What is the role of oral medications in the treatment of keratosis follicularis (Darier disease)?

Updated: Jul 31, 2018
  • Author: Pui-Yan Kwok, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
  • Print
Answer

Oral retinoids (eg, acitretin, isotretinoin, [47] etretinate, alitretinoin [54, 55] ) 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. [56]

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.

[57]

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. [58]

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. [54, 55]

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. [59]


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!