DARIER DISEASE (aka KERATOSIS FOLLICULARIS)

By Gary M. White, MD

Darier Disease


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.

Clinical

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].

Intellectual Disability

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].

Acrokeratosis verruciformis of Hopf vs Darier Disease

Treatment

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].

Electron Beam Therapy

EBT has been used successfully for Hailey Hailey Disease. Recalcitrant DD may respond to EBT as well.

Additional Pictures

Darier Disease Darier Disease

Darier disease of the nails.
Darier Disease Darier Disease

Darier Disease

Darier Disease Darier Disease

References

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

RegionalDerm

Homepage | FAQs | Use of Images | Contact Dr. White


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.