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Hereditary and Acquired Ichthyosis Vulgaris

Sections Hereditary and Acquired Ichthyosis Vulgaris
Overview
Presentation
DDx
Workup
Treatment
Medication
Media Gallery
References

Treatment

Medical Care

Hereditary ichthyosis vulgaris is a chronic disorder that may improve with age but often requires continuous therapy. The severity of acquired ichthyosis usually depends on the status of the underlying systemic condition. The main approach to treatment of both conditions includes hydration of the skin and application of an ointment to prevent evaporation. Hydration promotes desquamation by increasing hydrolytic enzyme activity and the susceptibility to mechanical forces. Pliability of the stratum corneum is also improved. Note the following:

Topical retinoids are helpful for some patients.

Alpha-hydroxy acids (eg, lactic, glycolic, or pyruvic acids) are effective for hydrating the skin. They work by causing disaggregation of corneocytes in the lower levels of the newly forming stratum corneum. Lactic acid is available as a 12% ammonium lactate lotion, or it can be compounded by prescription in a concentration of 5-10% in a suitable vehicle. Twice-daily applications have shown to be superior to petrolatum-based creams for controlling of ichthyosis vulgaris.

Removal of scales can be aided by keratolytics (eg, salicylic acid), which induce corneocyte disaggregation in the upper stratum corneum. A commercially available 6% salicylic acid gel can be used on limited areas.

Over-the-counter products often contain urea or propylene glycol. Moisturizers containing urea in lower strengths (10-20%) produce a more pliable stratum corneum by acting as a humectant. Propylene glycol draws water through the stratum corneum by establishing a water gradient. Thick skin is then shed following hydration. Propylene glycol is a common vehicle in both prescription and over-the-counter preparations. A new urea topical formulation applied to hyperkeratotic and dry skin in these patients compared favorably with a standard emollient cream.^[45]

A urea-based emulsion composed of 10% urea, ceramides, and natural moisturizing factors used twice daily may be beneficial.^[46]Urea helps maintain skin hydration and may have a role in assisting the regulation of epidermal genes used for proper barrier function.^[47]

Topical retinoids (eg, tretinoin) may be beneficial. They reduce cohesiveness of epithelial cells, stimulate mitosis and turnover, and suppress keratin synthesis.^[48]Tazarotene, a topical receptor-selective retinoid, has also been effective in one small trial.^[49]

Ichthyosis vulgaris is not responsive to steroids, but a mild topical steroid may be useful for pruritus.

Acquired ichthyosis vulgaris generally tends to improve with treatment of the underlying systemic condition.

Studies are being designed to quantify and compare the physiochemical properties of various topical emollients with the most desirable attributes to treat ichthyosis vulgaris.^[50]

Prevention

In many patients, scaling can be controlled by diligent and consistent treatment.

Medication

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Media Gallery

Hereditary ichthyosis vulgaris with thick scaling of the anterior shins.
Hematoxylin and eosin staining of acquired ichthyosis vulgaris in an adult. Shows epidermal atrophy with thinning of the granular layer. No dermal infiltrate is evident.

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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Jason F Okulicz, MD, FACP, FIDSA Director, HIV Medical Evaluation Unit, Infectious Disease Service, San Antonio Military Medical Center; Associate Professor of Medicine, F Edward Hebert School of Medicine, Uniformed Services University of the Health Sciences; Clinical Associate Professor of Medicine, University of Texas Health Science Center at San Antonio; Adjunct Clinical Instructor, Feik School of Pharmacy, University of the Incarnate Word

Jason F Okulicz, MD, FACP, FIDSA is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, Infectious Diseases Society of America

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Gilead Sciences.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, Association of Military Dermatologists, Phi Beta Kappa

Disclosure: Nothing to disclose.

Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Albert C Yan, MD Section Chief, Associate Professor, Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine

Albert C Yan, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology, Society for Pediatric Dermatology, American Academy of Pediatrics

Disclosure: Nothing to disclose.

Sections Hereditary and Acquired Ichthyosis Vulgaris
Overview
Presentation
DDx
Workup
Treatment
Medication
Media Gallery
References

Hereditary and Acquired Ichthyosis Vulgaris Treatment & Management

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