By Gary M. White, MD
Also known as keratosis follicularis, Darier disease (DD) is a rare autosomal dominant genodermatosis characterized by keratotic, verrucous and greasy papules in the seborrheic areas.
Onset is usually in teenage or young adult years. The patient presents with scaly, waxy, greasy papules in the seborrheic areas. It very commonly involves the chest but may occur anywhere. Heat, sunlight and sweating often aggravate the condition. A linear form following Blaschko´s lines occurs. Patients are at high risk for secondary infection, e.g. bacteria, yeast. Rarely, herpes simplex infection may supervene--so called Kaposi varicelliform eruption [JAAD 2015;72;481].
Darier disease is associated with intellectual disability. For example, individuals with Darier disease have a sixfold increased risk of being diagnosed with intellectual disability [BJD 2015;173, 155–158].
Patients should avoid excessive heat, humidity and sun exposure. Oral retinoids--both acitretin and isotretinoin--are the most effective in this disease, although relapse occurs when the medication is discontinued. If chronic use is pursued, the lowest effective dose should be found. If there is a flare of inflammation or crusting, a secondary infection by Staph or Herpes should be considered and treated appropriately.
There has been some benefit to topical steroids and topical retinoids, e.g. adapalene 0.1% gel (cleared after 6 weeks of nightly use in one patient [Cutis 1999;63;227]), tazarotene (two children cleared completely, each after using the gel once daily for 2 weeks [PD 1999;16;243] or tretinoin. One study reported benefit in two patients of topical tazarotene 0.01% (0.05% diluted with purified water) once daily. The authors recommend this lower concentration to minimize irritation.
There is a report of diclofenac sodium 3% in hyaluronic acid 2.5% (Solaraze) applied BID nearly clearing two patients in 3 and 8 months [JAAD April 2014].
5-FU topically for two weeks resulted in complete remission in two patients [BJD 1995;133;463]. One of my patients does well applying benzoyl peroxide after the shower although one should always warn the patient about potential irritation.
Dermabrasion or laser vaporization (CO2 or YAG [AD 1999;135;423]) can induce long-term remission in localized areas.
Patients with recalcitrant areas of disease with hyperhidrosis may respond to Botox injections [JAMA Derm 2018;154;106].
EBT has been used successfully for Hailey Hailey Disease. Recalcitrant DD may respond to EBT as well.
Darier disease of the nails.
Herpes infection of Darier Disease. JAAD March 2015 Volume 72, Issue 3, Pages 481–484
Darier Disease of the nails. JAAD January 2011 Volume 64, Issue 1, Pages 167.e1–167.e11
Cobblestone appearance of the oral mucosa. From Indian J Dermatol Venereol Leprol 2014;80:432-47
Homepage | FAQs | Use of Images | Contact Dr. White