Darier disease (DD), also known as keratosis follicularis, 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.
Nearly 50% of patients with DD develop skin-colored flat-topped papules that are clinically and histologically indistinguishable from acrokeratosis verruciformis of Hopf, an allelic disorder of DD caused by variants in the ATP2A2 gene.
Patients are at high risk for secondary infection, e.g. bacteria, yeast. Rarely, herpes simplex infection may supervene--so called Kaposi varicelliform eruption.
Mosaic form of Darier disease is a rare subtype of Darier disease and is also described as segmental, zosteriform, linear, or localized. It presents later in life (eg, in the fourth or fifth decade) and lacks other classic features
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), tazarotene (two children cleared completely, each after using the gel once daily for 2 weeks 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.
Dupilumab and tralokinumab, which target type 2 inflammation, have shown promise in improving symptoms.
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