LAUSR

Darier’s disease (DD) is a rare, autosomal dominant chronic disease caused by a mutation in the ATP2A2 gene leading to abnormal keratinocyte signaling, acantholysis, and dyskeratosis. It is characterized by keratotic papules coalescing into vegetative plaques in a seborrheic distribution (Burge & Wilkinson, 1992; Engin, Kutlubay, Erkan, & Tuzun, 2015). Mild disease can be managed with emollients, topical steroids, and topical retinoids. Oral retinoids lead to improvement in approximately 90% of patients with severe disease (Engin et al., 2015). For recalcitrant localized disease, ablative techniques can be successful (Kittridge, Wahlgren, Fuhrer, Zirwas, & Patton, 2010; Lindelof, 1987). We present a patient with refractory DD with excellent response to three different modalities of radiation therapy: electron beam, intensity modulated radiation therapy (IMRT), and brachytherapy.