Therapeutic Options for the Treatment of Darier’s Disease: A Comprehensive Review of the Literature

Darier’s disease (also known as keratosis follicularis or dyskeratosis follicularis) is an autosomal dominant inherited disorder which manifests as hyperkeratotic greasy papules in the first or second decade of life. Aside from symptom management and behavioral modifications to avoid triggers, there are currently no validated treatments for Darier’s disease (DD). However, a variety of treatments have been proposed in the literature including retinoids, steroids, vitamin D analogs, photodynamic therapy, and surgical excision. The purpose of this review article is to identify therapeutic options for treating DD and to outline the evidence underlying these interventions. A search was conducted in Medline for English language articles from inception to July 4, 2020. Our search identified a total of 474 nonduplicate studies, which were screened by title and abstract. Of these, 155 full text articles were screened against inclusion/exclusion criteria, and 113 studies were included in our review. We identified Grade B evidence for the following treatments of DD: oral acitretin, oral isotretinoin, systemic Vitamin A, topical tretinoin, topical isotretinoin, topical adapalene gel, topical 5-flououracil, topical calciptriol and tacalcitol (with sunscreen), grenz ray radiation, and x-ray radiation. All other evidence for treatments of DD consisted of case reports or case series, which is considered grade C evidence. Considering the quality and quantity of evidence, clinicians may consider initiating a trial of select topical or oral retinoids first in patients with localized or generalized DD, respectively.

Darier’s Disease Flare following COVID-19 Vaccine

Darier’s disease is a rare genodermatosis characterized clinically by dyskeratotic papules in the seborrheic and intertriginous areas and nail abnormalities. Dyskeratosis and acantholysis are typical histological findings. Darier’s disease is not known to be inflammatory by nature as inflammation occurs primarily due to local infections, and it may therefore differ from inflammatory dermatoses such as psoriasis and cutaneous lupus in response to antigen stimulation. Known triggers of Darier’s disease primarily include exogenous factors such as sun exposure, friction, or infection. We present a case of a 47-year-old white female with a flare of Darier’s disease 2 days following her first vaccination with COVID-19 vaccine (ChAdOx1-s [recombinant]) (Vaxzevria^® [previously known as COVID-19 vaccine AstraZeneca]). In this case report, we discuss possible mechanisms linking the vaccination and the flare of Darier’s disease. We consider inflammatory mechanisms as well as a random co-occurrence. Due to the close time-related association between the disease flare and the COVID-19 vaccination, we find an urge to make other clinicians aware of a possible association.