Sections

Sections Keratosis Follicularis (Darier Disease)
Overview
Presentation
DDx
Workup
Treatment
Medication
Questions & Answers
Media Gallery
References

Treatment

Medical Care

Basic measures

Comorbidities may include increased cardiovascular risk and should be evaluated. Depression may be associated with decreased quality of life.^{[43, 44, 45]}

Sunscreen, cool cotton clothing, and avoidance of hot environments can help prevent flares, especially during the summer.

Moisturizers with urea or lactic acid can reduce scaling and hyperkeratosis.

A low- or mid-potency topical steroid is sometimes useful for inflammation.

When bacterial overgrowth is suspected or crusting is prominent, application of antiseptics such as triclosan or soaks in astringents such as Burrow or Domeboro solution can be helpful.

Topical medications

Case reports have shown that topical retinoids (adapalene,^{[46, 47]}tazarotene gel 0.01%,^{[48, 49]}tretinoin^[50]) can reduce hyperkeratosis in 3 months. However, irritation is a limiting factor.

Emollients and topical corticosteroids can be used in combination with topical retinoids to reduce irritation.

Topical 5-fluorouracil (5-FU) has been used effectively in some patients.^{[51, 52]}

Tacalcitol lotion and sunscreen combination therapy has been reported for localized Darier disease.^[53]

Topical pimecrolimus has been reported to be successful in case reports.^[54]

A Spanish group reported the use of the COX inhibitor diclofenac sodium 3% gel successfully in two patients.^[55]

Botulinum toxin type A

Injection of botulinum toxin type A was reported in one case to significantly relieve the discomforting symptoms associated with keratosis follicularis (Darier disease) located in the submammary areas.^[56]

Systemic medications

Oral retinoids (eg, acitretin, isotretinoin,^[50]etretinate, alitretinoin^{[57, 58]}) have been the most effective medical treatment for keratosis follicularis (Darier disease), achieving some reduction of symptoms in 90% of patients. They reduce hyperkeratosis, smoothen papules, and reduce odor. In a study of 11 patients, 5 with keratosis follicularis (Darier disease) and 6 with pityriasis rubra pilaris, significant improvement occurred with isotretinoin therapy. All 11 patients received isotretinoin at 0.5 mg/kg/d, increasing to a maximum dose of 4 mg/kg/d, for a period of 16 weeks. Greater than 50% improvement occurred in all 5 patients with keratosis follicularis (Darier disease) and in 5 of 6 patients with pityriasis rubra pilaris. One patient showed no clinical improvement. Upon discontinuation of therapy, relapse occurred in all but 1 patient with pityriasis rubra pilaris.^[59] Targeted therapy is evolving.^[60]

Acitretin is effective at 0.6 mg/kg/d. The starting dose is 10-25 mg/d, which is gradually increased as tolerated. Long-term effects on bone should be monitored.^[61]

Isotretinoin at 0.5-1 mg/kg/d is especially useful in females of childbearing age because pregnancy need only be avoided for 1 month after stopping treatment. Unfortunately, prolonged remissions, such as those noted with isotretinoin for severe acne, are not seen in keratosis follicularis (Darier disease).

Etretinate (not available in the United States) has been reported useful if acitretin fails.^[62]

Alitretinoin (not available in the United States) at 30 mg/d has been used successfully by British and German groups for women of childbearing age because of its shorter half-life (2-10 h) compared with acitretin and is therefore an alternative to isotretinoin.^{[57, 58]}

Prolonged use of oral retinoids is limited by their significant adverse effects, including mucosal dryness, photosensitivity, hyperlipidemia, transaminitis, and skeletal hyperostosis. Oral retinoids are teratogenic, and appropriate counseling and contraception must be given.

Oral antibiotics are often necessary to clear secondary bacterial superinfection. They may also be used as prophylaxis to prevent infection.

Oral acyclovir may be used to treat or suppress herpes simplex virus infection.

Oral contraceptives have been reported to help with perimenstrual keratosis follicularis (Darier disease) flares.

Celecoxib, through cyclooxygenase-2 (COX-2) inhibition, was suggested as a possible therapeutic strategy based on one in vitro study which showed that COX-2 inhibition may restore downregulation of ATP2A2/SERCA2 expression in keratinocytes caused by ultraviolet B (UVB) irradiation.^[63]

Surgical Care

Dermabrasion has been used to smooth the hyperkeratotic lesions of keratosis follicularis (Darier disease), with acceptable results.^[64]

Electrosurgery^[65]and Mohs micrographic surgery have been used to treat localized keratosis follicularis (Darier disease) areas, with good results.

Laser ablation of recalcitrant plaques has been reported. In one report, 3 patients were treated with carbon dioxide lasers,^{[66, 67, 68]}2 with Er:YAG lasers,^[69]and 2 with pulsed-dye lasers.^[70]In all of these cases, treatment was successful, with only one patient developing disease recurrence in her axilla 7 months after treatment. Other reports describe resolution of disease using 1550-nm erbium-doped fractional fiber laser and other fractionated resurfacing devices.^{[71, 72]}

Carbon dioxide laser ablation with adjunctive dermabrasion, curettage, and shave excision in various combinations have also been reported to cause disease remission for 8 months to 2 years.^{[73, 74]}

Electron beam therapy has been used successfully for localized, recalcitrant Darier’s disease in a 51-year-old female.^[75]

Photodynamic therapy with 5-aminolevulinic acid was used to treat keratosis follicularis (Darier disease) lesions in 6 patients, with 4 patients showing sustained improvement or clearance for a follow-up period of 6 months to 3 years.^{[76, 77]}

Surgical excision of hypertrophic intertriginous keratosis follicularis has been described in one case report.^[78]

Medication

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Miyabe C, Mitsuhashi Y, Saito M, Tsuboi R. Novel mutation in the ATP2A2 gene in a Japanese Darier's disease patient with extremely hyperkeratotic lesions. J Dermatol. 2012 Apr. 39(4):401-3. [QxMD MEDLINE Link].
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Fong G, Capaldi L, Sweeney SM, Wiss K, Mahalingam M. Congenital Darier disease. J Am Acad Dermatol. 2008 Aug. 59(2 Suppl 1):S50-1. [QxMD MEDLINE Link].
Matsumoto A, Gregory N, Rady PL, Tyring SK, Carlson JA. Brief Report: HPV-17 Infection in Darier Disease With Acrokeratosis Verrucosis of Hopf. Am J Dermatopathol. 2017 May. 39 (5):370-373. [QxMD MEDLINE Link].
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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.