What are the treatment options for Ichthyosis?

From the Guidelines

Treatment for ichthyosis primarily involves managing symptoms through regular moisturizing and exfoliation, with a focus on improving skin hydration, promoting normal cell turnover, and reducing hyperkeratosis, as recommended by the European guidelines of care for congenital ichthyoses 1.

Key Components of Treatment

• Daily use of emollients like petroleum jelly, Aquaphor, or CeraVe, ideally applied immediately after bathing while skin is still damp, to help lock in moisture and reduce scaling.
• Keratolytic agents containing urea (10-40%), salicylic acid (2-6%), or lactic acid (5-12%) to help remove excess scales and promote skin hydration.
• For more severe cases, prescription retinoids such as acitretin (10-50mg daily) or isotretinoin (0.5-1mg/kg/day) may be prescribed, though these require monitoring for side effects and are contraindicated during pregnancy.
• Alpha-hydroxy acids like glycolic acid (5-15%) can also be effective for scale removal and promoting skin hydration.

Bathing and Skin Care

• Patients should take lukewarm baths (not hot) for 10-15 minutes daily, gently pat skin dry, and immediately apply moisturizers to help maintain skin hydration.
• Humidifiers can help maintain skin hydration, especially in dry environments.
• Cleaning of the skin is of utmost importance to remove scaling and residual ointments, to lessen discomfort and for hygiene, with mild soaps or soap-free cleansing bases recommended 1.

Monitoring and Follow-up

• Patients with ichthyosis require regular monitoring and follow-up to adjust treatment as needed and to manage any potential side effects.
• Patients with syndromic ichthyosis may require closer monitoring for side effects when using oral retinoids, even in cases of liver involvement or eye symptoms 1.

Quality of Life

• Ichthyosis can have a significant impact on quality of life, and treatment should focus on improving skin appearance and comfort, as well as managing any related complications or comorbidities 1.

From the FDA Drug Label

For Dermatologic Use: Salicylic Acid 6% is a topical aid in the removal of excessive keratin in hyperkeratotic skin disorders including verrucae, and the various ichthyoses (vulgaris, sex-linked and lamellar), keratosis palmaris and plantaris keratosis pilaris, pityriasis rubra pilaris, and psoriasis (including body, scalp, palms and soles). Indications and Usage For debridement and promotion of normal healing of hyperkeratotic surface lesions, particularly where healing is retarded by local infection, necrotic tissue, fibrinous or purulent debris or eschar. Urea is useful for the treatment of hyperkeratotic conditions such as dry, rough skin, dermatitis, psoriasis, xerosis, ichthyosis, eczema, keratosis pilaris, keratosis palmaris, keratoderma, corns and calluses, as well as damaged, ingrown and devitalized nails.

Treatment options for ichthyosis include:

• Salicylic acid (TOP): a topical aid in the removal of excessive keratin in hyperkeratotic skin disorders, including the various ichthyoses 2
• Urea (TOP): useful for the treatment of hyperkeratotic conditions, including ichthyosis 3

From the Research

Treatment Options for Ichthyosis

• Topical moisturizers and medications are the primary treatments for ichthyosis, aiming to reduce scaling and improve the condition 4
• Moisturizing creams containing urea, lactic acid, and other humectants and keratolytics are commonly used, along with regular bathing and mechanical scale removal 5
• Oral therapy with retinoids or retinoic acid metabolism-blocking agents may be beneficial for patients with lamellar ichthyosis or ichthyosiform erythroderma 5
• Chemical peels, such as 70% glycolic acid, can be an effective adjunctive treatment for reducing hyperkeratinization, especially when used in combination with other topical therapies 6
• Topical alpha/beta hydroxy acids, urea compounds, and emollients are also recommended for daily use to manage ichthyosis 6

Management and Therapy

• A multidisciplinary approach to treatment is essential, involving ichthyosis patient organizations and individualized therapeutic regimens 5
• Genetic testing is crucial for diagnostic certainty and understanding the molecular substrate for each patient, allowing for tailored treatment plans 7
• Replacement therapies aiming to substitute altered proteins and lipids, as well as gene therapy approaches, are being developed and assessed as potential treatments for ichthyosis 7
• Patient education and compliance are vital, as complex and costly treatment regimens may lead to decreased adherence 4