Medication Summary

The goals of pharmacotherapy for keratosis follicularis (Darier disease) are to reduce morbidity and prevent complications.

Class Summary

These agents decrease cohesiveness of abnormal hyperproliferative keratinocytes and may reduce potential for malignant degeneration. They also modulate keratinocyte differentiation.

Adapalene (Differin)

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Adapalene modulates cellular differentiation, inflammation, and keratinization. It may be tolerated by individuals who cannot tolerate tretinoin creams. It is available as 0.1% gel or solution.

Tazarotene (Avage, Tazorac)

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Tazarotene is a retinoid prodrug whose active metabolite modulates differentiation and proliferation of epithelial tissue; it may also have anti-inflammatory and immunomodulatory properties.

Tretinoin topical (Avita, Renova, Retin-A, Tretin X)

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Tretinoin inhibits microcomedo formation and eliminates lesions present. It makes keratinocytes in sebaceous follicles less adherent and easier to remove. It is available as 0.025, 0.05, and 0.1% creams or 0.01 and 0.025% gels.

Acitretin (Soriatane)

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Acitretin is a metabolite of etretinate and is related to retinoic acid and retinol (vitamin A). The mechanism of action is unknown but it is thought to exert a therapeutic effect by modulating keratinocyte differentiation, hyperproliferation, and tissue infiltration by inflammatory cells. Its mechanism of action on keratosis follicularis (Darier disease) is unknown.

Isotretinoin (Amnesteem, Claravis, Sotret)

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Isotretinoin is an oral agent that treats serious dermatologic conditions. It is a synthetic 13-cis isomer of the naturally occurring tretinoin (trans-retinoic acid). Both agents are structurally related to vitamin A. Isotretinoin decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization.

A US Food and Drug Administration–mandated registry is now in place for all individuals prescribing, dispensing, or taking isotretinoin. For more information on this registry, see iPLEDGE. This registry aims to further decrease the risk of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy.

Questions

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Media Gallery

Typical distribution of keratotic papules in the seborrheic regions. Courtesy of Susan Mallory, MD, Director of Pediatric Dermatology, Washington University School of Medicine.
Longitudinal ridges, red and white lines, and V-shaped nicks. Courtesy of Susan Mallory, MD, Director of Pediatric Dermatology, Washington University School of Medicine.
Acantholysis and dyskeratosis (abnormal keratinization) are the 2 main features of Darier disease. Loss of epidermal adhesion with acantholysis frequently results in the formation of suprabasal clefts (lacunae).

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Author

Pui-Yan Kwok, MD, PhD Henry Bachrach Distinguished Professor, Department of Dermatology and Cardiovascular Research Institute, University of California, San Francisco, School of Medicine

Pui-Yan Kwok, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American Association for the Advancement of Science, American Society of Human Genetics, Dermatology Foundation, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Jillian Wong Millsop, MD, MS Resident Physician, Department of Dermatology, University of California, Davis, School of Medicine

Jillian Wong Millsop, MD, MS is a member of the following medical societies: American College of Physicians, American Medical Association, American Medical Women's Association

Disclosure: Nothing to disclose.

Tina Bhutani, MD Clinical Research Fellow, Department of Dermatology, University of California School of Medicine, San Francisco

Tina Bhutani, MD is a member of the following medical societies: American Medical Association, American Medical Women's Association, American Association of Physicians of Indian Origin, National Psoriasis Foundation, Indian Medical Association

Disclosure: Nothing to disclose.

Wilson Liao, MD Associate Professor, Department of Dermatology, University of California, San Francisco, School of Medicine

Wilson Liao, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology, National Psoriasis Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Steven R Feldman, MD, PhD Professor, Departments of Dermatology, Pathology and Public Health Sciences, and Molecular Medicine and Translational Science, Wake Forest Baptist Health; Director, Center for Dermatology Research, Director of Industry Relations, Department of Dermatology, Wake Forest University School of Medicine

Steven R Feldman, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, North Carolina Medical Society, Society for Investigative Dermatology

Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbvie for consulting; Received honoraria from Galderma for speaking and teaching; Received consulting fee from Lilly for consulting; Received ownership interest from www.DrScore.com for management position; Received ownership interest from Causa Reseasrch for management position; Received grant/research funds from Janssen for consulting; Received honoraria from Pfizer for speaking and teaching; Received consulting fee from No.

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

Arash Taheri, MD Research Fellow, Center for Dermatology Research, Department of Dermatology, Wake Forest University School of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Sarah Fitzmaurice, MD, Jillian Wong Millsop, MD, MS, Tina Bhutani, MD, and Wilson Liao, MD, to the development and writing of this article.

Sections Keratosis Follicularis (Darier Disease)
Overview
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Keratosis Follicularis (Darier Disease) Medication

Medication Summary

Retinoid-like Agents