Darier disease

Management Strategy

General measures include antiseptic washes, liquid soap-free washes, and loose, cotton clothing. Some patients flare with ultraviolet (UV) exposure and need photoprotection.

In mild disease or linear disease reflecting genetic mosaicism, topical retinoids may be sufficient. These include topical isotretinoin (0.05%), tretinoin cream (0.05%), adapalene gel (0.1%), and tazarotene gel (0.1% short contact for 15 minutes). Treatment is applied on alternate days to begin with, increasing to once daily if possible, as irritation is common. The addition of a topical corticosteroid (alternating with the retinoid) may alleviate some of the side effects. Superinfection with viruses and bacteria is frequent, so combined corticosteroid/antibiotic preparations are logical. Sweating may be reduced by a trial of oral naltrexone.

In more extensive disease, an oral retinoid is required. Isotretinoin (0.5–1.0 mg/kg daily) and alitretinoin (30 mg daily) are preferred over acitretin because teratogenicity is a problem, and pregnancy is contraindicated for 2 years after stopping treatment with acitretin and 1 month after isotretinoin or alitretinoin. In males acitretin is an alternative but, bearing in mind that 50% of genetic relatives may have the same condition, such drugs could be dangerous if shared among females in the family. Compliance with contraceptive measures in females is very important as the treatment is usually long term.

The rare vesiculobullous form of the disease may respond to prednisolone. Hypertrophic flexural disease unresponsive to retinoids may require a surgical approach with laser , electrosurgery , debridement , or excision . Recurrence is a problem.

Oral lithium exacerbates the disease and should be avoided if possible.