichthyosis. […] In 2009, a pioneer study was performed, in collaboration with several university hospital centers, to evaluate the effects of Avne hydrotherapy treatment on a population of ichthyosis patients. […] This study underlines the therapeutic benefits of Avne hydrotherapy: Significant effectiveness in improving clinical signs and patients’ quality of life was demonstrated at the end of the hydrotherapy treatment and even 6 months afterwards. […] The short and medium term effectiveness of Avne hydrotherapy treatments was demonstrated in patients suffering from hereditary ichthyosis. […] A treatment adapted to your condition. Our hydrotherapy doctors evaluate and choose the care that is best for you. […] A treatment adapted to your condition. A medical opinion at the start of treatment and tailor-made treatments. […] Our medical-thermal team bring you the best of Avne by offering SensiCure, a hydrotherapy method that is open to all with unlimited access, following a medical consultation.

• #42 Ichthyosis Vulgaris (Ichthyosis Congenita) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/ichthyosis-vulgaris-ichthyosis-congenita/

  Treatment options for IV are sumarized in Table I. […] Topical white petrolatum or emollients containing propylene glycol, ceramide, or urea are the cornerstones of treating IV. White petrolatum acts as an occlusive emollient that traps in moisture. Propylene glycol draws water into the stratum corneum by creating a gradient, ceramide replaces the natural moisturizing factor that is deficient as a result of the loss of filaggrin, and urea helps works as a humectant. These are all excellent first-line therapies as they are effective and safe. […] Topical keratolytics such as salicylic acid and alpha-hydroxy acids (eg, lactic acid) are also very effective therapies that work by removing scale from the stratum corneum. […] For severe and recalcitrant disease, all of the above emollients can be used in conjunction with a modified soak and smear technique whereby patients soak in plain lukewarm water for 20 minutes and then apply the emollient to wet skin.

• #43 Ichthyosis Vulgaris (Ichthyosis Congenita) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/ichthyosis-vulgaris-ichthyosis-congenita/

  Improvement is gradual and can be expected over the course of 2 weeks of aggressive treatment with the above modalities. […] Given that the genetic and molecular mechanisms of IV are becoming clearer, we are likely to see newer therapies to specifically target pathways that are perturbed by loss of filaggrin expression and its active components that lead to disease.

• #44 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.

• #45 Lamellar Ichthyosis Treatment & Management: Approach Considerations, Consultations, Prevention

  https://emedicine.medscape.com/article/1111300-treatment

  Skin moisturizers are the main treatments for lamellar ichthyosis. Moisturizers serve as a barrier, and they also help in the removal of scales. Microfiber washcloths and urea also are used to remove the scales. For neonates, electrolytes should be monitored closely. The hyperkeratosis can interfere with sweat gland function, leading to disordered thermoregulation. Some patients may have severe heat intolerance. Oral retinoids are used for the purposes of speeding up epidermal turnover, and they can improve or prevent some of the consequences of the disease. Oral retinoids may cause an increase in sweating, leading to improved heat tolerance. Oral retinoids may also decrease the thick periocular scale and decrease the propensity to develop ectropion. Artificial tears and eye lubricants are effective in patients with ectropion; surgery is suggested in those with severe disease.

• #46 Lamellar Ichthyosis Treatment & Management: Approach Considerations, Consultations, Prevention

  https://emedicine.medscape.com/article/1111300-treatment

  Topical N-acetylcysteine for the treatment of lamellar ichthyosis: an improved formula. […] Successful treatment with topical N-acetylcysteine in urea in five children with congenital lamellar ichthyosis. […] Topical application of tazarotene in the treatment of nonerythrodermic lamellar ichthyosis. […] Oral liarozole in the treatment of patients with moderate/severe lamellar ichthyosis: results of a randomized, double-blind, multinational, placebo-controlled phase II/III trial.

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

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

  Improved treatment options have given babies a better chance at survival than in the past. […] But now, with improved treatment options and intensive medical care, babies have a better chance of living into childhood and reaching adulthood. […] 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. […] 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. […] Despite advances in the treatment of harlequin ichthyosis, many infants still die from the condition. […] But early introduction of oral retinoids may help increase the survival rate. In the same study, 83% of babies that were treated with oral retinoids survived.

• #48 Harlequin Ichthyosis: Causes, Symptoms & Treatments

  https://www.webmd.com/children/what-is-harlequin-ichthyosis

  Harlequin ichthyosis is a very rare and serious genetic condition that causes you to grow too much skin. Babies with this condition need round-the-clock care in a NICU. Thanks to new medicines and better NICU care, more babies with harlequin ichthyosis survive the first few weeks after they are born, which is the most dangerous time. With careful attention to taking care of their skin and managing other symptoms, people with harlequin ichthyosis can live a long time and have a good quality of life. […] Treatment for harlequin ichthyosis focuses on managing the symptoms, keeping the skin as protected as possible, and preventing infection. It will not make harlequin ichthyosis go away, but it can help people who have it live longer and be more comfortable. […] In the NICU, doctors and nurses will watch your baby constantly and give them round-the-clock care. Your baby might also get support from physical and occupational therapists, skin doctors (dermatologists), nutritionists, or other professionals.

• #49

  https://dermnetnz.org/topics/ichthyosis

  Ichthyosis is a disorder of cornification, characterised by persistently dry, thickened, 'fish scale’ skin. […] Ichthyosis may be provoked by certain medications: […] The following may be useful: Non-soap cleansers (soap may exacerbate dryness), bathing in salt water, rubbing with a pumice stone or exfoliating sponge to remove scale, moisturising creams containing urea, salicylic acid or alpha hydroxy acids. These are best applied to damp skin. In severe disease, the oral retinoids acitretin and isotretinoin, oral antibiotics for secondary bacterial infection, vitamin D supplementation for patients with severe forms of ichthyosis. […] Studies are on-going to evaluate the effect of monoclonal antibodies targeting the Th17 immune pathways. […] The most severe forms of ichthyosis may threaten the life of an affected newborn, but most people with ichthyosis have a normal lifespan.

• #50 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). […] 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. Treatment with systemic retinoids, like Acitretin and Isotretinoin, may be beneficial in severe forms. Vitamin D supplementation may be necessary in some cases. […] Acquired ichthyosis usually resolves with treatment of the underlying disease or withdrawal of the causative drug.

• #51 Lamellar Ichthyosis Treatment & Management: Approach Considerations, Consultations, Prevention

  https://emedicine.medscape.com/article/1111300-treatment

  Surgery is occasionally necessary for severe ectropion, with skin grafts being the usual therapy. Inverting sutures may also stabilize the ectropion as the child grows, and it is surprisingly well tolerated. […] Consult a dermatologist for the evaluation and treatment of the skin. […] Prenatal diagnosis is controversial. A fetal skin biopsy at 22 weeks may aid in prenatal diagnosis. In patients with a known gene locus, DNA linkage analysis may be useful. […] The quality of life of patients with the more severe congenital ichthyoses is often seriously affected and the parents’ request for prenatal diagnosis is not easily ignored. Because of advances in the understanding of the causative genetic defects for severe congenital ichthyosis, making DNA-based prenatal diagnosis is now possible for several congenital ichthyosis, using chorionic villus or amniotic fluid sampling procedures early in pregnancy, with a lower risk to fetal health and with a reduced burden on the parents.

• #52 Ichthyosis – BAD Patient Hub

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

  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. […] Steroid treatments are not effective in ichthyosis. […] Some people affected by ichthyosis may find they do not sweat effectively. […] Vitamin D levels are often low in individuals with ichthyosis. […] Ichthyosis can have a significant psychological and emotional impact on those affected by it.

• #53

  https://step2.medbullets.com/evidence/19824737

  Ichthyoses constitute a large group of cornification disorders that affect the entire integument. The therapeutic management is multidisciplinary and involves ichthyosis patient organizations in many countries. The mainstay of treatment remains with moisturizing creams containing, for example, urea, lactic acid and other humectants and keratolytics, regular bathing, and mechanical scale removal. Patients with lamellar ichthyosis or ichthyosiform erythroderma in particular profit from oral therapy with retinoids or retinoic acid metabolism-blocking agents. […] However, the dramatic increase in knowledge about the pathophysiology of these conditions has not led to a curative therapy so far.

• #54

  https://link.springer.com/article/10.1007/s40257-022-00718-8

  The management of ichthyosis poses a challenge for most physicians. Treatment options proposed in the literature include moisturizers, topical keratolytics, topical and systemic vitamin D analogues, and topical and systemic retinoids; however, some of these modalities are less reliable than others. […] Despite the therapeutic impasse imposed by the options above, the emergence of pathogenesis-based treatments along with novel gene therapies appear promising and hold the potential to halt or even revert disorders that arise from single genetic mutations, although research is still quite lacking in this domain. […] Hence, this review aims to highlight the various treatment modalities available for the management of the cutaneous manifestations of non-syndromic inherited ichthyosis, with an added emphasis on pathogenesis-targeted therapies.

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